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 Table of Contents  
POSTER PRESENTATIONS
Year : 2018  |  Volume : 13  |  Issue : 6  |  Page : 93-241

Poster Presentations


Date of Web Publication12-Dec-2018

Correspondence Address:
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-3698.247336

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How to cite this article:
. Poster Presentations. Indian J Rheumatol 2018;13, Suppl S2:93-241

How to cite this URL:
. Poster Presentations. Indian J Rheumatol [serial online] 2018 [cited 2019 Sep 15];13, Suppl S2:93-241. Available from: http://www.indianjrheumatol.com/text.asp?2018/13/6/93/247336




  OPB0002: Impact of yoga and meditation based lifestyle intervention on quality of life and disease activity in rheumatoid arthritis Top


Gautam Surabhi, Kumar Uma, Kanga Uma, Kumar Manoj, Dada Rima; All India Institute of Medical Sciences, New Delhi, India

Background: Apart from usual medical therapy, recovery of patients with rheumatoid arthritis (RA) in which quality of life (QoL) is greatly compromised is dependent on several physical and psychological factors. With advancing therapeutic options, achieving a state of remission has become the treatment goal in RA.

Objective: The objective was to explore the effect of yoga and meditation-based lifestyle intervention (YMLI) on disease activity, QoL, and inflammatory markers in active RA group compared with usual-care control group.

Methods: A total of 56 individuals were randomized into two groups: YMLI group and usual-care control group which were assessed pre (day 0) and post (8 weeks) intervention for reactive oxygen species (ROS), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and a panel of pro-inflammatory (interleukin [IL]-6, IL-17A, tumor necrosis factor-α [TNF-α]) and anti-inflammatory cytokines (transforming growth factor-β [TGF-β] and soluble human leukocyte antigen-G [sHLA-G]). Parameters of disease activity, disability quotient, pain acuity, and QoL were also assessed by Disease Activity Score (DAS28), Health Assessment Questionnaire (HAQ), Visual Analog Scale (VAS), and World Health Organization QoL (WHOQOL-BREF), respectively.

Results: YMLI participants showed significant improvements in DAS28 (P < 0.05***), HAQ (P < 0.05***), VAS (P < 0.05***), and WHOQOL-BREF scale (P < 0.05***) in all the four domains of physical health, psychological health, social relationships, and environmental health over the control group. In YMLI group, there was reduction in mean levels of CRP (P < 0.05***), ROS (P > 0.05), TNF-α (P < 0.05**), IL-6 (P < 0.05**), IL-17A (P < 0.05**), and ESR (P < 0.05*) and elevation in sHLA-G (P < 0.05**) and TGF-β (P < 0.05**) at 8 weeks compared to baseline level (day 0).

Conclusion: The present study demonstrated that yoga results in the regression of inflammatory processes by reducing inflammatory cytokines and regulating the levels of soluble HLA-G significantly in active RA patients. YMLI has significantly reduced pain perception, disability quotient, and disease activity and improved QoL. Thus, yoga aids in achieving immunological tolerance and molecular remission and hence can be beneficial as an adjunct therapy.


  OB0002: Importance of screening of folate pathway alterations for rheumatoid arthritis patients for selecting methotrexate-based therapy: A North-East patient-based study Top


Somdatta Das, Chitralekha Baruah1, Dr. Anjan Kr. Saikia2, Sujoy Bose3; Department of Bio-Engineering and Technology, Gauhati University, 1Department of Medicine, Gauhati Medical College and Hospital, 2Gastroenterology and Hepatology, Gauhati Neurological Research Centre, 3Department of Biotechnology, Gauhati University, Guwahati, Assam, India

Background: Folate pathway is associated with DNA synthesis, repair, and methylation. The disease-modifying antirheumatic drug methotrexate (MTX) shows variable treatment efficacy and response in rheumatoid arthritis (RA) patients, and is known to target two important genes MTHFR and TYMS in this pathway. Given the side effects of MTX toxicity, it makes the candidature of the screening of MTHFR and TYMS alteration(s) at genetic level and homocysteine estimation an important agendum prior to treatment.

Objectives: The study objective was to determine the association of genetic and biochemical alteration(s) in the folate pathway in RA pathogenesis in North-East Indian patients.

Methods: A total of 126 RA patients were enrolled for the study based on ACR and EULAR (2010) criteria, along with 160 community matched age and sex controls. MTHFR codon 677Cà T and TYMS 3'UTR 6 bp (ins/del) polymorphism was screened by polymerase chain reaction-restriction fragment length polymorphism method. Homocysteine estimation was studied by enzyme-linked immunosorbent assay. All statistical analyses were performed using SPSS software.

Results: The MTHFR 677T altered genotype showed an association with disease susceptibility (odds ratio [OR] = 3.084, P = 0.002), and the TYMS 3'UTR ins/ins showed an association with both disease susceptibility (OR = 1.525, P = 0.101) and severity (OR = 1.730, P = 0.051). The serum homocysteine levels were higher in severe RA cases (P = 0.026) as compared to controls. MTHFR variant genotype (P = 0.012) and TYMS variant del/ins (P = 0.007) and ins/ins (P = 0.003) variant genotype were associated with increased homocysteine levels.

Conclusion: Genetic alterations in MTHFR gene and TYMS 6 bp ins/ins genotype and hyper- homocystenemia are associated with RA pathogenesis. Hyper-homocystenemia has been documented for its association with comorbidities in RA patients, was found to be dependent on MTHFR gene and TYMS 6 bp ins/ins genotype, and therefore both homocysteine levels and folate pathway gene alterations hold prognostic significance for MTX treatment feasibility and predicting the treatment efficacy in North-East Indian RA patients.


  OPB0003: Perforin and Granzyme expression: Role of natural killer cell cytotoxicity in acute phase of Kawasaki disease Top


Aman Gupta, Jitendra Kumar Shandilya, Deepti Suri, Amit Rawat, Biman Saikia1, Surjit Singh; Allergy Immunology Unit, Advanced Pediatrics Centre, 1Department of Immunopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: Acute phase of Kawasaki disease (KD) is characterized by the activation of cytokine cascade resulting in multisystem vasculitis. Elevated levels of interleukin (IL)-1, IL-2, IL-6, IL-8, interferon-α, and tumor necrosis factor-α are well described in literature. However, the role of peripheral natural killer (NK) cell population and its cytotoxic proteins has not been extensively studied.

Objective: The objective was to study the expression of perforin and granzyme on CD3-56+ NK cells during acute and convalescent phases of KD and in febrile controls.

Methods: Fourteen children diagnosed with KD were recruited from the Pediatric Rheumatology Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, during July 2016–June 2017. The diagnosis of KD was based on the American Heart Association 2004 criteria. Twelve age-matched febrile children (with diagnosis other than KD) with duration of fever 5–10 days were taken as controls. Perforin and granzyme expression was assayed on CD3-56+ NK cells and compared between acute phase of KD before giving intravenous immunoglobulin, during convalescence (defined as >4 weeks but <6 weeks after the onset of illness) and in febrile controls.

Results: Significantly decreased mean perforin expression was noted during the acute phase of KD as compared to febrile controls (67.16% vs. 82.64%, P = 0.041). Mean granzyme expression was also lower in these children (87.23% vs. 97.63%, P = 0.887). Following treatment with intravenous immunoglobulin, mean perforin expression decreased (60.83% vs. 67.16%, P = 0.427), while mean granzyme expression increased (99.05% vs. 87.23%, P = 0.514) in children with KD. Children with coronary artery abnormalities (CAAs) had increased expression of perforin (69.06% vs. 66.11%, P = 0.898) and granzyme (96.17% vs. 82.77%, P = 0.933) during acute phase as compared to children without CAAs.

Conclusions: The acute phase of KD is characterized by significantly decreased expression of perforin. Perforin and granzyme B could be novel therapeutic targets for the management of acute phase of KD and for the prevention and treatment of cardiovascular diseases associated with KD.


  OPB0027: Evaluation of in vivo and in vitro T-cell responses to purified protein derivative as indicators of latent tuberculosis infection in disease-modifying antirheumatic drug-naïve rheumatoid arthritis patients Top


Suvrat Arya, Shashi Kant Kumar, Alok Nath1, Prerna Kapoor2, Amita Aggarwal, Ramnath Misra, Sudhir Sinha; Departments of Clinical Immunology and 1Pulmonary Medicine, Sanjay Gandhi Postgraduate Institute of Medical Sciences, 2DOTS Centre, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Latent tuberculosis infection (LTBI) is an “environmental” predisposing factor for rheumatoid arthritis (RA). Tuberculin skin test (TST) is widely used for the detection of LTBI, though evidences suggest that it could be underreporting the true prevalence of LTBI.

Objectives: The objectives were to determine and compare, as a measure of LTBI, the in vivo (TST) and in vitro (cell proliferative) T-cell responses to purified protein derivative (PPD) in disease-modifying antirheumatic drug (DMARD)-naïve RA patients.

Methods: The study participants comprised 215 DMARD-naïve (taking <15 mg prednisolone or equivalent steroids) RA patients and 43 health-care workers (HCWs) who served as “controls.” Demographic data including Bacillus Calmette–Guérin (BCG) vaccination, past tuberculosis (TB), or household contact with TB were recorded. TST was performed with 5 TU PPD. Blood T-cell (CD3+) responses to PPD were determined by flow cytometry in terms of the proliferation-induced nuclear protein – Ki67.

Results: Nearly 28% (61/215) of RA patients showed positivity for TST (induration ≥10 mm). Among TST-positive patients, 16.4% (10/61) had past TB and 9.8% (6/61) had a family history of TB. Their mean disease duration was 4.66 ± 3.91 years and mean Disease Activity Score 28 erythrocyte sedimentation rate was 6.27 ± 1.20. Almost 57% (35/61) of TST-positive and 30% (3/10) of TST-negative patients showed proliferative T-cell response to PPD. Among HCWs, 56% (24/43) and 67% (29/43), respectively, were positive for in vivo (TST) and in vitro (T-cell proliferative) responses to PPD. In addition, 67% of TST-negative HCWs were also positive for the in vitro response. BCG scar was seen in 55.7% of patients and 81% of HCWs, though it did not influence their responses to PPD.

Conclusions: A significant proportion of RA patients had a history of TB or exposure to TB. However, their in vivo and in vitro responses to PPD were significantly lower than those of HCWs. The results also point to partially complementary nature of in vitro and in vivo responses to PPD.


  OPB0005: Synergy between tuberculin skin test and proliferative T-cell responses to mycobacterium tuberculosis antigens for the detection of latent tuberculosis infection in a high disease-burden setting Top


Suvrat Arya, Shashi K Kumar, Alok Nath1, Prerna Kapoor2, Amita Aggarwal, Ramnath Misra, Sudhir Sinha; Departments of Clinical Immunology and 1Pulmonary Medicine, Sanjay Gandhi Postgraduate Institute of Medical Sciences, 2DOTS Centre, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Latent tuberculosis infection (LTBI) is defined as “a state of persistent immune response to Mycobacterium tuberculosis (MTB) without clinically manifested disease.” The tuberculin skin test (TST) and interferon gamma release assays are the most commonly used tests for the detection of LTBI. However, in a high tuberculosis (TB) burden setting such as India, both the assays have been found to grossly underestimate the true prevalence of LTBI.

Objective: This study was aimed at exploring whether an in vitro CD3+ T-cell response to purified protein derivative (PPD) can complement the in vivo TST response for the determination of true prevalence of LTBI in health-care workers (HCWs).

Methods: The study participants comprised 92 HCWs. Nine smear-positive TB patients served as “disease control.” To measure proliferative T-cell responses, whole blood (0.1 ml) was incubated (5 days) with test or control antigens. Cells were stained with fluorescent antibodies to T-cell surface markers and after fixation and permeabilization to Ki67 (nuclear proliferation marker). Data were acquired on a flow cytometer.

Results: Household contacts and cured TB cases showed significantly higher TST responses than remainder HCWs, irrespective of their Bacillus CalmetteGuérin vaccination status or age. Proliferative responses of CD4+ and CD8+ T-cells to MTB antigens were comparable. Nearly 68% and 100% of TST-negative HCWs were positive, respectively, for T (CD3+) cell responses to PPD and MTBMem. Cumulative positivity (TST or in vitro) was 86% for PPD and 100% for MTBMem. As stand-alone in vitro assay, MTBMem produced significantly higher positivity (95%) than PPD (67%). T-cell responses of pulmonary TB patients were generally suppressed.

Conclusions: These results demonstrate that in vivo and in vitro T-cell responses to PPD are complementary, and in vitro response to MTBMem can be developed as a highly sensitive biomarker for LTBI.


  OPB0009: Evidence of inflammation amplification by interleukin-6 and interleukin-17A in synovial compartment of juvenile idiopathic arthritis: Enthesitis-related arthritis Top


Rutviz Mistry, Sandeep Kumar, Sanjukta Majumder, Amita Aggarwal, Ramnath Misra; Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: F759 mice with single amino acid substitution in interleukin (IL)-6 receptor gp130 (Y759F) develop features of autoimmune disorders such as arthritis, multiple sclerosis due to IL-17A, and IL-6-mediated synergistic activation of positive feedback loop of STAT3 and nuclear factor-κB signaling (inflammation amplifier) in nonimmune cells such as fibroblasts. IL-17A activates epiregulin/ErbB1 axis, which in turn triggers the activation of multiple growth factors such as placental growth factor (PLGF), amphiregulin. These growth factors contribute to this amplification loop in rheumatoid arthritis patients and blocking them abrogates inflammation in animal models. In this study, we explored the possibility whether this inflammation amplifier loop is playing a role in synovitis of juvenile idiopathic arthritis-enthesitis-related arthritis (JIA-ERA) by estimating PLGF in sera and synovial fluid (SF) of patients.

Methods: Sera and SF of thirty adolescents of JIA-ERA, diagnosed as per the ILAR classification, with effusion undergoing therapeutic aspiration, were collected and stored between October 2016 and April 2018. Disease activity was measured. Levels of IL6, IL-17A, and PLGF were measured by enzyme-linked immunosorbent assay.

Results: The mean ages of patients and controls were 16.4 ± 3.35 and 17.65 ± 0.58 years, respectively (P = 0.11). The mean duration of disease was 4.318 years. Two-third of the patients were on nonsteroidal anti-inflammatory drugs and few were on disease-modifying drugs. Juvenile Spondyloarthritis Disease Activity and Ankylosing Spondylitis Disease Activity Score C-reactive protein of the patients were 4.11 ± 1.36 and 2.96 ± 0.82, suggesting active disease with moderate disease activity. IL-6, IL-17A, and PLGF were significantly higher in SF as compared to serum (P < 0.0001 for each). However, only IL-6 was significantly higher in serum of patients as compared to healthy controls (IL-6, P < 0.001; IL-17A, P = 0.092; and PLGF, P = 0.655). Serum IL-6 correlates with SF PLGF (r = 0.460, P = 0.01) and serum IL-17A (r = 0.443, P = 0.01). However, no correlation of ILs/growth factors in SF with disease activity scores was found.

Conclusion: The increased SF levels of IL-6, IL-17A, and PLGF suggest the activation of IL-6- and IL-17A-mediated amplification loop in synovial compartment of patients with JIA-ERA. Further study of other growth factors such as epiregulin, amphiregulin, and norepinephrine is warranted.


  OPB0012: Epigenetic regulation of multidrug resistance genes by histone deacetylase 2 in steroid resistance via regulation of P-glycoprotein and multidrug resistance-associated protein-1 in patients of nephrotic syndrome Top


Harshit Singh, Narayan Prasad1, Saurabh Chaturvedi, Akhilesh Jaiswal1, Mohit Kumar Rai, Kritika Singh, Ravi Mishra, Sushma Singh1, Mantabya Singh1, Ranjeet Singh Chauhan1, Vikas Agarwal; Departments of Clinical Immunology and 1Nephrology, SGPGIMS, Lucknow, Uttar Pradesh, India

Background: The action of glucocorticoids is to switch off activated inflammatory genes. The activated glucocorticoid receptors (GRs) interact with co-repressor molecules to impair nuclear factor kappa B-associated co-activator activity, reducing histone acetylation. Reduction in histone acetylation occurs via recruitment of histone deacetylase 2 (HDAC2) to the activated inflammatory gene complex by activated GR, resulting in efficacious suppression of activated inflammatory genes within the nucleus.

Objective: The objective was to evaluate the role of HDAC2 in glucocorticoid resistance

Methods: A total of twenty individuals; ten patients with steroid-sensitive nephrotic syndrome (SSNS) and ten patients with steroid-resistant nephrotic syndrome (SRNS) were recruited. mRNA expression of P-glycoprotein (P-gp), multidrug resistance-associated protein-1 (MRP-1), and HDAC2 were analyzed on peripheral blood mononuclear cells (PBMCs) in SRNS (age: 8.43 ± 3.8 years) and SSNS (age: 7.54 ± 3.5 years). PBMCs were treated with 1 μM of theophylline (HDAC2 stimulator) and 0.8 μM of trichostatin A (HDAC2 inhibitor) for 48 h. HDAC2 gene expression levels were analyzed with quantitative polymerase chain reaction.

Results: Expression of P-gp [4.79 ± 0.10 vs. 2.13 ± 0.12, [Figure 1]a]/MRP-1 [3.99 ± 0.08 vs. 1.99 ± 0.11, [Figure 1]b] on PBMCs increased in SRNS compared to that of SSNS, whereas HDAC2 mRNA levels decreased [2.97 ± 0.15 vs. 6.02 ± 0.13, [Figure 1]c]. Theophylline decreased the mRNA levels of P-gp/MRP-1 in PBMCs of SRNS with maximal induction at 1 μM (fold change 2.65 and 2.21, *P < 0.0001). However, HDAC2 mRNA expression increased (fold change 5.67, *P < 0.0001). In SSNS, P-gp/MRP-1 mRNA expression decreased at 1 μM (fold change 1.25, 1.24, *P < 0.0001), while mRNA expression increased (fold change 6.93, *P < 0.0001). Trichostatin A increased the mRNA levels of P-gp/MRP-1 in PBMCs of SRNS with maximal induction at 0.8 μM (fold change 7.51/7.31, *P < 0.0001) and decreased the level of HDAC2 (fold change 1.50, *P < 0.0001). Similarly, in SSNS, P-gp/MRP-1 mRNA expression increased at 0.8 μM (fold change 3.49/3.35, *P < 0.0001) and HDAC2 decreased (fold change 2.53, *P < 0.0001) at 0.8 μM.
Figure 1

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Conclusion: We observed that HDAC2 regulates P-gp and MRP-1.


  OPB0011: Discordance between antinuclear antibody patterns by immunofluorescence and line immunoassay in systemic lupus erythematosus Top


Daisy Doley, Rebecca Marak, Sanjeeb Kakati, Sakir Ahmed; Department of Medicine, Assam Medical College and Hospital, Dibrugarh, Assam, India

Background: Antinuclear antibody (ANA) test by indirect immunofluorescence (IIF) reveals distinct patterns. Each pattern is associated with antibodies to certain nuclear antigens.

Objectives: The objectives were to study the immunofluorescence pattern of ANA and to look for any concordance between ANA-IIF and line immunoassay (LIA) in systemic lupus erythematosus (SLE).

Methods: sera of 134 diagnosed cases of SLE were tested for ANA by IIF on HEp-2 cell and LIA using AESKUBLOT© ANA-17 Pro which has 17 antigens per test strips. Concordance between the IIF patterns and LIA results was checked.

Results: A total of 44 (32.8%) sera exhibited the coarse speckled pattern, followed by homogeneous in 24 (17.9%); fine speckled in 20 (14.9%); cytoplasmic, rim, and centromere in 2 (1.5%) each; and Golgi pattern in 1 (0.7%). A total of 39 (29.1%) sera exhibited a mixed pattern. Twenty (14.9%) patients had no positivity on LIA profile. The most frequently found autoantibody was anti-SSA (Ro60) in 65 (48.5%) patients followed by anti-dsDNA in 64 (47.8%), anti-SSA (Ro52) in 50 (37.3%), and anti-histone in 46 (34.3%) patients. The coarse speckled pattern (29.9%) correlated with antibodies against SSA, dsDNA, Sm, histone, and nucleosome. Among dsDNA-positive patients, the most frequently encountered were coarse speckled and mixed patterns in 15.7%. However, there was no further concordance between IIF and LIA.

Conclusion: There was a high rate of discordance between IIF and LIA. Thus, there is a need to perform both and find which matches the clinical profile of the patient better.


  OPB0014: Elevated levels of granzyme B-producing circulating lymphocytes in psoriatic arthritis: An observational study Top


Satarupa Dutta, Anirudhha Bagchi1, Ayindrila Saha, Sulagna Chatterjee, Sumantro Mondal, Alakendu Ghosh; Departments of Rheumatology and 1Pharmacology, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, India

Background: Psoriatic arthritis (PsA) is a progressive and often destructive form of inflammatory arthritis, with inflammation of synovium, entheses, and spine. Interleukin (IL)-12 is a major pro-inflammatory cytokine which gets increased in patients with PsA. Higher frequency of CD8+ T-cells in PsA patients contributes to cytotoxicity with a poorly understood mechanism.

Objective: The study objective lies in the investigation of the frequency of lymphocyte subsets and their functionality through granzyme B (GzmB) production in peripheral blood of patients with PsA.

Methods: PsA patients (CASPAR criteria, 2006) and age- and sex-matched healthy controls (n = 10) were recruited. Disease activity scores (Psoriasis Area and Severity Index and Disease Activity Index for Psoriatic Arthritis) of PsA patients were measured. Patients were on methotrexate (MTX) for more than 3 months. Venous blood was drawn; peripheral blood mononuclear cells were isolated; and the frequency of CD8+T-lymphocytes, CD4+ T-lymphocytes, natural killer cells (CD3-/CD16+/CD56+), natural killer T (NKT)-cells (CD3+/CD16+/CD56+), and B-lymphocytes (CD19+) were observed by immunophenotyping. Pro-inflammatory intracellular cytokine (i.e., IL-12) and GzmB levels were observed too by flow cytometric analysis in lymphocytes and monocytes of PsA patients.

Results: The frequency of CD8+ T-cells (P < 0.001) and NKT cells (P < 0.05) in PsA patients is significantly upregulated in comparison with healthy controls. GzmB production by lymphocytes is also significantly (P < 0.001) higher in patient group. This study also suggests that the frequency of monocytes producing IL-12 is significantly (P < 0.001) higher than that of lymphocytes in PsA patients.

Conclusion: Enhanced IL-12 level may cause proliferation and activation of CD8+ T-cells and NKT cells, which might contribute to increased cytotoxicity by producing significantly higher level of GzmB in PsA patients. Despite the administration of MTX, elevated level of cytotoxicity indicates that MTX is not adequate enough to reduce inflammation in PsA.


  OB0003: Evaluating most feasible outcome measure for disease activity, adherence to DMARDs and prescription patterns in a government hospital OPD in the state of Gujarat Top


Viraj Panchal, Supriya Malhotra1, Devang Rana1, Puja Srivastava2, Sapan Pandya2; 2ndyear MBBS, Undergraduate, 1Departments of Pharmacology and 2Medicine, Smt. N. H. L. Municipal Medical College, Ahmedabad, Gujarat, India

Background: Most patients presenting to government hospital are from lower socioeconomic class and do not always get investigations done. A purely clinical index like Clinical Disease Activity Index (CDAI) would be a useful measure in such resource-poor settings. In addition, there is scarce data on drug adherence and patterns of disease-modifying antirheumatic drug (DMARD) use from our country.

Objectives: The objectives were (1) to evaluate if CDAI is as good as Disease Activity Score (DAS) 28, erythrocyte sedimentation rate (ESR), or C-reactive protein (CRP); (2) DMARDs' adherence to patients; and (3) prescription patterns of DMARDs.

Method: This was a prospective cross-sectional study of 4 months' duration. DAS28-ESR, DAS28-CRP, and CDAI were calculated. Modified Morisky Medication Adherence Scale-8 was used to study the adherence, and details of DMARDs used were recorded in pro formas. Correlation was done using Pearson's correlation coefficient test. Validation of the scale was done using Cohen's kappa test. P <0.05 was considered statistically significant.

Results: We evaluated a total of 104 patients, most of them being females with a mean age of 45 years. The mean DAS28-ESR was 4.59 ± 1.36, mean DAS28-CRP was 3.86 ± 1.25, and mean CDAI was 16.9 ± 9.26. The correlation coefficient of DAS28-ESR with DAS28-CRP was 0.894, that of DAS28-ESR with CDAI was 0.887, and that of DAS28-CRP with CDAI was 0.904. Reliability test was carried out, and Cronbach's alpha value obtained was 0.444. Most patients were on medium adherence (44.23%) followed by low adherence (39.42%) and high adherence (16.34%). Methotrexate was the most prescribed drug as a monotherapy or in a combination with hydroxychloroquine followed by leflunomide, sulfasalazine, and prednisolone in that order.

Conclusion: The CDAI can substitute for SDAI or DAS28 ESR/CRP, and this would be very useful in a resource-poor setting. Most patients in our outpatient department were only moderately adherent to the prescribed DMARDs and MTX was the most prescribed drug in our setup as monotherapy or combination therapy.


  PB0006: Is pulmonary sarcoidosis is same like pulmonary tuberculosis defining with marker CD64 Top


Ravi Mishra, Mohit Kumar Rai1, Harshit Singh1, Saurabh Chaturvedi1, Zia Hashim, Vikas Agarwal1; Departments of Pulmonary Medicine and 1Clinical Immunology, SGPGI, Lucknow, Uttar Pradesh, India

Background: Sarcoidosis is a complex clinical condition. It is still unclear that what drives abnormal immune response in sarcoidosis; either it is microbial trigger or abnormal immune activation by loss of tolerance. Timely diagnosis and information about disease-causing agent may help in early intervention. CD64 is expressed by monocytes/macrophages, dendritic cells, neutrophils, and mast cells. Previous studies have established that higher expression of CD64 acts as a marker of microbial infection and improves antigen capture and phagocytosis of immune complexes and antibody-dependent cellular cytotoxicity. Thus, to assess the causative agent, we studied CD64 expression.

Methods: In this study, we had recruited 41 patients based on ATT culture, computed tomography thorax, X-ray, serum angiotensin-converting enzyme, and other diagnostic tests of both diseases. Group 1 included 15 healthy controls (8 males and 7 females, mean age of 34.5 ± 8.4 years), Group 2 included 12 sarcoidosis patients (8 males and 6 females, mean age of 34.4 ± 10.5 years), and Group 3 included 14 tuberculosis patients (8 males and 7 females, mean age of 36.5 ± 12.5 years) as a study control for microbial infection. Heparinized blood samples were collected and surface stained for CD64 antibody (Ebiosciences, USA) using appropriate isotypic control samples which were analyzed in separate tubes. CD64 was analyzed. Flow cytometric analysis was performed on FACS Canto II (Becton Dickinson, Mount view, CA, USA).

Results: In Groups 2 and 3, CD64 expression remains same on lymphocytes and monocytes, while their expression gets upregulated on neutrophil compared to Group 1. Expression of CD64 gets upregulated on sarcoidosis patients (P = 0.002) compared to healthy controls similar to tuberculosis patients. Following antibiotic treatment, clinical condition of patients improved, which goes hand in hand with significant reduction in CD64 expression.

Conclusions: In critically ill sarcoidosis patients, significant increase of CD64 similar to tuberculosis patients suggests that sarcoidosis may be caused by microbial infection.


  PB0002: Correlation of clinical characteristics and serial determination of neutrophil CD64 expression in patients with sepsis Top


Kritika Singh, Harshit Singh, Saurabh Chaturvedi, Rupali Patnaik1, Mohan Gurjar1, Vikas Agarwal; Departments of Clinical Immunology and 1Critical Care Medicine, SGPGIMS, Lucknow, Uttar Pradesh, India

Background: Sepsis is a major cause of mortality in intensive care unit (ICU). Early identification and timely interventions hold a key role to improve morbidity and mortality in sepsis. Biomarkers are still being explored in the early diagnosis and management of sepsis. Neutrophilic CD64 has been found to be a predictor of survival and severity of sepsis.

Objective: The goal of this study was to correlate the serial determination of CD64 with patient characteristics and ICU outcome.

Method: A prospective observational study in 12-bedded critical care unit of a tertiary care center was conducted after taking ethical clearance from the local body. Patients aged <18 years and those with absence of sepsis were excluded from the study. Demographics, illness severity scores, clinical parameters, laboratory data, and 28-day outcome were recorded. Serial CD64 analysis was done (on days 0, 4, and 8) in consecutive patients. CD64 was assessed by flow cytometry method. A cutoff value of >30% was suggestive of sepsis. For statistical analysis, SPSS software version 20 was used (P < 0.05 was considered statistically significant).

Results: Sixteen consecutive patients with a mean age of 45.56 ± 17.16 years with a male-to-female ratio of 1:1 were recruited. Median Acute Physiology and Chronic Health Evaluation II score was 8 (7–12) and mean Sequential Organ Failure Assessment (SOFA) score was 9 ± 2.5. Mean CD64 at admission was 62 ± 29.64. Nine patients presented with septic shock on admission. Comparing survivors and nonsurvivors, all demographic parameters were similar except age which was higher in nonsurvivor group (P < 0.05). SOFA was higher in nonsurvivors (P = 0.07). CD64 could not differentiate between survivors and nonsurvivors but was higher in septic shock patients than those with sepsis (P = 0.05) at admission (68.26 ± 27.12 vs. 42.17 ± 30.76) and also at other points of measurement. Among the septic shock subgroup, patients who survived had a decreasing trend of CD64 (P = 0.02).

Conclusion: CD64 was higher in septic shock patients. Trend of CD64 may help in identifying treatment responders in septic shock patients.


  PB0003: Utility of soluble triggering receptor expressed on myeloid cells type 1 and procalcitonin to differentiate bacterial infection from disease flare in systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis Top


Mantabya Singh, Harshit Singh, Saurabh Chaturvedi, Sajal Ajmani, Vikas Agarwal; Department of Clinical Immunology, SGPGIMS, Lucknow, Uttar Pradesh, India

Background: Differentiation between a flare of a rheumatic disease and systemic infection in a patient receiving immunosuppressive treatment is vitally important as the treatment differs dramatically. However, in routine clinical practice, this remains a common dilemma since many patients present with signs and symptoms of nonspecific inflammation such as fever, arthralgia, and shortness of breath that can be caused by both disease activity and infection. Soluble triggering receptor expressed on myeloid cells type 1 (sTREM-1) and procalcitonin are evaluated for the selection of infection.

Objective: The objective was to evaluate sTREM-1 in serum to differentiate between systemic infection and active autoimmune inflammation in patients with systemic lupus erythematosus (SLE) and antibody-associated vasculitis (AAV).

Methods: A total of 76 patients and 20 healthy controls were included in the study. Patients were divided into the following three groups: active disease, proven infection, and probable infection. sTREM-1 and procalcitonin levels were measured in duplicate after a single freeze-thaw cycle in batched assays by sandwich enzyme-linked immunosorbent assay as per manufacturer's protocol.

Results: Total leukocyte count, erythrocyte sedimentation rate, C-reactive protein, C3, C4, and anti-dsDNA, in SLE, and titers of anti-myeloperoxidase and anti-PR3 in antineutrophil cytoplasmic antibody-associated vasculitis were not significantly different in patients with active disease and infection.

Mean (range) sTREM-1 was higher in both patients with active disease (1184 [717–1609] pg/ml) and infection (899 [531–1284] pg/ml) in comparison to healthy controls (255.1 [95.1–634.8] pg/ml). Procalcitonin was higher in patients with infection compared to those with active disease. Procalcitonin value of 244 pg/ml had a sensitivity of 75% and a specificity of 85% to differentiate infection from active disease with an area under the curve of 0.75. The positive and negative predictive values were 70.4% and 87.8%, respectively.

Conclusion: Compared to sTREM-1, procalcitonin is better in differentiating infection from disease activity in patients with SLE and AAV.


  OPB0017: Common polymorphisms in methotrexate pharmacokinetic pathway influence symptomatic and laboratory adverse effects in patients with rheumatoid arthritis treated with methotrexate Top


Varun Dhir, Amit Sandu, Archana Bhatnagar, Veena Dhawan; Post Graduate Institute of Medical Education and Research, Chandigarh, India

Background: Many patients with rheumatoid arthritis (RA) cannot tolerate methotrexate (MTX) – the gold standard disease-modifying antirheumatic drug due to adverse effects (AEs) – both symptomatic and laboratory. Genetic polymorphisms in pharmacokinetic pathway of MTX may be determinants.

Objectives: The objective was to study the impact of seven polymorphisms – ABCB1 3435C>T, ABCB1 1236C>T, FPGS 1994A>G, FPGS G>A, GGH 452C>T, GGH 401C>T, and RFC1 80G>A – on symptomatic and laboratory AEs in RA.

Methods: This was a prospective study that enrolled patients with RA who fulfilled 1987 ACR criteria and were not on MTX. They were treated with MTX for 24 weeks. Symptomatic AEs were ascertained using a questionnaire. In addition, laboratory AEs – episodes of transaminitis (serum glutamic oxaloacetic transaminase or serum glutamic pyruvic transaminase >40 IU/L) and cytopenias (platelet <100,000/μl and total leukocyte count <4000/μl) – were noted. Single-nucleotide polymorphisms (SNPs) were tested using Taqman probe-based real-time-polymerase chain reaction. Chi-square test was used to look at the association between genotype and AEs.

Results: This study included 117 patients with RA (male:female = 14:103), with mean age and disease duration of 42.7 ± 11.9 and 2.0 ± 1.7 years, respectively. Mean (±standard deviation) MTX dosage at 24 weeks was 22.0 ± 4.0 mg/week. Forty patients had at least one symptomatic AE – nausea in 29; anxiety and dizziness in 9; fatigue in 3; and lung toxicity, fever, and oral ulcers in 1 each. FPGS 1994GG genotype was associated with a significantly lower risk of AEs to MTX (odds ratio: 0.3 [95% confidence interval: 0.1–0.6]). On multivariate analysis, FPGS1994GG genotype and lower body mass index were significant predictors for symptomatic AE with an accuracy of 66%. There were 48 patients who had at least one episode of transaminitis and 16 with one episode of cytopenias (laboratory AEs). Among the laboratory AEs, rs1051266 (RFC1 80G>A) was found to be significantly associated with the occurrence of cytopenias (P = 0.02).

Conclusion: SNP FPGS 1994A>G was associated with symptomatic AE, whereas RFC80 A>G was associated with cytopenias on MTX treatment in RA.


  PB0004: Interleukin-17/interferon-γ double-positive Th17 cells selectively express P-glycoprotein and are refractory to glucocorticoids Top


Akhilesh Jaiswal, Narayan Prasad, Mantabya Singh, Mohit Rai1, Harshit Singh1, Saurabh Chaturvedi1, Vikas Agarwal1; Departments of Nephrology and 1Clinical Immunology, SGPGIMS, Lucknow, Uttar Pradesh, India

Background: Th17 cells and cytokine interleukin (IL)-17 are involved in many autoimmune diseases. Recently, IL-17/interferon-γ (IFN-γ) double-positive Th17 cells were found to be allied with inflammatory diseases. P-glycoprotein (P-gp) on lymphocyte effluxes out steroid and prevents its action. We conducted this study with a hypothesis that P-gp-positive IL-17/IFN-γ double-positive Th17 cells are liable for steroid resistance in nephrotic syndrome.

Objective: We planned to study the frequency of P-gp-expressing pathogenic Th17 cells in steroid-sensitive nephrotic syndrome (SSNS) and steroid-resistant nephrotic syndrome (SRNS) patients.

Methods: We analyzed the frequency of pathogenic IL-17/IFN-γ double-positive Th17 lymphocytes and P-gp expression on their surface by flowcytometry in SSNS (n = 32; mean age: 9.06 ± 5.84) and SRNS (n = 28; mean age: 11.29 ± 3.73) patients. We also included 15 age- and sex-matched healthy controls. All patients were of biopsy-proven minimal change disease and all patients were treated with steroids. All patients were recruited as per the criteria of ISKDC.

Results: We found a significant increase in the frequency of Th1 (P = 0.01), Th17 (P = 0.006), and IL-17/IFN-γ double-positive Th17 (P < 0.001) cells in SRNS as compared to SSNS patients and healthy controls (P < 0.001). Of the total Th1, Th17, and pathogenic Th17, 78.45%, 72.37%, and 95.8% of cells expressed P-gp on their surface in SRNS; however, 45.0%, 30.27%, and 30.1% of cells expressed P-gp in SSNS group and 30.91%, 15.51%, and 15.62% in healthy controls, respectively.

Conclusions: Higher frequency of IL-17/IFN-γ double-positive Th17 cells with high P-gp expression may be associated with steroid resistance in nephrotic syndrome patients.


  PB0005: Tacrolimus may regulate the pathogenic Th17 cells in lupus nephritis refractory patients Top


Anamika Anuja, Mohit Kumar Rai, Durga Prasanna Misra, Vikas Agarwal; Department of Clinical Immunology, SGPGIMS, Lucknow, Uttar Pradesh, India

Background: About 15%–30% of lupus nephritis (LN) patients do not respond to first-line immunosuppressive therapy. Higher expression of P-glycoprotein (P-gp)-mediated efflux of corticosteroids may contribute to the treatment unresponsiveness. Rise in population pathogenic Th-17 population LN regulates the response to the therapy in LN. In the previous study, we observed that refractory LN patients with tacrolimus had better clinical response. In this in vitro study, we had evaluated the effect of tacrolimus on pathogenic Th-17 population in refractory LN patients.

Methods: Ten LN patients (mean age: 34.06 ± 10.84 years) who were refractory to the cyclophosphamide treatment were recruited. Their peripheral blood was collected in heparinized vial and stimulated with phorbol myristate acetate and ionomycin and co-cultured with or without tacrolimus for 24 h. Pathogenic Th-17 (CD4 + interleukin-17 + interferon-γ) population was analyzed using flow cytometer.

Results: The frequency of Th-17 cells was higher in patients without tacrolimus (5.5% ± 1.1%) compared to those under tacrolimus treatment (4.24% ± 1.3%) (P = 0.006). The frequency of pathogenic Th17 cells was relatively higher in patients without tacrolimus (1.75% ± 0.14%) compared to those with tacrolimus (0.53% ± 0.16%) (P = 0.004). P-gp expression on pathogenic Th-17 cells without tacrolimus (90.5% ± 5.67%) compared to those with tacrolimus reduces the P-gp on pathogenic Th-17 cells (67.53% ± 8.89%).

Conclusion: Tacrolimus significantly reduced the frequency of pathogenic Th17 cells and the expression of P-pg on pathogenic Th-17 cells.


  OPB0026: Interleukin-17/interferon-γ double-positive Th17 cells selectively express P-glycoprotein and are refractory to glucocorticoids Top


Akhilesh Jaiswal, Narayan Prasad, Mantabya Singh, Mohit Rai1, Harshit Singh1, Saurabh Chaturvedi1, Vikas Agarwal1; Departments of Nephrology and 1Clinical Immunology, SGPGIMS, Lucknow, Uttar Pradesh, India

Background: Th17 cells and cytokine interleukin (IL)-17 are involved in many autoimmune diseases. Recently, IL-17/interferon (IFN)-γ double-positive Th17 cells were found to be allied with inflammatory diseases. P-glycoprotein (P-gp) on lymphocyte effluxes out steroid and prevents its action. We conducted this study with a hypothesis that P-gp-positive IL-17/IFN-γ double-positive Th17 cells are liable for steroid resistance in nephrotic syndrome.

Objective: We planned to study the frequency of P-gp-expressing pathogenic Th17 cells in steroid-sensitive nephrotic syndrome (SSNS) and steroid-resistant nephrotic syndrome (SRNS) patients.

Methods: We analyzed the frequency of pathogenic IL-17/IFN-γ double-positive Th17 lymphocytes and P-gp expression on their surface by flow cytometry in SSNS (n = 32; mean age: 9.06 ± 5.84) and SRNS (n = 28; mean age: 11.29 ± 3.73) patients. We also included 15 age- and sex-matched healthy controls. All patients were of biopsy-proven minimal change disease and all patients were treated with steroids. All patients were recruited as per the criteria of ISKDC.

Results: We found a significant increase in the frequency of Th1 (P = 0.01), Th17 (P = 0.006), and IL-17/IFN-γ double-positive Th17 (P < 0.001) cells in SRNS as compared to SSNS patients and healthy controls (P < 0.001). Of the Th1, Th17, and pathogenic Th17, 78.45%, 72.37%, and 95.8% cells expressed P-gp on their surface in SRNS; however, 45.0%, 30.27%, and 30.1% cells expressed P-gp in SSNS group and 30.91%, 15.51%, and 15.62%, in healthy controls, respectively.

Conclusions: Higher frequency of IL-17/IFN-γ double-positive Th17 cells with high P-gp expression may be associated with steroid resistance in nephrotic syndrome patients.


  OPB0020: Therapeutic effectiveness of black tea in regulating the bio-molecules of microvascular dysfunction and fibrosis in patients with diffused systemic sclerosis: An ex vivo study Top


Dipanjan Bhattacharjee, Sanchaita Misra, Ayindrila saha, Sudipta Chatterjee, Alakendu Ghosh; Department of Rheumatology, Institute of Postgraduate Medical Education and Research/SSKM Hospital, Kolkata, West Bengal, India

Background: Microvascular dysfunction and uncontrolled fibrosis are the major clinical challenges in systemic sclerosis (SSc). Dysregulation of vasodilator (nitric oxide [NO]) causes vascular damage triggering cytokine release (transforming growth factor-β [TGF-β], IL6, etc.). Subsequent uncontrolled fibroblast activation leads to tissue fibrosis. Phytochemicals are being studied for their therapeutic effectiveness, primarily for lower toxicity. Black tea (Camellia sinensis), a globally consumed beverage, is reported to have anti-oxidant and anti-inflammatory effects in in vitro models.

Methods: Fifteen consecutive diffused-SSc (dSSc) patients (ACR 2013) and ten age- and sex-matched healthy controls were recruited in the study. Blood samples were collected from all participants. Serum TGF-β and interleukin (IL)-6 were evaluated by enzyme-linked immunosorbent assay and nitrite levels biochemically. Peripheral blood mononuclear cells (PBMCs) were isolated, cultured in vitro, and treated with 250 and 500 ng/ml (IC25 and IC50, respectively) of black tea extract (Sigma-Aldrich). Transcriptional expression of collagen I was measured from the RNA extracted from the PBMCs by quantitative polymerase chain reaction. Extracellular NO, TGF-β, and IL-6 were measured from the cell supernatants 24 h posttreatment.

Objective: (1) Evaluation of the regulators of endothelial dysfunction, inflammation, and fibrosis in patients with dSSc and (2) Therapeutic effectiveness of black tea extract on the regulators of endothelial dysfunction, inflammation, and fibrosis in ex vivo system.

Results: Serum IL-6 and TGF-β (P < 0.0001 and P = 0.0009, respectively) were significantly higher in the patient group, whereas the serum NO was significantly lower (P < 0.0001) compared to that of controls. Treatment with black tea extract (IC25 and IC50) effectively increased the NO production (P < 0.0001) and significantly downregulated the level of IL-6 (P = 0.008), TGF-β (P < 0.0001), and transcriptional expression of collagen I (P = 0.005) with respect to the baseline. IC50 dosage of black tea (500 ng/ml) was found to be more effective in this regulation.

Conclusion: Effective downregulation of pro-inflammatory and pro-fibrotic molecules and upregulation of NO by black tea extract indicate its effectiveness in ex vivo system. Hence, it is encouraging to validate this finding in in vivo models.


  PB0008: Association of IL-6 -174 G/C and TNF-α 308 G/A promoter genes polymorphism with the susceptibility of Takayasu Arteritis in North Indian population Top


Abhishek Zanwar, Sandeep Kumar, Reena kumari, Avinash Jain, Ramnth Misra; Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Introduction: Takayasu arteritis (TaK) is a large-vessel vasculitis of unknown etiology that involves the aorta and its branches. Restriction to particular geographic area suggests genetic predisposition, environmental factor, or interplay of both. Many cytokines have been implicated in the pathogenesis of TaK. Hence, we planned to study polymorphism at the promoter of cytokine genes tumor necrosis factor-alpha (TNF-α), interleukin (IL)-4, IL-6, and IL-10.

Methods: In the present study, we have recruited 251 individuals, including 81 patients with TA on the basis of ACR 1990 criteria and 170 healthy controls that comprised the control group. All samples were genotyped for single-nucleotide polymorphisms in all genes using TaqMan genotyping assay in real-time-polymerase chain reaction. A case–control association study was performed.

Results: We included 81 cases of TaK with a mean age of 30.63 ± 10.76 years (range: 14–60 years) and 170 age-matched healthy controls. Allelic and genotypic frequencies did not deviate from Hardy–Weinberg equilibrium in the controls (P > 0.05). On allele contrast, significant association with susceptibility to TaK was detected with polymorphisms in IL-6 174 G/C and TNF-α 308 G/A genes. In case of IL-6 174 G/C gene polymorphism, variant genotype (GC + CC) versus wild-type genotype (GG) was found significantly higher in cases as compared to controls (P = 0.029 [odds ratio] OR = 1.87: 95% confidence interval [CI] = 1.09–3.20) and variant allele (C) (P = 0.005; OR = 1.84; 95% CI = 1.21–2.80), and also in case of TNF-a 308 G/A polymorphism, variant genotype (GA + AA) versus wild-type genotype (GG) was found significantly higher in cases as compared to controls (P = 0.025; OR = 2.23; 95% CI = 1.15–4.34) and variant allele (A) (P = 0.013; OR = 2.19; 95% CI = 1.20–3.97). Those with mutant TNF-a 308 polymorphism had higher disease activity when compared to those with wild type (P < 0.001).

Conclusion: Single-nucleotide polymorphisms in TNF-α 308 G/A and IL-6 174 G/C genes are associated with the susceptibility to TaK in North Indian population. Those with mutant TNF-α 308 polymorphism have higher disease activity.


  OPB0022: Cell-free DNA as an alternative predictor of disease activity in a specific subset of lupus nephritis Top


Sulagna Chatterjee, Sudipta Chatterjee, Nitaipada Bhattacharya, Dipendranath Ghosh, Alakendu Ghosh; Department of Rheumatology, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, India

Background: Systemic lupus erythematous (SLE) involves immune complex deposition in kidneys, which forms the pathogenesis of lupus nephritis. It is linked to cell death and insufficient removal of apoptotic materials. Cell death leads to the release of autoantigen and nucleic acids (circulating DNA) into the circulation, which promotes autoimmunity. Hence, the presence of released cell-free DNA (cfDNA) from the dead cells might be linked to the disease activity.

Objective: The study objectives included (1) comparative quantification of peripheral cfDNA in patients with lupus nephritis and healthy controls and

(2) studying the association between cfDNA and markers of disease activity in patients with lupus nephritis.

Methods: Thirteen patients with lupus nephritis (Class III and Class IV) and ten healthy controls (age and sex matched) were recruited. cfDNA was isolated from the plasma (isolated from peripheral blood) of individual samples using commercial kit. Concentrations of dsDNA were evaluated by enzyme-linked immunosorbent assay and C3 and C4 by nephelometry for all participants. Isolated cfDNA was quantified spectrophotometrically and validated by polymerase chain reaction. Systemic Lupus Erythematous Disease Activity Index (SLEDAI) (2K) was calculated.

Results: Plasma level of cfDNA was significantly higher (P < 0.001) in patient group compared to that of controls. Significant (P = 0.04) negative correlation was found between cfDNA and dsDNA (r = −0.6). It was observed that cfDNA negatively correlated with C3 (r = −0.6) and C4 (r = −0.3) in patients having low dsDNA level but with high disease activity (SLEDAI score ≥6).

Conclusion: Increased cfDNA in patients indicates that cfDNA might have a role in the pathogenesis of lupus nephritis. Negative correlations of cfDNA with dsDNA, C3, and C4 in a subset of patients with high disease activity, but low dsDNA level, could predict that cfDNA would serve as an alternative predictor for disease activity in lupus nephritis.


  PB0011: Spondylodiskitis in ankylosing spondylitis: Andersson lesion Top


Karthik Urala H A, Shiva Prasad BN; Apollo BGS hospital , Mysore, Karnataka, India

Case Report: A middle-aged male with ankylosing spondylitis (AS) for 15 years on nonsteroidal anti-inflammatory drugs and sulfasalazine (2 g/day) presented with sub-acute-onset pain over the mid-back region. On examination, he had kyphosis with restricted neck movement due to pain and tenderness over the mid-thoracic region. Erythrocyte sedimentation rate was 8 mm/1st h and C-reactive protein was 2.35 mg/dL. Magnetic resonance imaging (MRI) spine showed ossification of anterior longitudinal ligament, shiny corners of lower dorsal and lumbar vertebrae, and healed inflammatory changes of bilateral sacroiliac joints with D10–D11 disc T2 hyperintensity with abnormal signal in adjacent vertebral corpus with no paraspinal collection suggestive of noninfective spondylodiscitis with Andersson lesion (AL) with AS. Mantoux test and tuberculosis quantiferon tests were negative. Chest X-ray was normal. He was started with eternacept injection, 50 mg subcutaneously, once weekly. After 4 weeks of starting treatment, the patient showed significant improvement in pain and mobility.

Discussion: AS is an inflammatory disorder that primarily affects the axial skeleton, predominantly affecting males. AL is a well-known complication in patients with AS, characterized by the development of localized vertebral or discovertebral lesions of the spine. It is an aseptic spondylodiskitis consisting of inflammatory changes involving the diskus and adjacent vertebral endplates. AL may result from inflammation or (stress) fractures of the complete ankylosed spine. MRI is considered as the best modality in visualizing AL with the highest sensitivity. Generally, reduced signal intensity of the disc space and surrounding vertebral bodies and increased signal intensity after enhancement with contrast medium are noticeable on T1-weighted images. There is no evidence for an infectious origin without an indication for biopsy.

Conclusion: In long-standing AS patients presenting with mid-thoracic pain, AL should be thought of after ruling out secondary infections.


  PB0012: Neutrophilic CD64 helps in diagnosis of infection irrespective of organ involvement Top


Sushma Singh, Harshit Singh, Saurabh chaturvedi, Kritika Singh, Ranjeet Singh, Manjunath Hatti, Ravi mishra, Vikas Agarwal; SGPGIMS, Lucknow, Uttar Pradesh, India

Background: Bacterial and opportunistic infections are the major causes of mortality. Such patients may present initially with the signs and symptoms of nonspecific inflammatory process. Neutrophilic CD64 expression rapidly increases as physiological response to microbial components, complement products, and cytokines, which occurs within 4–6 h.

Aim: This study aimed to evaluate CD64 expression in patients who were admitted with liver disease, renal manifestation, autoimmune diseases, and pulmonary dysfunction.

Materials and Methods: Fifteen (10 months, aged 49.25 ± 13.58 years) patients with liver disease, ten (8 months, aged 39.15 ± 14.58 years) with renal manifestation, 15 (6 months, aged 45.25 ± 16.58 years) with auto-immune diseases, and 18 (12 months, aged 41.21 ± 12.58 years) patients who had pulmonary dysfunction were recruited in the study. Out of the 18 patients with pulmonary dysfunction, 10 were from pulmonary intensive care unit (ICU) and were followed up and their CD64 expression was evaluated on baseline, 4th day, and 8th day.

CD64 expression was analyzed via flow cytometry using PE-conjugated anti-human CD-64 antibody.

Results: Out of the 15 patients with liver disease, 10 were diagnosed with active bacterial infection (median CD64 expression – 86.46% [75.23%–99.3%]) and 5 had active liver disease (16.24% [12.31%–23.15%]); similarly, out of 10 patients, 6 were diagnosed with infection (90.15% [81.14%–99.3%]) and 4 had active renal disease (18.24% [13.98%–25.12%]). Out of the 15 patients with autoimmune disease, 7 had active bacterial infection (91.02% [80.25%–98.3%]), 4 had systemic lupus erythematosus (21.56% [14.25%–28.56%]), and 4 had rheumatoid arthritis (17.56% [13.96%–25.2%]).

Out of 18 patients from pulmonary medicine department, 10 were on ICU and were followed up (CD64 expression at baseline: 85.14 ± 10.25, at 4th day: 65.15 ± 12.35, and at 8th day: 30.25 ± 12.8); till the last follow-up, one patient was lost due to death and three were diagnosed with sarcoidosis (median CD64 expression 16.56% [12.96%–24.2%]), and five were with asthma (15.56% [14.96%–21.2%]).

Conclusion: Neutrophilic CD64 expression may be a better and early diagnostic tool in the detection of active bacterial infection.


  PB0013: P-glycoprotein and multidrug resistance-associated protein-1 on different T-cell subsets in idiopathic nephrotic syndrome in children Top


Ranjeet Singh Harshit Singh1, Narayan Prasad Saurabh Chaturvedi1, Akhilesh Jaiswal, Vikas Agarwal1; Departments of Nephrology and 1Clinical Immunology, SGPGIMS, Lucknow, Uttar Pradesh, India

Background: Idiopathic nephrotic syndrome (INS) represents one of the most common types of primary glomerular disease in children. INS has been considered a T-cell disorder; glucocorticoids remain the mainstay of therapy. However, 60%–80% of patients become resistant to steroids. The overexpression of P-glycoprotein (P-gp) and multidrug resistance-associated protein 1 (MRP-1) might be responsible for steroid resistance due to their ability to modulate the pharmacokinetics of steroids.

Aim: This study aimed to evaluate the role of P-gp and MRP-1 on CD4+ and CD8+ T-cell subsets in steroid-resistant as well as steroid-sensitive patients.

Methods: P-gp and MRP-1 expression was evaluated on blood and functional activity on peripheral blood mononuclear cells in steroid-sensitive nephrotic syndrome (SSNS) (n = 80, male 33, mean age = 8.54 ± 4.3) and steroid-resistant nephrotic syndrome (SRNS) (n = 50, male 29, mean age = 7.43 ± 4.6) patients. P-gp and MRP-1 expression was analyzed by flow cytometry. The absolute values were calculated using the following formula: (% of positive cells × relative fluorescent intensity), and multiresistance activity factor (MAF) for each transporter was calculated using the following formula: MAFMDR1 = 100× (FMDR1 − F0)/FMDR1.

Results: Among 130 patients, demographic significant difference was observed in serum albumin (SSNS = 2.87 ± 0.98 and SRNS = 2.27 ± 0.79, P = 0.012) and proteinuria (SSNS = 13.18 ± 3.09 and SRNS = 284 ± 193.45, P < 0.001).

The percentage of P-gp- and MRP-1-positive cells was significantly higher in SRNS as compared to SSNS (11.07 ± 5.23 vs. 5.70 ± 2.97, P < 0.001; 17.12 ± 7.10 vs. 7.15 ± 3.83, P < 0.001). Absolute P-gp and MRP-1 expression was significantly high in SRNS (63.01 ± 21.01 vs. 33.51 ± 20.30, P < 0.005; 67.04 ± 22.40 vs. 40.19 ± 19.17, P < 0.005, respectively). P-gp expression on CD4+ and CD8+ cells was significantly high in SRNS (6.18 ± 2.34 vs. 3.21 ± 1.06, P = 0.008; 6.42 ± 1.09 vs. 1.96 ± 0.91, P < 0.001, respectively). MRP-1 expression on CD4+ and CD8+ cells was significantly higher in SRNS (11.14 ± 5.33 vs. 3.16 ± 1.31, P = 0.043; 5.06 ± 0.89 vs. 1.40 ± 0.93, P < 0.001, respectively). The functional activity of P-gp and MRP-1 was significantly increased in SRNS as compared to SSNS (48.10 ± 20.10 vs. 91.94 ± 34.07, P < 0.001; 86.19 ± 31.72 vs. 51.10 ± 32.83, P < 0.001, respectively).

Conclusion: We conclude that overexpression of P-gp and MRP-1 on CD4+ and CD8+ cells may contribute to resistance to corticosteroids in idiopathic nephrotic syndrome in children.


  PB0014: Skewed activation of T-helper subtypes during systemic lupus erythematosus disease flare Top


Akash Protim Gogoi, Shashi Baruah, Sanjeeb Kakati1; Department of Molecular Biology and Biotechnology, Tezpur University, Tezpur, 1Department of Medicine, Assam Medical College and Hospital, Dibrugarh, Assam, India

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease with a wide range of autoreactive antibodies and T-cells, which cause tissue inflammation and organ damage. T-helper (Th) cells play a crucial role in mediating and regulating the immune response. Dysregulated Th cell functions have been reported in SLE.

Objective: To investigate the change in Th subtype specific cytokine and transcription factor expression during flare and remission state of SLE.

Methods: CD4+ T-cells were isolated from patients with SLE during flare and subsequent remission state. Th cell subtype-specific cytokine and transcription factor mRNA expression study was carried out using real-time polymerase chain reaction.

Results: mRNA expression levels of interferon-γ (IFN-γ) (2.47 ± 0.63* and 2.06 ± 0.37#), T-bet (4.12* ± 1.78 and 5.35 ± 1.21#) and interleukin (IL)-17 (3.78 ± 1.03* and 2.61 ± 0.61#) were up-regulated both in flare* and remission#. While GATA3 (2.35 ± 0.82) and FoxP3 (1.60 ± 0.29) were found to be up regulated only during remission. IL-4, GATA3 and FoxP3 mRNA expressions were elevated during remission in comparison to flare. An overall positive correlation between IFN-γ and T-bet (r = 0.63, P = 0.015) was observed. IL-17 and related orphan receptors gamma (r = 0.55, P = 0.082) were positively correlated during flare and at the same time, both showed a positive correlation with IFN-γ and T-bet. While a negative correlation of FoxP3 and RORγ were observed (r = −0.714, P = 0.058) during remission. The ratios of IFN-γ: IL-4 (P = 0.015), T-bet: GATA3 (P = 0.015), IL17:FoxP3 (P = 0.062) and RORγ: FoxP3 (P = 0.062) notably decreased from flare to remission.

Conclusion: The cytokine markers of inflammatory Th1 and Th17 specific cytokine markers were upregulated in SLE disease. During flare, there was higher activation Th1 and Th17 cells as compared to the anti-inflammatory Th2 and suppressor Treg cells, thus unable to turn down the inflammation.


  PB0015: Alterations of monocyte cytokines in systemic lupus erythematosus flare Top


Debashree Talukdar, Shashi Baruah, Sanjeeb Kakati1; Department of Molecular Biology and Biotechnology, Tezpur University, Tezpur, 1Department of Medicine, Assam Medical College and Hospital, Dibrugarh, Assam, India

Background: Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease characterized by unexpected events of disease exacerbations; flare followed by periods of low disease activity and remission. Monocytes/macrophages are the components of innate immune system, and aberrations of these cells have been reported in SLE pathogenesis.

Objective: The objective was to study the change of cytokines secreted by monocytes in SLE flare.

Methods: Peripheral blood monocytes were isolated from SLE patients during flare and subsequent remission by using CD14+ cell separation kit. mRNA quantification of the monocyte secreted cytokines; interleukin (IL)-1β, IL-12, tumor necrosis factor-α (TNF-α) (pro-inflammatory) IL-6 (pro/anti-inflammatory) IL-10 and transforming growth factor-β1 (TGF-β1) (anti-inflammatory) was performed using real-time-polymerase chain reaction.

Results: IL-1β (1.64 ± 0.62), IL-6 (6.35 ± 2.51), and TNF-α (3.49 ± 1.89) mRNA expression levels were upregulated during flare and subsequently reduced in remission. The expression level of IL-10 was upregulated both in flare (3.18 ± 0.64) and remission (1.27 ± 0.36) although the level of the cytokine was markedly higher in the patients during flare as compared to during remission (P = 0.016). IL-12 expression level was downregulated in flare (0.12 ± 0.03) which increased and became approximately equal to the normal level in remission (0.95 ± 0.01, P = 0.008). A similar change was also observed in case of TGF-β1 expression (0.26 ± 0.05 and 0.94 ± 0.31; P = 0.054). IL-10 showed negative correlation with C-reactive protein in flare (r = −0.905, P = 0.005) and an overall positive correlation with anti-dsDNA antibody titer (r = 0.498, P = 0.030).

Conclusion: Monocyte cytokines with inflammatory properties (IL-1β, IL-6, and TNF-α) were upregulated, while anti-inflammatory TGF-β1 was downregulated in flare. Aberrant expressions of IL-12 and IL-10 by monocytes were observed in the SLE patients.


  OPB0029: Urinary monocyte chemoattractant protein 1 does not differentiate between histological classes of lupus nephritis: A pilot study Top


Varun Dhir, Ranjeeth Gone, Amit Sandhu, Aman Sharma, Manish Rathi, Rithambhra Nada, Shefali Sharma; Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: Current treatment guidelines for lupus nephritis prioritize the histological class, which requires undergoing a renal biopsy. Monocyte chemo-attractant protein-1 (MCP-1), a chemokine produced locally during active nephritis, has been found to be promising for differentiating active from inactive disease and may vary across histological classes.

Objectives: The study objective was to compare urinary MCP-1 (uMCP1) levels between different histological classes of lupus nephritis.

Methods: This was a case–control study that was conducted at a tertiary care center in North India. “Cases” were defined as patients with systemic lupus erythematosus (SLE) satisfying criteria for active nephritis (proteinuria >1 g and/or active sediments – hematuria or cellular casts) and undergoing kidney biopsy. Controls were patients of SLE without active nephritis (<500 mg proteinuria and no active sediments). uMCP1 measurement was done using sandwich enzyme-linked immunosorbent assay kit. This was normalized for urinary creatinine excretion (pg/mg).

Results: Mean ages of cases (n = 36) and controls (n = 26) were 31.1 ± 10.2 and 34.6 ± 8.0 years, respectively (P = 0.3). uMCP-1 was significantly higher in cases than controls (1214 ± 1467.1 vs. 184.5 ± 186.8 pg/mg, P < 0.001). uMCP-1 levels showed significant correlation with 24-h proteinuria, 24-h protein-creatinine ratio (PCR), spot PCR, and Systemic Lupus Erythematosus Disease Activity Index. A cutoff of 339 pg/mg on receiver operating characteristics had sensitivity and specificity of 80% and 92%, respectively, for differentiating active from inactive nephritis. However, there was significant difference of uMCP-1 levels neither between different classes of nephritis (Class II, n = 2; Class III, n = 8; Class IV, n = 16; and Class V, n = 5) (P = 0.593) [Figure 1] nor when comparing proliferative (Class III or IV) to nonproliferative (Class II or V) (P = 0.7). Furthermore, uMCP1 levels did not show significant correlation with Renal Activity Index (r = −0.2, P = 0.3).
Figure 1

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Conclusion: uMCP1 was an indicator of active nephritis but was not significantly different in different histological classes nor did correlate with Renal Activity Index. However, this study had limited patients with class other than Class III or IV.


  OPB0030: Myeloid-related proteins 8/14 as disease activity and theragnostic biomarkers in axial spondyloarthritis Top


Arvind Ganapati, Jayakanthan Kabeerdoss, Ruchika Goel, Mahasampath Gowri1, Antonisamy B1, Debashish Danda; Departments of Clinical Immunology and Rheumatology and 1Biostatistics, Christian Medical College Hospital, Vellore, Tamil Nadu, India

Background: The utility of myeloid-related proteins (MRPs) 8/14 in axial spondyloarthritis (AxSpA) as a biomarker of disease activity and predictor of treatment response (theragnostic) is controversial.

Objectives: The study objective lies in the evaluation of serum MRP 8/14 in AxSpA as (1) disease activity biomarker and (2) a predictor of outcome in axial SpA by the Assessment of Spondyloarthritis (ASAS)20 after 6 months of treatment with sulphasalazine and methotrexate.

Methods: Serum MRP 8/14 was assayed by enzyme-linked immunosorbent assay platforms (R and D Systems, USA) at baseline in 83 AxSpA patients satisfying ASAS 2009 criteria and 30 healthy age- and sex-matched controls. Repeat measurements were done at 3 months for 60 patients on treatment with methotrexate (10–25 mg/week) and sulfasalazine (2–3 g/day) combination plus on-demand nonsteroidal anti-inflammatory drugs (NSAIDs).

Results: Baseline median MRP 8/14 level in AxSpA patients was 3.00 μg/ml (1.64, 5.6) compared to 2.3 μg/ml (1.36, 4.65) in controls (P=0.2). Among the 83 patients with 136 disease activity assessment scores at baseline and 3 months posttherapy, median MRP 8/14 level in patients with active disease (n = 111) as defined by Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) ≥4 was 2.2 μg/ml (1.4, 5.2), as compared to 2 μg/ml (1.5, 3.8) (P=0.5) in inactive disease (BASDAI <4) (n = 25). Median baseline MRP 8/14 level in ASAS20 responders (n = 36) was 3.6 μg/ml (1.8, 5.9) as compared to nonresponders, i.e., 2.4 μg/ml (1.6, 6.4) (n = 35), 6 months posttherapy (P=0.4). Median r MRP 8/14 level in ASAS20 responders (n = 33) was −1 μg/ml (−2.9, 0.2) as compared to −0.2 μg/ml (−4.8, 1.0) for nonresponders (n = 27), 6 months posttherapy (P=0.5).

Conclusion: Serum MRP 8/14 did not serve as a disease activity or theragnostic marker in our cohort of AxSpA patients treated with combined disease-modifying antirheumatic drugs and on-demand NSAIDs.


  PB0020: Diverse synovial cell-derived microparticle profile in patients with inflammatory arthritis Top


Benita N R Michael, K G Chengappa, V S Negi; Department of Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

Introduction: Microparticles (MPs), a heterogeneous group of small extracellular vesicles released from cells during activation and apoptosis, are reported to be elevated during pathological conditions. Synovial fluid annexin V+ MPs and their subsets were profiled based on cell lineage-specific markers by multicolor flow cytometry and compared between patients with rheumatoid arthritis (RA), juvenile idiopathic arthritis (JIA), and osteoarthritis (OA) (disease controls).

Methods: Five ml of synovial fluid was collected from the knee joints of 18 disease-modifying antirheumatic drug (DMARD)-naïve patients with RA, 9 DMARD-naïve patients with JIA, and 16 patients with OA. Cell-free synovial samples were stained with annexin-V-APC and anti-CD45 APC H-7, anti-CD20 BV421, anti-CD14 PE-Cy7, anti-CD4 BB515, anti-CD8 PE-CF594, anti-CD66b PE, and Anti-CD61 BV510 antibodies (cell lineage markers) followed by multicolor flow cytometry analysis. Results are expressed as a mean ± standard deviation of percentage population.

Results: Synovial fluid annexin V+ MPs and their subsets in patients with RA, JIA, and OA were tested by Kruskal–Wallis test followed by Dunn's multiple comparisons test. Kruskal–Wallis test revealed a significant difference in the levels of synovial fluid annexin V+ MPs (P = 0.0009), leukocyte-derived MPs (P < 0.0001), Th cell-derived MPs (P = 0.0002), Tc cell-derived MPs (P = 0.0023), monocyte-derived MPs (P < 0.0001), platelet-derived MPs (P = 0.0459), and granulocyte-derived MPs (P < 0.0001) between RA, JIA, and OA. Dunn's multiple comparisons test revealed no significant difference in MP profiles between RA and JIA. Annexin V+ MPs and their major subsets were significantly elevated in RA and JIA compared to OA with a higher percentage in RA group.

Conclusions: Elevated levels of synovial fluid annexin V+ MPs and their subsets in patients with RA and JIA compared to OA suggest their important role in driving the pathogenesis of these autoimmune diseases, especially in the affected joints.


  OPC0060: A study of human leukocyte antigen-B alleles in patients of spondyloarthritis and their association with clinical patterns of arthritis Top


Jeet Patel, Ved Chaturvedi, Lalit Duggal, Monika Jain, Neeraj Jain, Bhandari Gurbir Singh, Mayank Gupta; Department of Clinical Immunology and Rheumatology, Sir Ganga Ram Hospital, New Delhi, India

Background: Spondyloarthritides (SpA) are a heterogeneous group of arthritis with varied etiological factors. A strong genetic association has been found for SpA.

Objectives: The study objectives were to find the role of human leukocyte antigen-B (HLA-B) alleles including HLA-B27 in SpAs and to find their association with disease patterns.

Method: A total of 100 SpA patients from northern part of India were included after taking consent. Assessment in SpondyloArthritis International Society classification criteria for axial/peripheral SpA, classification criteria for reactive arthritis (ReA), and ClASsification for Psoriatic ARthritis criteria for psoriatic arthritis (PSA) were used to classify patients. HLA-B genotyping was done by sequence-specific primer tray kit. HLA-B genotyping of 100 healthy kidney transplant donors was taken as controls.

Results: Out of the 100 patients, the number of males and females was 58 and 42, respectively. HLA-B27 was positive in 57% and negative in 43% of patients. No alleles other than HLA-B27 (P = 0.0001) were statistically significant in 100 patients. In HLA-B27-negative patients, HLA-B40 showed statistical significance (P = 0.0101), while HLA-B52 and HLA-B35 were the next most common alleles. In PSA, the association of HLA-B27 (P=0.034), HLA-B15 (P=0.034), and HLA-B13 (P=0.020) was statistically significant. In ReA, inflammatory bowel disease-related SPA, and SPA (ankylosing spondylitis), HLA-B27 was present in 25%, 42%, and 70.8% of patients, respectively. In isolated peripheral arthritis, HLA-B27 was present in 36% with statistically significant P = 0.0031) without other HLA-B allele association. HLA-B27 was associated with axial disease, facet joint arthropathy, and magnetic resonance imaging-proven active sacroiliitis (P = 0.0001). HLA-B27-positive patients had early age of onset, younger age at diagnosis, and higher Ankylosing Spondylitis Disease Activity scores compared to HLA-B27-negative patients. HLA-B15 (P = 0.0018) and HLA-B27 (P = 0.043) had a significant association with uveitis.

Conclusion: HLA-B 27 is still the most important HLA-B allele for diagnosis, prognosis and severity of disease. Apart from HLA-B27, this study showed significant association of HLA-B40 in HLA-B27 negative patients and association of HLA-B13 and 15 in PSA. HLA-B15 and HLA-B 27 were associated with uveitis.


  OPC0001: Hypertrophic pachymeningitis in systemic lupus erythematosus: an extremely rare presenting feature Top


Chanaveerappa Bammigatti, R Shrivarthan, Deepanjali Surendran; Department of Medicine, JIPMER, Puducherry, India

Case Report: A 16-year-old girl presented with low-grade fever for 2 months and skin lesions for 1 month. Five days prior to hospitalization, she had severe bilaterally predominant retro-orbital headache associated with vomiting. She also complained of pain and early-morning stiffness involving the small joints of the hand. On examination, she had mild pallor and erythematous rash over the bridge of the nose and ear lobes with a few purpuric lesions over the volar aspect of the forearm and deltoid region. Metacarpophalangeal and proximal interphalangeal joints of both hands were tender for palpation with no associated swelling. There was a 1 cm × 1 cm ulcer over the hard palate. Central nervous system examination revealed no abnormality. Fundus examination revealed bilateral papilledema. She had normocytic normochromic anemia, leukopenia, and elevated erythrocyte sedimentation rate. Computed tomography of the brain revealed diffuse cerebral edema, and magnetic resonance imaging showed diffuse pachymeningeal thickening and enhancement over the bilateral cerebral convexities and in the tentorium cerebelli. Cerebrospinal fluid analysis did not reveal any abnormalities. Antinuclear antibody was 4+ with positive anti Sm/RNP, anti-Sm and anti-proliferating cell nuclear antigen antibodies. The patient was given intravenous methylprednisolone pulse for 3 days followed by tapering doses of oral prednisolone along with azathioprine with dramatic response.

Discussion: Hypertrophic pachymeningitis is an uncommon condition characterized by diffuse thickening of the dura mater. Hypertrophic pachymeningitis in the absence of any inciting factors is labeled as primary or idiopathic. The common causes of hypertrophic pachymeningitis include tuberculosis, sarcoidosis, connective tissue diseases, vasculitis, and malignancy.

Hypertrophic pachymeningitis as a presenting manifestation of systemic lupus erythematosus (SLE) is extremely rare. Only three cases of hypertrophic pachymeningitis in association with SLE are reported in the English literature.

Conclusion: Hypertrophic pachymeningitis is an extremely rare presenting manifestation of SLE and it responds dramatically to immunosuppressive therapy.


  OC0002: Kawasaki disease is a common childhood vasculitis: An experience from a tertiary care referral center of North-East India Top


Dhrubajyoti Sharma, Sarada Mazumdar1, Akher Ali1, Shivangi Bora1, Anik Mazumdar1; Professor of Pediatrics and In-charge of Pediatric Rheumatology and Immunodeficiency Services of Gauhati Medical College and Hospital, 1Department of Pediatrics, Gauhati Medical College, Guwahati, Assam, India

Background: Kawasaki disease (KD) is the leading cause of vasculitis affecting medium vessels. However, the vast majority of children with KD in India are still not being diagnosed. There is a paucity of data on KD in Assam and other northeastern states.

Objective: This is a retrospective study to report our experience with a cohort of 37 patients with KD registered at our center over the last 15 months.

Method: This study was carried out at the Pediatric Rheumatology and Immunodeficiency unit, Gauhati Medical College, Guwahati, Assam, India. A total of 37 cases had been registered at our center from March 1, 2017 to June 30, 2018. Data were retrieved from medical records and analyzed. The diagnosis of KD was made according to revised criteria developed by the American Heart Association-2017.

Results: In our cohort, one-third of patients with KD (12/37) were clustered in the months of May–July with a nadir in the months of December–January. Four cases were atypical KD [Table 1]. Investigations carried out in our patients are highlighted in [Table 1]. Thirty-two patients (86%) had received treatment with intravenous immunoglobulin as initial treatment. Additionally, we used infliximab in six patients and glucocorticoids in two patients [Table 1]. Coronary artery aneurysm (CAA) was detected in ten patients (27%) in our cohort by transthoracic two-dimensional echocardiography. Thirty-two patients had completed a mean follow-up of 7.82 ± 5.2 months (250 patient-months). On follow-up, there was significant reduction of coronary artery diameters in patients with mild-to-moderate CAA, and no treatment-related adverse effects or serious infections were observed.
Table 1: Demographic, laboratory, and treatment parameter of patients

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Conclusion: KD is a common pediatric rheumatological illness at our center. Most cases were diagnosed in the months of May, June, and July with a nadir in the months of December and January. Total follow-up duration in our cohort was 250 patient-months.


  PC0003: Profile of ten cases with childhood systemic lupus erythematosus: A single-center experience Top


Dhrubajyoti Sharma, Sarada Mazumdar, Akher Ali, Shivangi Bora; *Asst. Professor of Pediatrics and In-charge of Pediatric Rheumatology and Immunodeficiency Services; **Post Graduate Trainees, Gauhati Medical College

Background: Childhood-onset systemic lupus erythematosus (cSLE) occurs in 10%–15% of all cases of systemic lupus erythematosus (SLE). cSLE in Assam and other northeastern states is still underreported.

Objective: This is a retrospective study to report our experience with a cohort of ten patients with cSLE registered at our center over the last 15 months.

Method: This study was carried out at the Pediatric Rheumatology and Immunodeficiency Unit, Gauhati Medical College, Guwahati, Assam, India. A total of ten cases had been registered at our center from March 1, 2017 to June 30, 2018. Data were retrieved from medical records and analyzed. The diagnosis of SLE was made according to the revised criteria developed by Systemic Lupus International Collaborating Clinic-2012.

Results: In our cohort, the median age at presentation was 12 years (range, 8–18 years) and median age at the onset of symptoms was 10.5 years (range, 8–16 years). The age, sex, presenting manifestations, complications, treatment, outcome, and follow-up are highlighted in [Table 1]. Lupus nephritis (LN) was present in 6/10 patients. However, renal biopsy could be performed only in one patient [patient number 9, [Table 1]]. There was two deaths in our cohort – one patient died at the first admission due to acute left ventricular failure as a result of myocarditis [patient number 1, [Table 1]] and another patient died after 3 months of follow-up due to severe infection [patient number 3, [Table 1]]. Seven patients in our cohort had completed a median of 4-month (range, 1–12 months) follow-up and all achieved remission with treatment.
Table 1: Profile of cases with systemic lupus erythematosus at our centre

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Conclusion: cSLE is one of the common pediatric rheumatological illnesses at our center. Six out of ten patients had LN. Total follow-up duration in our cohort was 32 patient-months.


  OPC0188: Clinical feature and outcome of acute exacerbation in CTD-ILD - a single centre study Top


Prasanta Padhan, Bhaskar Thakur, Pratima Singh; Departments of Rheumatology, Bio-statistics and Pulmonary Medicine, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India

Background: In idiopathic pulmonary fibrosis (IPF), acute exacerbation (AE) is an increasingly recognized entity and is perceived as a severe event with high mortality, but there is limited amount of clinical data on AE-interstitial lung disease (ILD) in non-IPF ILD such as connective tissue disease associated-ILD (CTD-ILD). The present study was conducted to provide the clinical features, prognosis, and outcome of patients with AE-CTD-ILD.

Methods: Between the years of 2012 and 2018, 105 consecutive patients with CTD with chronic ILD, defined as ILD which had been stable on immunosuppressive drugs for over 3 months, were included in the study. AE was defined using the criteria recently proposed by the IPF net, with slight modification for adaptation to CVD-IP6, and patients having CTD with AE needed to meet all the five criteria.

Results: Fifteen patients with a mean age of 45.8 ± 13.9 years out of 105 patients with CTD-ILD developed AE; among them, the most common subgroup (n = 5, 33%) constituted diffuse cutaneous systemic sclerosis. The mean duration between the diagnosis of ILD and AE (months) was 56.5 ± 38.0 with a mean follow-up duration 24 ± 18.1 months; the baseline PaO2 was 81.7 ± 8.1 and mean forced vital capacity was 57.9 ± 8.9. Among 15 patients with AE CTD-ILD, 5 (33%) patients died. Those who had significantly lower baseline PaO2 (mean ± standard deviation), 72.6 ± 3.4 versus 86.2 ± 5.3 (P = 0.002) had higher mortality. Again, those who had shorter duration (months) of disease between with the onset of ILD to AE had higher mortality, 40.4 ± 45.1 versus 64.6 ± 33.6 (P = 0.098).

Conclusions: In our study, majority of patients with AE CTD-ILD survived due to aggressive immunosuppression including the use of biologics. Those who had significantly lower baseline PaO2 and those who had shorter duration of disease between with the onset of ILD to AE had higher mortality.


  OPC0005: Rheumatological manifestations of X-linked agammaglobulinemia: Profile of 17 cases from a tertiary care center in North India Top


Aman Gupta, Rakesh Kumar Pilania, Deepti Suri, Anju Gupta, Amit Rawat, Surjit Singh; Allergy Immunology Unit, Advanced Pediatrics Department, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Background: X-linked agammaglobulinemia (XLA) is characterized by recurrent bacterial sinopulmonary infections. Arthritis can be seen in 10%–30% of patients with hypogammaglobulinemia and is usually a mono- or oligo-arthritis of large joints, but polyarthritis has also been defined.

Objectives: We describe our experience of managing children with XLA who presented with rheumatological manifestations.

Methods: Sixty-two children were diagnosed with XLA during the last 10 years at the Primary Immunodeficiency Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India. Seventeen children had rheumatological manifestations. A retrospective case review with respect to clinical presentation, musculoskeletal findings, and treatment was done.

Results: Rheumatological manifestations were seen in 27.4% of patients with XLA. Thirteen out of 17 patients had a proven mutation in Btk gene. The mean age at symptom onset was 3.3 years and mean age at diagnosis of XLA was 5.6 years. Rheumatological manifestations were seen at a mean age of 8.7 years. In two patients, arthritis preceded the diagnosis of XLA, while ten patients developed rheumatological manifestations after the diagnosis of XLA. Arthritis as an initial presentation of XLA was seen in five patients. Oligoarthritis was the most common presentation in 15 patients. Knee was the most commonly involved joint (11 patients), others being ankle, shoulder, wrist, hip, proximal interphalangeal joints of hands, sacroiliitis, and spondylodiscitis involving L4–L5 and L5–S1 vertebrae. Three patients manifested as septic arthritis. All patients received intravenous immunoglobulin (IVIg – 400 mg/kg) and co-trimoxazole prophylaxis. Arthritis showed clinical improvement after IVIg replacement in 12 patients. One patient expired, while four patients were lost to follow-up. None of the patients had recurrence of arthritis; however, radiological evidence of spondylodiscitis persisted in one patient.

Conclusions: Arthritis is a common manifestation in patients with hypogammaglobulinemia. Family history and a history of recurrent infections in a child with arthritis may provide a clue toward the diagnosis of primary immunodeficiency.


  PC0005: Assessment of the efficacy and safety of anti-interleukin-17A monoclonal antibody secukinumab in Indian patients with active ankylosing spondylitis and active psoriatic arthritis Top


Bindhyachal Kumar Gupta, Sundeep Upadhyaya, Rohini Handa, Sirinder J Gupta; Department of Rheumatology, Indraprastha Apollo Hospitals, Sarita Vihar, Delhi, India

Background: Anti-tumor necrosis factor (TNF) agents are currently the “standard of care” drugs. However, some patients did not respond to anti-TNFs. In addition, anti-TNFs have a high propensity to cause tuberculosis (TB). Secukinumab has been found to be effective in ankylosing spondylitis (AS) and psoriatic arthritis (PsA) but also advantage of lower risk of TB, vitals to the Indians.

Objective: The objective was to study the efficacy and safety of secukinumab in patients with active AS and PsA.

Methods: Adult patients who fulfill the Modified New York Criteria for AS and the ClASsification of Psoriatic ARthritis criteria for PsA and have active disease were given subcutaneous injection of secukinumab 150 mg at weeks 0, 1, 2, 3, and 4 and then once every 4 weeks till 6 months. All patients were biologic disease-modifying antirheumatic drug (DMARD) naïve and had inadequate response to nonsteroidal anti-inflammatory drugs and/or classic DMARDs for at least 3 months. The primary efficacy endpoint was Assessment in SpondyloArthritis International Society20 and American College of Rheumatology (ACR)20 at 12 weeks. Secondary endpoints were comparison of change from baseline in the C-reactive protein(CRP), Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI), Ankylosing Spondylitis Disease Activity Score (ASDAS)-CRP, and Disease Activity in PsA (DAPSA) at 12 and 24 weeks.

Results: A total of 28 patients were included in the study. Fourteen and twenty patients were analyzed for 6 months and 3 months, respectively. All patients were male, with a mean age of 33 years and a mean duration of 7.5 years; human leukocyte antigen-B27 was negative in three AS patients. Fifteen of 18 AS patients achieved Assessment in SpondyloArthritis International Society 20 (83.3%) and all the two PsA patients achieved ACR20 (100%). Changes in parameters were statistically significant for baseline and 3-month values as follows: CRP (23.8 ± 18.0 and 7.8 ± 8.2, P = 0.002), BASDAI (5.2 ± 1.3 and 1.9 ± 1.5, P < 0.001), ASDAS-CRP (3.9 ± 0.7 and 2.1 ± 1.1, P < 0.001), and BASFI (4.9 ± 2.8 and 1.7 ± 2.7, P = 0.001), respectively. Changes in parameters were statistically significant for baseline and 6-month values as follows: CRP (27.4 ± 20.6 and 7.8 ± 9.5, P = 0.008), BASDAI (4.9 ± 1.1 and 1.4 ± 1.3, P < 0.001), ASDAS-CRP (3.8 ± 0.7 and 1.8 ± 0.9, P < 0.001), and BASFI (4.5 ± 3.1 and 0.9 ± 2.4, P=0.005), respectively. Changes in the baseline DAPSA for the two patients of PsA at 3 and 6 months were 58.2/9.2/4.2 and 43.6/14.3/10.2. Two patients had fungal infection and one patient had new-onset inflammatory bowel disease.

Conclusion: It is an effective therapy with safety profiles in patients with active AS and PsA.


  PC0072: Temporal and secular trends in osteoarthritis in a community-based rheumatology practice – 2007–2017: Additional focus on phenotypic pattern Top


Bharat Veer Manchanda, Ravi Ghorpade, Anuradha Venugopalan, Arvind Chopra; Centre for Rheumatic Diseases (CRD), Pune, India (www.rheumatologyindia.org)

Introduction: Data on osteoarthritis (OA) in the Indian population and rheumatology practice are sparse. The prevalence of adjusted OA reported in a community survey (COPCORD) in Pune region was 4.01 in urban Pune and 6.25 in rural Bhigwan (J Rheumatol 2009; 36:3). The aim was to study the trends and pattern of OA in community rheumatology settings.

Methods: Data were extracted from a comprehensive referral database at the Center for Rheumatic Diseases, Pune, Maharashtra, India, maintained since 1996. This was a cross-sectional retrospective study with prospective follow-up. The study period was from January 2007 to December 2017. Diagnosis was clinical, and the dominant phenotype at the first examination was the basis of OA pattern.

Results: Among 43,913 patients who attended the clinic for the first visit, 21.6% of them had a clinical diagnosis of OA (male:female = 1:31) versus 31% patients of rheumatoid arthritis (RA) in the same period. Among the OA subsets, 94% had OA knees, 67% had spine affection, 19.1% had hand (distal interphalangeal usually), 18.7% had primary generalized OA, and 0.2% had OA hip. A total of 689 (7.2%) patients were in the age group of 25–44 years (male:female ratio was 1:25); 91% knees and 56% spine. Comorbidity was hypertension in 18% of patients, diabetes in 11.8%, and ischemic heart disease in 3.7%. The mean erythrocyte sedimentation rate (Westergren method) was 40.35 mm at the end of 1st hour, mean C-reactive protein (nephelometry) was 12.7 mg/l, and mean serum uric acid was 4.7 mg/dl. The mean annual proportion of OA during the initial 3 years of this decade was 23% as compared to 26% in the last 3 years; correspondingly, it was 30.2% and 34.4%, respectively, for RA.

Conclusion: This study has shown a substantial burden of OA in rheumatology practice though it is less than RA. The proportions seem to be sustained over the decade with ~3%–4% increase. It is of concern that a large number of young individuals also suffered from OA. A similar trend is also observed for RA.


 Brucellosis More Details in a rheumatology clinic of a tertiary hospital in North India">  OPC0008: Study of profile of patients diagnosed as osteoarticular brucellosis in a rheumatology clinic of a tertiary hospital in North India Top


B K Kundu; Department of Medicine, PGIMER, New Delhi, India

Background: Brucellosis is a worldwide zoonosis and endemic in the Mediterranean region, Gulf countries, Latin America, and the Indian subcontinent. Manifestations of brucellosis are myriad and hence remain under the radar of the clinician. Up to 47% of brucellosis patients may experience osteoarticular complications. It can take place at early stages of disease, at any time during the course of illness, or some features can be present at the onset of the disease. There is a lack of data in the Indian setting regarding osteoarticular brucellosis. Presently, characterization of patients of osteoarticular brucellosis has not yet been done in India.

Objectives: The objectives were to highlight the spectrum of clinical manifestations and laboratory parameters of cases diagnosed as osteoarticular brucellosis in rheumatology clinic.

Methods: Records of patients who have been diagnosed as osteoarticular brucellosis based on antibody detection by complement fixation test or by enzyme-linked immunosorbent assay were included in the study. All patients who have positive tests as mentioned above will be incorporated into the study.

Results: Patients of osteoarticular brucellosis are mostly young with no sex preponderance. They do not recall fever. Their presenting features mimic spondyloarthritis.

Conclusions: It is very difficult to distinguish osteoarticular brucellosis from spondyloarthritis. Apart from a high index of suspicion, few clinical and laboratory features may be helpful as indicators for further evaluation.


  OPC0009: Coexistence of Takayasu's arteritis with tuberculosis: the missing link Top


Samarasimha Reddy, Atul Kakar, Atul Gogia, S.P.Byotra; Ganga Ram Institute of Postgraduate Medical Education & Research, New Delhi, India

A 26-year-old female presented with nonhealing ulcer on the left arm for 6 weeks. She recalled that she was pinched by her colleague over the same area, following which she developed pain, swelling, and high-grade fever. She was diagnosed to have abscess for which antibiotics were initially started; however, as there was no response, surgical debridement was done. On admission, the patient was febrile with normal vitals and all palpable pulses. Local examination revealed ulcer over the left arm [Figure 1] on the lateral aspect of size 4 cm × 5 cm with pus discharge. Systemic examination was normal except bilateral conjunctival congestion. On investigations, the patient had mild anemia, mild leukocytosis, markedly elevated pro-inflammatory markers, and sterile cultures. Local debridement was done, and histopathology reported it as chronic inflammatory abscess. Ophthalmologist opinion revealed bilateral anterior uveitis. At this point, she received prednisolone eye drops and injectable antibiotics. In view of persistent fever, further workup was done which showed antinuclear antibody (IF) 2+, homogenous pattern with 1:100 titer, and Mantoux of 19 mm. Her viral markers were negative. Whole-body positron emission tomography scan was suggestive of large fluorodeoxyglucose-avid fibroconsolidative lesions in the lungs with large-to-medium vessel vasculitis. Bronchoalveolar lavage was positive for Mycobacterium tuberculosis (MTB) Xpert®. This patient had evidence of pulmonary nodules which were due to tuberculosis and typical radiological features of large-vessel vasculitis. She was started on anti-tuberculosis treatment, corticosteroids (intravenous + subtenon), and methotrexate. The latter was added due to persistent uveitis. After 6 weeks of follow-up, ulcer, eye symptoms, and fever were settled.

Discussion: MTB has been implicated in the pathogenesis of Takayasu's arteritis; however, in many cases, it is difficult to prove. Patients with Takayasu's arteritis have heightened immune response to MTB antigens, in particular to its 65 kDa heat shock protein.

Conclusion: This case shows the association of MTB with large-vessel vasculitis such as Takayasu's arteritis. The case demonstrates the missing link of etiology.


  PC0011: “Qualitative and quantitative assessment of nail-fold capillaries among healthy South Indian volunteers”: A pilot study Top


Sangeetha K.N, Sandra Sanil, Ramya J, Vineeta Shobha; St. Johns Medical College Hospital, Bengaluru, Karnataka, India

Background: Nail-fold capillaroscopy (NVC) is an important diagnostic tool and forms a part of classification criteria for primary Sjögren's syndrome, but little is known about the prevalence and distribution of NVC changes in healthy individuals.

Objectives: The objective was to assess the nail-fold capillaries using nail-fold videocapillaroscope among healthy volunteers in South India.

Methods: NVC was performed in fifty healthy adult volunteers (19 males and 31 females). The nail folds of eight fingers, except thumb, were assessed according to standard guidelines.

Results: In all the fifty individuals, capillaries in 1 mm2 area were analyzed and their average was noted. There was only one smoker and comorbidities included hypertension (1 patient) and hyperthyroidism (1 patient). The NVC characteristics are listed in [Table 1]. Abnormalities in capillary architecture, such as bifid capillaries, distortion, tortuosity, and disorganization, were frequently noted. Difficulties/pitfalls in the visualization and assessment of capillaries included pigments in dark-skinned individuals and unrecognized minor trauma.
Table 1: Parameter


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Conclusions: The usefulness of NVC lies in clinical correlation, as the various abnormalities are seen even in normal healthy individuals. Certain abnormalities such as capillary dropouts, giant capillaries, and neovascularization were not seen in our cohort and if found may indicate an underlying pathology.

[TAG:2]OC0007: Three-cohort Phase I study to evaluate the pharmacokinetics of GRC 27864 after single-dose oral administration of a tablet formulation (25 mg, 50 mg, and 75 mg) and relative bioavailability of the tablet formulation (75 mg) in comparison to the granule formulation of 75 mg[/TAG:2]

Barkate H, Tandon M1, Sant S2, Gudi G3, Talluri R4, Levine-Dolberg O5, Patil S6; Vice President, Medical Services, Glenmark Pharmaceuticals Ltd., 1Vice President and Head, Clinical Development – ROW, Glenmark Pharmaceuticals Ltd., 2Ex-General Manager, Clinical Development, Branded Generics, Glenmark Pharmaceuticals Ltd., Mumbai, India, 3Vice President, Drug Metabolism & Pharmacokinetics, Glenmark Pharmaceuticals Ltd., USA, 4Senior Principal Scientist - Drug Metabolism & Pharmacokinetics, Glenmark Pharmaceuticals Ltd. Navi Mumbai, India, 5Vice President & Global Head of Medical Affairs, Research & Development. Glenmark Pharmaceuticals Ltd., Watford, UK, 6Senior Manager, Medical Services, Glenmark Pharmaceuticals Ltd., Mumbai, India

GRC 27864 is a novel potent, selective, orally bioavailable inhibitor of mPGES-1. A single oral dose of GRC 27864 as granules in suspension, with doses up to 1000 mg, and multiple oral doses up to 130 mg/day for 28 days have earlier been studied. The primary objective was to assess the pharmacokinetic dose proportionality of GRC 27864 with the tablet formulation and the relative bioavailability of the tablet versus granule formulation of GRC 27864 after single-dose oral administration in healthy male adults.

Methods: (1) Cohort I (n = 12): Open-label, randomized (1:1), two-sequence, two-treatment, two-period crossover design with washout period of at least 3 weeks. (i) GRC 27864 granules 75 mg followed by GRC 27864 tablet 75 mg (1 × 75 mg tablet) and (ii) GRC 27864 tablet 75 mg (1 × 75 mg tablet) followed by GRC 27864 granules 75 mg; (2) Cohort II (n = 8): 50 mg dose of GRC 27864 (2 × 25 mg tablets); and (3) Cohort III (n = 8): 25 mg dose of GRC 27864 (1 × 25 mg tablet).

Results: Based on analysis of variance comparison of pharmacokinetics (PK) parameters of 75 mg of GRC 27864 tablet versus granule formulations, geometric mean ratios (GMRs) were 109.9, 108.6, and 109.0 for Cmax, area under curve (AUC) 0-last, and AUC0-∞, respectively. The 90% confidence intervals of GRC 27864 GMRs for tablet versus granules lay within the bioequivalence range [Figure 1]. With the tablet formulation, GRC 27864 Cmax increased in a slightly less than dose-proportional manner, while a trend for a close to dose-proportional increase was observed for AUC [Figure 2]. All doses and formulations were found to be safe and well tolerated.
Figure 1: Geometric mean ratios and 90% confidence intervals of GRC 27864 Cmax and area under curves for the relative bioavailability analysis (n=12)

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Figure 2: Scatter plots for GRC 27864 Cmax and area under curves0-∞

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Conclusion: The tablet and granule formulations of 75 mg of GRC 27864 were found to be bioequivalent. With the tablet formulation, the PK profiles of GRC 27864 were almost linear, with a close to dose-proportional increase in exposures.


  OPC0013: Correlation between spot protein/creatinine ratio and 24 hour urinary protein excretion in childhood lupus nephritis Top


Manjari Agarwal, Sujata Sawhney; Sir Gangaram Hospital, New Delhi, India

Background: In children with lupus nephritis, 24-h estimation of urinary protein measurement is considered as gold standard. However, it is fraught with difficulties of collection. This study was undertaken to find out if a random urine spot protein/creatinine is reliable and can be interchanged with a 24-h urine protein estimation.

Objective: (1) To find out the correlation of spot protein/creatinine estimation and 24-h urine protein estimation and (2) to find the value of spot protein/creatinine ratio that best correlates with the 24-h protein value.

Methods: A total of 209 paired samples of spot protein/creatinine ratio and 24-h urine protein were retrospectively recorded from charts of 64 children with lupus nephritis. Analysis was done using SPSS software version16.1.

Results: Spot protein creatinine ratio showed a good correlation with 24-h urine protein values with P < 0.01. The correlation was poor for spot protein/creatinine ratio <0.5 (P = 0.5).

Using receiver operating characteristic curve, the cutoff value of spot protein creatinine ratio to predict nephrotic range proteinuria (>1 g) was 1.81 with area under curve 95% (0.875). 95% confidence interval (0.817–0.932), sensitivity 73.1%, specificity 90.8%, positive predictive value 0.79, and negative predictive value 0.87.

Conclusions: The spot protein/creatinine ratio in ranges >0.5 correlate well with 24-h protein values and can be reliably used in routine clinical practice.

References

  1. Christopher-Stine L, Petri M, Astor BC, Fine D. Urine protein-to-creatinine ratio is a reliable measure of proteinuria in lupus nephritis. J Rheumatol 2004;31:1557-9.
  2. Lane C, Brown M, Dunsmuir W, Kelly J, Mangos G. Can spot urine protein/creatinine ratio replace 24 h urine protein in usual clinical nephrology? Nephrology (Carlton) 2006;11:245-9.



  OPC0014: Estimated glomerular filtration rate in a single centre childhood lupus nephritis cohort Top


Manjari Agarwal, Sujata Sawhney; Sir Gangaram Hospital, New Delhi, India

Introduction: Ongoing proteinuria causes long-term damage to the kidney. This is reflected by a change in estimated glomerular filtration rate (eGFR) over a period of time. A fall in eGFR is a poor marker and heralds damage.

Objectives: The objective was to estimate the eGFR by using the bedside Schwartz formula at the onset of nephritis, at 1 year after treatment, and at last follow-up.

Methods: Charts of 82 children with lupus nephritis were reviewed retrospectively. Height records at disease onset were available for 69 children and these were included for analysis. eGFR was calculated using the following bedside Schwartz formula: 0.413 × (height/creatinine).

Results: A total of 69 children with lupus nephritis were included. Median age at the onset of nephritis was 12 years. Median duration of follow-up was 55 months (range: 9–164 months). Median eGFR at the onset of nephritis prior to treatment was 82 ml/min/1.73 m2 (interquartile range [IQR]: 63–115). Median eGFR at 1 year after treatment was 127 ml/min/1.73 m2 (IQR: 89–150). eGFR at the last follow-up was 128 ml/min/m2 (IQR: 101–145). The change in eGFR from baseline to 1 year was statistically significant (P ≤ 0.001) and the change at the last follow-up visit from baseline was also statistically significant (P ≤ 0.001). A total of 39 children had eGFR <90 ml/min/m2 at the onset of nephritis and of these, 14 had eGFR <60 ml/min/m2. After 1 year of therapy, one had eGFR <60 ml/min/m2 and 18 had eGFR <90 ml/min/m2. This could imply that early aggressive treatment led to a significant improvement.

Conclusion: Stringent follow-up is practiced at our unit, and a tight treat to a target approach is employed. This is probably a reason for better outcomes. Another possibility is that many children have not spent a long time in the disease course and a longer follow-up is needed to determine the renal damage.


  OPC0015: Sleep disturbance and anxiety depression in rheumatoid arthritis and its correlation with disease activity Top


H. Singh, A. Kumar, J. Singh, M. Yadav, N. Gupta. S. Giri; Department of Medicine, Pt. BDS PGIMS, Rohtak, Haryana, India

Background and Objectives: Sleep and mood disturbances have been reported in rheumatoid arthritis (RA) with prevalence of 75% and 30%, respectively, but no data has been available in Indian population, especially regarding the impact of disease activity and its correlation with sleep and mood disturbances in RA.

Objective: The objective was to study the correlation of sleep disturbance and anxiety and depression with disease activity in RA.

Methods: A total of hundred patients of RA as per the American College of Rheumatology criteria (1987) were enrolled in the study. All patients were evaluated for disease activity (using Disease Activity Score [DAS] 28 and Clinical Disease Activity Index [CDAI]), sleep disturbance (using Pittsburgh Sleep Quality Index [PSQI]), and mood disturbance (using Hospital Anxiety and Depression Score [HADS]) at baseline and 4 months.

Result: The mean age was 43.07 ± 12.41 years, with 79 females and 21 males. At baseline and 4 months, DAS28 scores were 6.66 ± 0.86 and 3.50 ± 0.90; CDAI scores were 38.20 ± 11.43 and 8.53 ± 5.86; PSQI scores was 17.55 ± 2.819 and 3.94 ± 2.498; and HADS scores were 27.99 ± 4.382 and 4.41 ± 6.11, respectively. The Pearson's correlation coefficient of PSQI was found to be positively correlated with disease activity at baseline but negatively at 4 months, whereas Pearson's correlation coefficient for HADS with disease activity was found to be positively correlated at baseline and 4 months.

Conclusion: Based on the above results, disease activity is associated with mood disturbances in RA, whereas sleep disturbances did not follow the improvement trend of disease activity. It is suggested that RA patients with high disease activity should be assessed with HADS to delineate the associated mood disturbances.


  OPC0016: Tuberculosis in patients with rheumatic diseases and biological therapies: A single-center experience Top


Arun Gogna, Sameer Gulati, Anshul Goel; Vardhman Mahavir Medical College and associated Safdarjung Hospital, New Delhi, India

Introduction/Background: Increased use of biologicals has increased the risk of opportunistic infections, especially tuberculosis. The present study will add to our knowledge concerning the occurrence of tuberculosis rheumatology patients on biologicals.

Aims/Objectives: The aim of the study was to determine the incidence of tuberculosis in rheumatology patients on biologicals and its relation with the duration of therapy, type of biological, and the latent tuberculosis infection (LTBI) status.

Methods: This was a retrospective study conducted in the rheumatology clinic of Vardhman Mahavir Medical College/Safdarjung Hospital, New Delhi, India. The case records of the patients were reviewed to select those on biologicals for at least 1 year. The demographic data of patients, nature and duration of biologicals along with LTBI status, and occurrence of tuberculosis were noted.

Results: Thirty-eight patients with a mean age of 39.8 ± 16.4 years were included in the study. Twenty-seven and 11 patients were started on tumor necrosis factor inhibitors (TNFi) (infliximab-4, adalimumab-12, golimumab-8, and etanercept-3) and rituximab, respectively. Eleven patients were offered tuberculosis prophylaxis based on their LTBI workup. They were initiated on biologicals (infliximab-2, adalimumab-6, golimumab-2, and etanercept-1) after completion of 4 weeks of chemoprophylaxis. Four patients developed active tuberculosis (adalimumab-3 and infliximab-1), 5.5 ± 2.9 months after initiation of biologicals. There were two patients with pulmonary and two with extrapulmonary tuberculosis. Out of the four patients with active tuberculosis, three had received chemoprophylaxis for LTBI. The incidence of tuberculosis was 56 cases per 1000 patients being treated with biologicals per year.

Conclusions: Active tuberculosis may develop in rheumatology patients being treated by biologicals even after receiving tuberculosis chemoprophylaxis. All the four patients with active tuberculosis in our study were being treated by TNFi. These findings highlight that all patients on biologicals should be closely monitored for the development of active tuberculosis. Further research on the development of better algorithms for the diagnosis and management of LTBI is required at present.


  OPC0020: NA Top


Janhavee Jadhav

Background: Rheumatoid factor (RF) and anti-cyclic citrullinated protein (ACCP) estimation has been used to improve the diagnosis of rheumatoid arthritis (RA). However, their role in prognostication of RA, individually and in combination, is not well studied. This is especially true for Indian patients.

Methods: Sequential 945 patients who had their RF and ACCP determined were included in the study. They were followed up for 3–24 months. Swollen joint count, erythrocyte sedimentation rate, Disease Activity Score 28, and Indian version of Health Assessment Questionnaire (HAQ) were checked during each visit. They were treated with conventional disease-modifying agents (DMARDs).

Results: At presentation, patients with both antibodies positive had the most severe disease, while those with both antibodies negative had the least severe disease. Among discordant antibody status (one antibody positive and the other negative), patients with ACCP positivity presented with higher disease activity than with RF positivity. Patients with dual-antibody positivity were much less likely to be in remission than those with dual-antibody negativity. The percentage of patients in remission was 34.2, 29.5, 32.4, and 24.7, respectively, for RF−/ACCP−, ACCP+/RF−, ACCP−/RF+, and ACCP+/RF+, respectively. Both ACCP (odds ratio [OR]: 0.76; 95% confidence interval [CI]: 0.74–0.78) and RF (OR: 0.68; 95% CI: 0.66–0.70) positivity were associated with lower odds of sustained remission (P < 0.05).

Conclusion: Dual-antibody positive status at presentation carries poor prognosis, higher disease activity, lower HAQ, and lesser chance of remission in RA patients with conventional treatment. Patients with dual-antibody negative status had the best prognosis. Though patients with discordant antibody status had an intermediate prognosis, the ones with ACCP had poorer remission rates.


  OPC0126: Ulnar artery occlusion and hand arterial flow abnormalities correlate with the prevalence of digital pits in early scleroderma Top


Pratyusha Rajavarapu, R Narayanan, Liza Rajasekhar; Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background: Unlike microvascular involvement, macrovascular involvement in systemic sclerosis (SSc) is not extensively studied, especially in early SSc as it was considered as a rare and late manifestation.

Objectives:

  1. To assess forearm and hand arteries in early SSc using color Doppler ultrasound (CDUS)
  2. To correlate flow abnormalities with the prevalence of digital pits.


Methods: All consecutive patients satisfying the 2013 American College of Rheumatology/ European League Against Rheumatism criteria for SSc with duration of the first non-Raynaud's symptom <3 years attending rheumatology outpatient department were included in this observational study. Patients who received prostanoids or bosentan in the last 3 months and those with hypertension, diabetes, peripheral arterial disease, and smoking history were excluded from the study. Demographic data, disease duration, number of digital pits/ulcers, and modified Rodnan Skin Score (mRSS) were noted. CDUS of radial; ulnar; and radial and ulnar proper palmar digital arteries (PPDA) of thumb and index fingers was performed using a linear probe of 13–18 mHz, and normal or abnormal flow pattern was recorded. Approval was taken from the Institutional Ethics Committee.

Results: Patient and CDUS details are summarized in [Table 1]. Among 189 fingers with abnormal PPDA, 56 had digital pits (P = 0.0367). Median mRSS was 20 (18) in both diffuse and limited subgroups. Patients with abnormal flow pattern had a higher mRSS (P = 0.01). No significant association was found between abnormal flow pattern and pulmonary arterial hypertension (PAH) (P = 0.7) or interstitial lung disease (ILD) (P = 0.16). Ulnar artery occlusion (UAO) was seen in 17 (21.2%) patients and was bilateral in 6 patients. UAO was commoner with diffuse disease in 12 patients (70.5%) (P = 0.07). Digital pits were seen in 20/23 hands with UAO and 61/137 hands without UAO, which is statistically significant (P = 0.0002). No statistically significant association was found between UAO and mRSS, PAH, or ILD.



Conclusion: UAO and abnormal flow pattern in PPDA are demonstrable even in early SSc and correlates with the presence of digital pits and higher skin scores.


  OPC0022: Gastrointestinal manifestations in systemic lupus erythematosus: A retrospective study Top


Sanket Shah, Chengappa K.G.1, Pooja Belani, Shanoj KC, Manoj Khatri, Vir Singh Negi2; Senior Resident, 1Assistant Professor, 2Professor and Head of the Department, Department of Clinical immunology, JIPMER, Puducherry, India

Background: In Systemic Lupus Erythematosus (SLE) there is a wide variety of gastrointestinal (GI) manifestations and when it is present at the onset, there is a possibility of delayed diagnosis. It is important to identify the clinical and immunological markers of GI manifestation which may lead to an early diagnosis and improved outcome.

Objectives: To study the association of various clinical and immunological markers with GI manifestation in SLE.

Methods: In this retrospective study, 997 SLE (SLICC-ACR-2012 criteria) patients attending the Department of Clinical Immunology were enrolled. Comprehensive clinical examination and immunological profile data were retrieved from hospital records. GI involvement was defined as vasculitis, pancreatitis, lupus enteritis, ascites, organomegaly and motility disorder attributed to the disease. The association of various clinical and immunological markers was evaluated using a binary regression model.

Results: Sixty-one patients with SLE having GI manifestation [Figure 1] were compared to 274 SLE patients without GI manifestations. The mean age was 27.81 ± 10.49 years (93.1% females); median disease duration was 12 months (IQR 15). In 44.26% patients, GI manifestation was observed at onset, while the duration of appearance of GI manifestation was 14 months (IQR-40) in the remaining. Median follow up was 13 months(IQR 17). Complete improvement in GI symptoms was noticed at 15 days (IQR-15) with immunosuppressive therapy. Eight patients (13%) required ICU care and one patient required surgical intervention. One patient expired at 20 days of symptom onset. The logistic regression model suggested thrombocytopenia and higher disease activity were predictors of GI manifestations.



Conclusions: GI manifestations in SLE are associated with major organ involvement and with higher disease activity. GI manifestation responds adequately to immunosuppressants with the good short-term outcome.


  OC0011: Comparison of the therapeutic efficacy of transcutaneous electrical nerve stimulation versus ultrasound-guided genicular nerve block in patients with knee osteoarthritis Top


Ishita Dey, R. N. Haldar, Saumen Dey, Vasundhara Ghosal; Department of Physical Medicine and Rehabilitation, Senior Resident, Physical Medicine and Rehabilitation

Background: Osteoarthritis (OA) knee is a common disease of aged population and one of the leading causes of disability. There are various treatment modalities to manage the pain in this condition including transcutaneous electrical nerve stimulation (TENS) and genicular nerve block (GNB).

Objectives: The objective was to assess and compare the effect of ultrasound (US)-guided GNB and repeated daily TENS for 3 weeks in chronic knee pain in patients with OA knee.

Methods: This randomized clinical trial was conducted on 64 patients (32 TENS and 32 GNB) for 18 months at the Department of Physical Medicine and Rehabilitation, IPGMER and SSKMH. Inclusion criteria included primary OA knee, radiologic Keller–Lawrence Grading Scale score: 3–4, age: 40–80 years, individuals poorly responding to conservative treatments (previous high molecular weight hyaluronate injection), patients unwilling for surgical management, and those with Visual Analog Scale (VAS) score >5. Exclusion criteria included prior knee surgery, secondary OA, sciatic/other neuropathic pain, and IACS in the last 3 months. After Ethics Committee clearance and obtaining informed consent, 64 patients with primary knee OA were randomized into two treatment groups. TENS was applied for 3 weeks in the first group, and in the second group, US-guided superiomedial, superiolateral, and inferiomedial GNB was done.

Pain and knee functions were assessed with VAS and Western Ontario and McMaster Universities (WOMAC) index at baseline and at 1 and 3 months.

Result: Statistically significant reduction in VAS and WOMAC was detected over time in both groups. WOMAC was significantly better in GNB group than in TENS group at 1-month follow-up.

Conclusion: Both TENS and GNB have good clinical effects on knee OA with superiority of GNB.


  OPC0117: Duration of illness and presence of anti-dsDNA and nucleosome predict organ damage in systemic lupus erythematosus Top


S Chandrashekara, K R Anupama, B S Renuka; ChanRe Rheumatology and Immunology Center and Research, Bengaluru, Karnataka, India

Aim: This study aimed to investigate the potential serological and clinical markers associated with increased organ damage in systemic lupus erythematosus (SLE) patients.

Materials and Methods: SLE patients fulfilling the Systemic Lupus International Collaborating Clinics/American College of Rheumatology criteria (2010) were consecutively recruited. One hundred and fifty-four SLE patients were included in the study and fifty patients were excluded due to incomplete data. Clinical history, serological parameters including autoantibody profile and disease activity at baseline, and medications used were collected. Disease damage was assessed using the SDI scores at the point of recruitment. Patients were classified on the basis of Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) total score (based on the Safety of Estrogens in Lupus Erythematosus National Assessment-SLEDAI groupings) as follows: minimum disease activity or remission (0–3), mild (4–8), moderate (9–11), and severe (12 and above) disease activity.

Results: Ninety-eight female and six male patients with an average age of 28 (13–59) years were considered. Time to first visit to the clinician and follow-up period noted were 6 (1–132) months and 35 (2–160) months, respectively. Total duration of illness was 52.5 (4–217) months. Twenty patients had organ damage (SDI ≥1) and 83 (SDI = 0) had no organ damage. Comparison of patients with and without organ damage showed increased duration of illness and presence of significantly more anti-dsDNA or nucleosome or both autoantibodies in organ damage patients. Age, gender, total count, neutrophil-to-lymphocyte ratio, erythrocyte sedimentation rate, C-reactive protein, C3, baseline disease activity, autoantibodies, immunosuppressant usage, and steroid and biologics usage did not differ between the groups. Multivariate logistic regression revealed that increased duration of illness and presence of either anti-dsDNA or nucleosome or both were significant predictors of organ damage. Patients with either dsDNA or nucleosome or both were 6.5 times more likely to have organ damage.

Conclusion: Patients having longer disease duration and presence of anti-dsDNA or nucleosome or both have an increased risk of organ damage.


  OPC0189: Patient Reported Outcome-CLinical Arthritis (PRO-CLARA) in the evaluation of disease activity in rheumatoid arthritis Top


H Singh, M Gaikwad, M Yadav, J Singh, K Yadav, R Sangwan; Department of Medicine, Pt. BDS PGIMS, Rohtak, Haryana

Background: Patient Reported Outcome-CLinical Arthritis (PRO-CLARA) (a patient-reported outcome measure) is a new, feasible, easy-to-administer, less expensive, and reliable index to assess the impact of disease activity on daily living (which is patient's prime concern) in rheumatoid arthritis (RA). Experience on the use of PRO-CLARA is still limited.

Objective: The objective was to assess disease activity in RA using PRO-CLARA index and its correlation with routine disease activity indices.

Methods: A cross-sectional study was done on 100 RA patients (as per American College of Rheumatology criteria) who presented in rheumatology clinic at Pt. B.D. Sharma PGIMS, Rohtak (Haryana). Patients were assessed for disease activity using Disease Activity Score (DAS) 28, Clinical Disease Activity Index (CDAI), and PRO-CLARA at baseline, 2 months, and 4 months.

Results: The mean age was 46.17 + 12.89 years with 84 females and 16 males. Eighty-two patients were seropositive for RA. The disease activity at baseline, 2 months, and 4 months using DAS28 was 6.65 ± 0.68, 4.05 ± 0.81, and 3.33 ± 0.96; using CDAI was 36.5 ± 9.7, 18.36 ± 6.58, and 9.69 ± 6.69; and using PRO-CLARA was 8.78 ± 0.64, 6.62 ± 1.31, and 4.21 ± 1.59, respectively (P < 0.001). Reliability index (Cronbach's alpha) was assessed to be 0.68, 0.76, and 0.98 for DAS28, CDAI, and PRO-CLARA, respectively.

Conclusion: PRO-CLARA was found to be significantly correlated with DAS28 and CDAI. Since PRO-CLARA assesses disease activity along with the impact of disease activity in daily living, it could be a useful patient-assessed index in regular clinical practice for monitoring RA patients.


  OC0010: A comparative study on the efficacy of suprascapular nerve block versus subacromial steroid injection in shoulder impingement syndrome Top


Ambar Konar, Rajesh Pramanik, Firdaus Kamal, Debayan Ghorai; IPGMER and SSKM Hospital, Kolkata, West Bengal, India

Background: Shoulder impingement syndrome is one of the most common musculoskeletal pathologies leading to PM and R Outpatient Department visit. Over decades of use, literature shows lower infiltration and efficacy of intra-shoulder joint steroid in some etiological subtypes of impingement syndrome. Interestingly, intervention at suprascapular nerve, the major afferent supply around shoulder, has emerged as a treatment option for painful shoulder rehabilitation. This study is a sincere attempt to look for the efficacy of suprascapular nerve block (SSNB) over subacromial corticosteroid injection in shoulder impingement syndrome.

Objectives:

  1. To look for improvement in Visual Analog Scale (VAS) and Shoulder Pain and Disability Index (SPADI) after SSNB or subacromial steroid injection in shoulder
  2. To compare the efficacy of both the approaches.


Methods: This was a randomized controlled trial conducted on a total of seventy patients, 35 in each group, for 18 months at the Department of PM and R, IPGMER and SSKM Hospital, Kolkata, West Bengal, India.

Inclusion criteria:

  • Conservative treatment failure
  • Ultrasonography showing Neer Stage I and II impingement
  • VAS >5.


Exclusion criteria:

  • Coagulopathy
  • Rotator cuff tear
  • History of IACS within the last 3 months.


After Ethics Committee clearance, CTRI registration, and obtaining informed consent, two groups were made randomly. Group A received steroid injection by subacromial approach [Figure 1] and SSNB was done in Group B. All patients were included in step-wise rehabilitation program. Assessment by VAS and SPADI was done at baseline and 2/4/12 weeks after injection.

Results: Statistically significant improvement was seen in both groups with better result in SSNB. Intergroup comparison of SPADI was done by Student's unpaired t-test

Conclusion: SSNB is a better alternative both in short term and long term to subacromial steroid injection in the management of shoulder impingement syndrome.




  PC0013: Seronegative systemic lupus erythematosus or dermatomyositis? Connective tissue disorder gone haywire… Top


Pritam Das, Ashis Kumar Choudhury, Rohit Prabha Gaude, Sankha Shubhra Chakrabarti, Saumya Gupta, Deepak K Gautam, I S Gambhir; Institute of Medical Sciences, BHU, Varanasi, Uttar Pradesh, India

Introduction: A unique constellation of dermatomyositis, systemic lupus erythematosus (SLE), autoimmune hepatitis (AIH) type I, and Coombs-positive autoimmune hemolytic anemia with constrictive pericarditis makes connective tissue disorder a rare case.

Case Report: A 14-year-old female presented with complaints of fever for 3 months, erythematous rashes over the cheek and around eyelids for 3 months, and polyarthritis and right-sided chest pain during bending forward for 20 days. On examination, heliotrope rashes and Gottron's papule were present. The patient had pancytopenia and Coombs-positive hemolytic anemia. Liver function test was deranged with raised alkaline phosphatase. Antimitochondrial antibody and F-actin were positive suggestive of AIH Type I. However, overlap syndrome can also be possible (AIH and primary biliary cirrhosis). The patient also had constrictive pericarditis which can be explained due to autoimmune process. The patient also had exudative pleural fluid with raised lactate dehydrogenase, which can again be due to autoimmune process. However, antinuclear antibody (ANA) was negative in multiple repeated investigations. The diagnosis of SLE was kept on the basis of autoimmune hemolytic anemia, thrombocytopenia, arthritis, serositis, oral ulceration, malar rashes and photosensitivity, low C3 levels, and positive immunoglobulin M anticardiolipin antibodies. A possibility of seronegative SLE was kept. Hence, a diagnosis of seronegative SLE with dermatomyositis with AIH was kept. The novelty of the case was in its presentation and the progression of the disease.

Conclusion: Seronegativity in lupus patients occurs due to the entrapment of ANA in circulating immune complexes. Lupus hepatitis and AIH are two separate immunologic conditions involving the liver, a leading dilemma in diagnosis. The most confounding issue is that patients with AIH may be at an increased risk of developing systemic connective-tissue diseases and vice versa. The mode of treatment is different in both the forms of hepatitis. Constrictive pericarditis is also a rare complication of SLE.


  OPC0024: A case of Idiopathic CD4 Lymphocytopenia With Lupus Nephritis And Ulcerative Colitis Top


kalpana Acharya; Dr. RML Hospital, New Delhi, India

Case study: A 42-year-old female presented with complaints of symmetrical polyarthralgia involving both large and small joints for 1 year with generalized, nonitchy, and nonscarring alopecia and nonscarring photosensitive rash on bilateral cheeks for 6 months. She also complained of bloody diarrhea 8–10 times/day not associated with pain abdomen/tenesmus, also associated with dysphagia to both solid and liquid. Joint examination showed tenderness without synovitis and deformity. Blood investigations revealed anemia with deranged kidney function tests. Urine examination revealed dysmorphic red blood cells, and 24-h urinary protein was 1.1 g. Antinuclear antibody was positive with a titer of 1:320, anti-dsDNA (4+), anti-nucleosome (4+), and anti-histones (4+). Kidney biopsy showed Class 2 lupus nephritis, with activity score and chronicity score of 0/24 and 3/24, respectively. Endoscopy revealed esophageal-candidiasis (lupus anticoagulant-D). HIV was negative twice. CD4 levels were persistently low (<250/μl) on two separate occasions. Other viral serologies and serum immunoglobulins were normal. Contrast-enhanced computed tomography abdomen was suggestive of inflammatory bowel disease (IBD). Colonoscopy and biopsy revealed ulcerative colitis (UC) (Grade IV). Fecal calprotectin was raised, pointing toward flare. The patient was started on steroid enema, steroids, sulfasalazine, and HCQs, and she responded dramatically.

Discussion: Genetic markers located in the short arm of chromosome 6 have been associated both with systemic lupus erythematosus (SLE) and UC. Literature suggests that idiopathic CD4 lymphocytopenia (ICL) may indirectly trigger autoimmune diseases; however, our patient had both SLE and UC, which is very rare.

Conclusion: This case reinforces the fact that all chronic diarrheas in a patient with SLE are not always tuberculosis or lupus enteritis. A probability of IBD although remote should be kept in mind because both the diseases have an autoimmune pathology and the treatment also overlaps. This is a very rare presentation of three rare diseases (ICL, SLE with nephritis, and UC) present in a single patient and manifesting simultaneously.


  OPC0180: A case of anti-phospholipid syndrome presenting as pulmonary arterial hypertension in a young male Top


Ashis Kumar Choudhury, Pritam Das, Rohit Prabha Gaude, Sankha Shubhra Chakrabarti, Saumya Gupta, Deepak K Gautam, I S Gambhir; Institute of Medical Sciences, BHU, Varanasi, Uttar Pradesh, India

Case Report: A 12-year-old male presented with a history of two episodes of vomiting of altered blood in the last 7 days. He had undergone upper gastrointestinal endoscopy, which revealed normal findings. On repeated history taking, we also came to know that he has breathlessness on exertion for the last 8 years. On examination, his vitals were stable, and general examination was unremarkable. On cardiovascular system examination, laterally shifted apex beat along the left 5th intercostal space, Grade-II left parasternal heave, and loud P2 were found. Other system examination was within normal limits. Routine laboratory parameters were within normal limits. Electrocardiography was suggestive of right ventricular hypertrophy. Echocardiography revealed dilated right artery (RA), right ventricle (RV), and pulmonary artery with pulmonary artery systolic pressure of 80 mmHg, which is suggestive of severe pulmonary arterial hypertension (PAH). Computed tomography pulmonary angiography revealed feature suggestive of PAH with RA and RV dilation and thrombosis of the right and left branches of portal vein. Anti-phospholipid syndrome (APS) profile was done which came out to be positive for anticardiolipin antibody. PAH can be explained by vascular pathology which may be due to recurrent pulmonary vascular thrombosis or emboli.

Discussion: APS is an auto-antibody-mediated acquired thrombophilic disorder which can present with recurrent venous or arterial thrombosis and pregnancy morbidity. Diagnosis of APS can be ensured by the presence of at least one clinical and one laboratory criteria. The clinical criteria include any vascular thrombosis or pregnancy morbidity and the laboratory criteria include lupus anticoagulant, anti-cardiolipin, or anti beta2-glycoprotein I antibodies to be positive in intermediate or high titers on two occasions, 12 weeks apart.

Conclusion: APS can be the underlying cause of various clinical scenarios with systemic vascular thrombosis, as PAH with portal vein thrombosis in our case. It should always be considered in a patient who present with arterial or venous thrombosis or pregnancy morbidity with age <55 years.


  OC0013: Retroperitoneal mass in dermatomyositis: Malignancy versus infection Top


Sairam B, Atul Kakar, Atul Gogia, S. P. Byotra; Sir Ganga Ram Hospital, New Delhi, India

Case Report: A 63-year-old male presented with complaints of backache, which was radiating to the left thigh, and low-grade fever for 15 days. He was diagnosed as a case of dermatomyositis 3 years back, on methotrexate therapy (20 mg weekly) for 2 years. On admission, the patient was febrile, tachycardic, and normotensive with no cutaneous features suggestive of active dermatomyositis. Musculoskeletal examination showed antalgic gait with restricted movement of hip and knee joints on the left side. There was no fixed flexion deformity of hip. Spine and other systemic examinations were normal. Contrast-enhanced computed tomography chest and abdomen showed heterogeneous mass with central necrotic area, possibly retroperitoneal sarcoma with degeneration. Magnetic resonance imaging abdomen was also suggestive of hemorrhagic mass lesion in the left psoas muscle, possibly retroperitoneal sarcoma. Ultrasonography-guided fine-needle aspiration cytology was attempted; however, it drew only 0.5 ml of thick material, cytology of which showed no malignant cells. We decided to open up the mass under general anesthesia. Biopsy was suggestive of chronic inflammation with marked proliferating fibrous tissue and culture showed  Salmonella More Details enteritidis. Intravenous ceftriaxone was given and the patient recovered.

Discussion: Malignancy can be associated with dermatomyositis. Common carcinomas include adenocarcinoma of lung, pancreas, stomach, and nasopharynx. There are few case reports of retroperitoneal sarcoma associated with dermatomyositis.

Conclusion: Sarcoma can present with fever, occasionally in case there is necrosis. However, in any patient, especially immunocompromised, infection should always be considered as a possibility. We present this case to highlight the importance of culture in any patient on immunosuppressants no matter how vague his/her presentation is.




  PC0015: Rare manifestations of Behcet's disease: Differential response to treatment Top


Debaditya Roy, Chirantan Majumdar, Kaushik Basu; Department of General Medicine, Medical College and Hospital, Kolkata, West Bengal, India

Behcet's disease is a chronic, multisystem disease characterized by oral and genital apthae; cutaneous lesions; and ophthalmic, neurologic, or rheumatological manifestations. Cardiac involvement is comparatively rare (7%–46%), ranging from asymptomatic pericardial effusion to life-threatening myocardial infarction. Similarly, while recurrent, nonerosive, inflammatory asymmetric mono/oligoarthritis (81%) is the most common manifestation, erosive forms of arthritis such as sacroiliitis is comparatively rare (7.5%). Lastly, thrombocytopenia in Behcet's disease is yet another rare manifestation, reported only in a few case reports. Our patient, a 40-year-old male, presented with both classical and rare manifestations through the evolving course of this disease interspersed by relapses and remissions. The initial symptoms were that of oral and genital ulceration with typical pustular vasculitic rash and classical inflammatory joint pain along with features of anterior uveitis. He was initially treated with tablet colchicine, tablet prednisolone, and additionally with injection etanercept. In spite of it, he had multiple relapses along with the development of a persistent thrombocytopenia (having a normal bone marrow report), advent of a low back pain in the form of sacroiliitis evidenced on magnetic resonance imaging (MRI), and heaviness in the chest evidenced as pericardial effusion in echocardiography. After ruling out other secondary causes of pericarditis, and keeping in mind his poor response to oral medications for pericarditis (i.e., nonsteroidal anti-inflammatory drugs, colchicine and prednisolone), he was treated with injection infliximab (at 3 mg/kg). However, though thrombocytopenia still persisted, complete resolution of the effusion was seen on repeat echocardiography and also resolution of the sacroiliitis on repeat MRI with subsidence of symptoms on follow-up after 2 months. This case report thus adds to the growing evidence of the association of thrombocytopenia due to Behcet's disease and sensitizes clinicians about early detection and need for early intervention of these rare symptoms as prognosis of cardiac lesions is relatively poorer in Behcet's disease, and sacroiliitis often runs a destructive course over time.


  OC0014: NA Top


Chanchal Gera, Hepciba Glory Boyidi1, Navjot Singh1; SPS Hospitals, 1Christian Medical College, Ludhiana, Punjab, India

Background: In systemic lupus erythematosus (SLE) patients among various antinuclear antibodies (ANAs), known associations are anti-dsDNA antibodies with lupus nephritis, anti-SSA and anti-SSB antibodies with sicca symptoms, and anti-RNP antibodies with Raynaud's phenomenon. So far, we know very little about the role of these autoantibodies in the pathogenesis of SLE including their role in a variable presentation of SLE.

Objective: The objective was to study the correlation of different ANAs with various manifestations of SLE patients.

Materials and Methods: Sixty-three patients diagnosed as SLE by fulfilling Systemic Lupus International Collaborating Clinics criteria, attending a tertiary care institute in North India, were analyzed regarding clinical profile and laboratory characteristics. Detailed autoimmune profile (17 antibodies) was done for 35 patients, and the correlation of different ANAs with various manifestations of SLE was studied. Analysis was done using SPSS software version 21.0. Qualitative variables were correlated using Chi-square test/Fisher's exact test. P < 0.05 was considered statistically significant.

Results: dsDNA was significantly associated with prolonged fever (P = 0.0348). Anti SS-A/Ro60 was the most common antibody and found in 80% of our patients. Anti-SS-B/La has shown positive correlation with anemia (P = 0.033) and thrombocytopenia (P = 0.011) and negative correlation with photosensitivity (P = 0.004). Proteinuria in our SLE patients was significantly associated with anti-nucleosome antibodies (P = 0.015) and antihistone antibodies (P = 0.007). Significant association is noted between antinucleosome antibodies and nonscarring alopecia (P = 0.018) as well. Differently, SmD1 antibodies were noted to have statistical significance to polyarthritis (P = 0.018) and splenomegaly (P = 0.044). Incidence of hypothyroidism was more in patients who had antihistone antibodies (P = 0.033).

Conclusion: We have found many new associations between clinical features and various autoantibodies in our SLE cohort. In view of small sample size, we need more studies to support these observations.


  OC0015: Correlation of different antinuclear antibodies with various manifestations in systemic lupus erythematosus patients Top


Chanchal Gera, Hepciba Glory Boyidi, Navjot Singh;

Background: In systemic lupus erythematosus (SLE) patients among various antinuclear antibodies (ANAs), known associations are anti-dsDNA antibodies with lupus nephritis, anti-SSA and anti-SSB antibodies with sicca symptoms, and anti-RNP antibodies with Raynaud's phenomenon. So far, we know very little about the role of these autoantibodies in the pathogenesis of SLE including their role in a variable presentation of SLE.

Objective: The objective was to study the correlation of different ANAs with various manifestations of SLE patients.

Materials and Methods: Sixty-three patients diagnosed as SLE by fulfilling the Systemic Lupus International Collaborating Clinics criteria, attending a tertiary care institute in North India, were analyzed regarding clinical profile and laboratory characteristics. Detailed autoimmune profile (17 antibodies) was done for 35 patients, and the correlation of different ANAs with various manifestations of SLE was studied. Analysis was done using SPSS software version 21.0. Qualitative variables were correlated using Chi-square test/Fisher's exact test. P < 0.05 was considered statistically significant.

Results: dsDNA was significantly associated with prolonged fever (P = 0.0348). Anti-SS-A/Ro60 was the most common antibody and found in 80% of our patients. Anti-SS-B/La has shown a positive correlation with anemia (P = 0.033) and thrombocytopenia (P = 0.011) and a negative correlation with photosensitivity (P = 0.004). Proteinuria in our SLE patients was significantly associated with anti-nucleosome antibodies (P = 0.015) and antihistone antibodies (P = 0.007). Significant association is noted between antinucleosome antibodies and nonscarring alopecia (P = 0.018) as well. Differently, SmD1 antibodies were noted to have statistical significance to polyarthritis (P = 0.018) and splenomegaly (P = 0.044). Incidence of hypothyroidism was more in patients who had antihistone antibodies (P = 0.033).

Conclusion: We have found many new associations between clinical features and various autoantibodies in our SLE cohort. In view of small sample size, we need more studies to support these observations.


  OPC0027: Assessment of disease activity in rheumatoid arthritis patients using Mean Overall Index for Rheumatoid Arthritis Top


H Singh, M Mangla, A Kumar, M Gaikwad, M Yadav, J Singh; Department of Medicine, Pt. BDS PGIMS, Rohtak, Haryana, India

Background: Mean Overall Index for Rheumatoid Arthritis (MOI-RA) is a new, continuous composite disease activity index, which includes mean of standardized values of tender and swollen joint counts (28 joint counts); patients' and physicians'/evaluators' assessments of global health, patients' assessment of pain, Health Assessment Questionnaire, and erythrocyte sedimentation rate. Till now, there is a paucity of data on MOI-RA in India.

Objective: The study objective lies in the assessment of disease activity of RA using MOI-RA and its correlation with disease activity score using 28 joint counts (DAS-28) and Clinical Disease Activity Index (CDAI).

Materials and Methods: The present study was a cross-sectional study done on 100 RA patients (American College of Rheumatology criteria) who presented to the rheumatology clinic at PGIMS, Rohtak (Haryana). Patients were assessed for disease activity using DAS 28, CDAI, and MOI-RA at baseline and at 3 months.

Results: The mean age of the study group was 40.04 ± 11.47 years with 84 females and 16 males, with 70 among them being seropositive for RA. Disease activity scores assessed at baseline and at 3 months using DAS28 were 5.91 ± 1.309 and 2.74 ± 0.84; using CDAI were 30.56 ± 17.82 and 3.79 ± 4.18; and using MOI-RA were 43.54 ± 18.81 and 8.11 ± 5.67, respectively (all three P < 0.001). Correlation of MOI-RA with DAS28 and CDAI at baseline was 0.962 and 0.961, respectively, and at 3 months, it was 0.929 and 0.939, respectively. Reliability indexes (assessed by Cronbach's alpha) were 0.751, 0.445, and 0.360 for MOI-RA, DAS-28, and CDAI, respectively.

Conclusion: MOI-RA was found to be significantly correlated with DAS28 and CDAI. The MOI-RA includes all important measures of disease activity from both physicians' and patients' perspective, especially impact on daily living, so it could be useful in regular clinical practice for monitoring RA patients.


  OPC0162: Study of clinical profile of macrophage activation syndrome in systemic lupus erythematosus patients in a tertiary care hospital and applicability of Hemophagocytic Lymphohistiocytosis criteria Top


Praveen kumar M, Phani Kumar D, Liza Rajasekhar; Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Objective: The objective was to study the clinical and laboratory features of macrophage activation system (MAS) in systemic lupus erythematosus (SLE) and to assess the applicability of Hemophagocytic Lymphohistiocytosis (HLH) criteria 2004 in SLE patients.

Methods: Clinical, laboratory, and treatment outcomes of patients with diagnosis of MAS from 2011 to 2018 were noted from inpatient case files. These parameters were matched with HLH 2004 criteria and patients who fulfilled the five criteria were considered to have definite MAS and those who did not fulfilled were considered as incomplete MAS.

Results: Twenty-two out of 2500 SLE patients were identified (4 males and 18 females) with a mean (±standard deviation) age of 24.5 years (±11.3). Median (range) duration of SLE at the onset of MAS was 21 months (range: 6–82 months). MAS was the presenting manifestation of SLE in eight patients. Fourteen (63%) and eight patients (37%) were diagnosed as definite and incomplete MAS, respectively. Clinical features noted were fever (22/22), hepatomegaly (8/22), splenomegaly (6/22), and lymphadenopathy (5/22). The mean hemoglobin, serum ferritin, and triglycerides levels were 8.2 g% (1.14), 1371 mcg/l (589.2), and 295 mg/dl (132), respectively. The median leukocyte count, platelet count, and aspartate transaminase level were 2650/mm3 (1200–5000), 80,000/mm3 (62,000–150,000), and 122 IU (55–236) respectively. Bone marrow evidence of hemophagocytosis was found in four patients. Fifteen patients were treated with methyl prednisolone pulse therapy and six with 1 mg/kg steroid. Only three patients required cyclosporine for recovery.

Conclusion: Sixty-three percentage of patients diagnosed as definite MAS and 86% of patients showed response to steroid therapy. HLH criteria holds good to diagnose MAS in SLE. MAS should be considered in lupus patients presenting with fever, cytopenias, and transaminitis.


  OC0016: HSP in Elderly patients- More RPGN, More recurrences Top


Pritam Das, Ashis Kumar Choudhury, Rohit Prabha Gaude, Sankha Shubhra Chakrabarti, Saumya Gupta, Deepak K Gautam, I S Gambhir; Institute of Medical Sciences, BHU, Varanasi, Uttar Pradesh, India

Henoch–Schönlein purpura (HSP) is an immune-mediated (IgA deposition) vasculitis. The elderly patients have more severe natural history with more predominant renal involvement with chances of developing end-stage renal disease. Recurrence and relapse is more predominant. Plasmapheresis and rituximab may be required for preventing further renal damage. Here, we present two cases of HSP in the elderly in Uttar Pradesh, India, within the last 6 months. Our index case is a 60-year-old male, presenting with facial puffiness, fever, and lower-limb rashes for 20 days. The patient had deranged renal function test (RFT) on admission with oliguria. The rashes increased in severity, gradually involving the trunk. Skin biopsy was suggestive of leukocytoclastic vasculitis. Direct immunofluorescence showed 3+ granular IgA deposits along the wall of dermal vessels. The patient was treated with corticosteroids. The patient had no relapse till date. Our 2nd case was a 55-year-old female presenting with abdominal pain and skin rashes, in the lower limbs for 15 days and slightly deranged RFT. The patient was started on corticosteroids. After 1 month, the patient developed skin rashes with increased severity. Plasmapheresis was done. Elderly patients presenting with rashes suggestive of venulopathy should be monitored closely with RFT. Two cases in the elderly had been reported from this epidemiological background for the last 6 months (workup is going on one more case). There may be more genetic predisposition in this area which is being triggered by environmental triggers. We hypothesize that HSP in the elderly is underrecognized and has a unique epidemiological pattern; treatment should be started early and plasmapheresis is a better option for relapse or recurrence.


  PC0016: Effect of alendronate on bone mineral density (BMD) in systemic lupus erythematosus (SLE) Top


Shankar Machigar, Yousuf khan, Abhilasha Manwatkar, Lalna Kalekar, Yojana Gokhale; Rheumatology Services, Department of Medicine, Lokmanya Tilak Medical College, Mumbai, Maharashtra, India

Introduction: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease, treated with steroids and immunosuppressants. Alendronate is known to decrease steroid-induced bone loss, but related data in Indian literature are sparse.

Methods: This is a prospective study of 21 newly diagnosed SLE (Systemic Lupus International Collaborating Clinics 2015 criteria), enrolled over 6 months and followed up for 1 year. Osteoporosis risk factors were noted. Disease activity was calculated by Systemic lupus erythematosus Disease Activity Index (SLEDAI). All patients were treated with steroids ± immunosuppressants as per indication and were started on alendronate (35 mg) weekly at onset till steroid dose was ≤5 mg/day. DEXAscan by GE lunar was done at baseline and repeated after 1 year. Those with low Vitamin D were given injection Vitamin D3 before DEXAscan. Fracture Risk Assessment Tool (FRAX) score at baseline and at 1 year was calculated.

Results: Twenty-one SLE presented with a mean age of 25.33 years; one patient lost to follow-up. Baseline characteristics are summarized in [Table 1]. With alendronate, bone mineral density (BMD) increased by 0.49% at spine and decreased by 0.92% at femur. Spine fracture risk by FRAX at baseline was 1.1, it doubled (2.2) at 1 year with steroid use. Hip fracture risk was 0.1% at baseline and increased 5 times (0.5%) at 1 year. In patients with cumulative dose of steroid of <5.5 g/year, BMD increased by 2.4% and 2.9% at spine and femur, respectively. In patients with SLEDAI <10, BMD increased by 1.7% and 0.9%, while in those with SLEDAI >10, BMD decreased by 0.41% and 3.25% at spine and femur, respectively. SLE patients with low Vitamin D who were given Vitamin D3 supplementation gained BMD by +1.3% at spine and +1.9% at femur as against those who were not given Vitamin D3 due to normal Vitamin D level.



Conclusion: In all SLE patients on alendronate, BMD increased at spine and decreased at femur after 1 year. However, as compared to other studies on SLE with steroid (without alendronate), bone loss at femur was relatively less. High cumulative steroid dose and high disease activity increased bone loss. Replenishing Vitamin D reduced bone loss. Fracture risk increased at 1 year.


  OPC0134: Lupus myocarditis is an early severe complication in systemic lupus erythematosus with significant mortality: Case series from North India Top


Aadhaar Dhooria, Atit Gawalkar, Krishna Santosh1, M B Adarsh, Shefali Sharma, Aman Sharma, Sanjay Jain, Varun Dhir; Departments of Internal Medicine and 1Cardiology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background/Purpose: Myocarditis is a severe manifestation of systemic lupus erythematosus (SLE). We aimed to describe the clinical features and outcomes of lupus patients with myocarditis in a North Indian population.

Methods: Clinical records of SLE patients were screened for a clinical and laboratory diagnosis consistent with lupus myocarditis (global hypokinesia with left ventricular ejection fraction <50% in the absence of massive pericardial effusion, severe pulmonary artery hypertension, regional wall motion abnormality, or primary valvular disease). Cardiac outcomes as well as survival were assessed using Kaplan–Meier survival analysis and Cox regression analysis.

Results: A total of 37 patients with SLE who had features consistent with lupus myocarditis were included in this study. Twelve patients (32%) presented with lupus myocarditis as the first presentation. All patients received corticosteroids, while 27 patients received additional intravenous cyclophosphamide pulses. Ten deaths (27.7%) were noted, of these nine died during the initial presentation, while one patient died 2 months later of disseminated varicella infection. The deaths were attributed to disease activity alone in two patients, activity with infection in five patients, and infection alone in the remaining three patients. Raised serum procalcitonin at presentation (>0.9 ng/ml) (P = 0.049), higher blood urea (P = 0.038), and low serum complement C3 (<50 mg/dl) (0.002) were associated with increased mortality. Patients with raised serum procalcitonin at presentation had reduced survival (mean survival of 2.5 months, confidence interval [CI]: 0.1–4.8 months) as compared to those without a raised value (mean survival of 49.2 months, CI: 38.2–60.2 months) [P = 0.014; [Figure 1]].



Conclusions: Lupus myocarditis is a manifestation that occurs early in the course of lupus and is associated with a fatal outcome in one-third of the patients even in the short term. Elevated serum procalcitonin, probably reflecting concomitant infections, is a marker of reduced survival in these patients.


  OPC0050: Juvenile spondyloarthropathy with recurrent macrophage activation syndrome Top


Chanchal Gera, Chepsy C Philip1; SPS Hospital, 1Christian Medical College, Ludhiana, Punjab, India

Case Report: Macrophage activation syndrome (MAS) is characterized by overwhelming inflammatory reaction due to an uncontrolled and dysfunctional immune response leading to continual activation and expansion of T-lymphocytes and macrophages, which leads to massive hypersecretion of pro-inflammatory cytokines. MAS occurs in the context of infectious, malignancy, metabolic, and autoimmune diseases, but seems particularly linked to systemic juvenile idiopathic arthritis (sJIA), occurring in at least 7%–13% of sJIA patients, but only few case reports are available with juvenile spondyloarthropathy. We report the case of a young boy with juvenile spondyloarthropathy with multiple episodes of MAS. Genetic analysis was done to rule out primary MAS, for example, deletion or duplication of UNC13D, PRF1, and STX11 genes, which turned out to be negative. The patient was successfully treated with dexamethasone with slow tapering. Sulphasalazine was added to keep his inflammatory arthritis under control. He has got one more episode of macrophage activation since the last 1 year.

Discussion: Juvenile spondyloarthopathy can be complicated by systemic manifestations, for example, MAS. In case of recurrent MAS, genetic analysis should be done to rule out familial MAS.


  OC0018: Polymyalgia rheumatica: Are we under diagnosing in India? Top


Pravin Patil, Sameerkumar Shah; Apex Rheumatology Clinic, Pune, Maharashtra, India

Background: Worldwide, polymyalgia rheumatica (PMR) is considered the most common inflammatory rheumatic disease in the elderly. There are several conditions that can mimic PMR, making the evaluation of PMR challenging.

Objective: The objective was to describe the clinical profile of patients with PMR in India.

Methods: EMR database of patients available from August 2015 to 2018 was searched for the diagnosis of PMR. Out of 10,522 patients, we found a total of five patients with a diagnosis of PMR. We evaluated clinical, laboratory, and radiographic findings. All patients fulfilled 2012 European League Against Rheumatism/American College of Rheumatology classification criteria.

Result: The following table describes the characteristic features of PMR observed in our patients.

Conclusion: PMR is probably less well recognized in India. There is a long delay between symptom onset and diagnosis. US in PMR depicts characteristic findings that can aid in distinguishing PMR from the mimics.




  PC0017: Anemia in systemic lupus erythematosus: Etiology and correlation with autoantibody profile Top


V Vasdev, S Bhatt, S Mishra, A Hegde, S Ramakant, A Kumar; Army Hospital Research and Referral, New Delhi, India

Background: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder. Anemia is a common clinical finding in patients with SLE. The association of various causes of anemia with specific autoantibodies of SLE and their prognosis is not well known from adequately sized studies.

Objectives: This prospective study aims to assess the prevalence of anemia, its etiological profile, and correlation of anemia and its subtypes with the autoantibody profile in SLE patients.

Methods: A total of 124 SLE patients following up at a tertiary care center were screened for the presence of anemia (according to the WHO definition). Anemic patients underwent evaluation with red blood cell indices, peripheral blood smear, iron studies, and Coomb's test, and classified into nutritional anemia, anemia of chronic disease (ACD), and autoimmune hemolytic anemia. The correlation between anemia and its subtypes with the autoantibody profile was studied.

Results: The prevalence of anemia was 60.5% with 49.3% having moderate, 32% severe, and 18.7% mild grade anemia, as per the WHO classification. ACD was the most common cause of anemia accounting for 73.3% of all patients, while iron deficiency anemia was least at 10.7%. A statistically significant correlation was found between anti-dsDNA antibody and presence of anemia (P = 0.001). Anti-dsDNA antibodies were also seen in half of the patients (26/55) with ACD, and in most patients with nutritional anemia (7/8), but were less common among patients with hemolytic anemia (1/12). No statistically significant correlation was found between anemia and other antibody subtypes.

Conclusion: There is a high prevalence of anemia in SLE, with ACD being the most common etiological subtype. This study hints toward positive correlation among anemic patients and anti-dsDNA autoantibodies. However, more elaborate studies with larger sample size are required to confirm our results.


  PC0018: A clinical spectrum of gout: Analysis of a large database in a referral community-based center Top


Bharat Veer Manchanda, Amit Jain, Abraham Mohan, Anuradha Venugopalan, Arvind Chopra; Center for Rheumatic Diseases (CRD), Pune, Maharashtra, India (www.rheumatologyindia.org)

Introduction: We believe that gout is universally the same. This may not be true. The prevalence of gout in an Indian community was 0.06 in urban areas and 0.13 in rural areas (J Rheumatol 2009; 36:3). We decided to study Indian gout in a community setting.

Methods: Data were extracted from the comprehensive referral database at the Center for Rheumatic Diseases (CRD) maintained since 1996. This was a cross-sectional retrospective study with prospective follow-up. The study period was from January 2007 to December 2017. A total of 80–110 patients from all over Maharashtra are examined daily in CRD. Patients with asymptomatic hyperuricemia were excluded. Data described below are at first examination in CRD.

Results: A total of 585 patient records (male:female ratio = 12:1) were retrieved (45% rural, mean age: 48 years [range: 16–92], mean disease duration: 78 months [range 3 days–63 years], with 5.8% positive family history gout). Nearly 8.5% used tobacco and 10.4% patients consumed regular alcohol; ~10% were pure vegetarian from birth. Around 51% of patients reported classical podagra; 8.7% showed tophi including common sites such as knee, ankle, and metatarsophalangeal joints; ear was infrequent. The articular pattern was as follows: 46% polyarticular, 47% oligoarticular, and 7% monoarticular: ~80% patients with supporting radiological picture. Less than 5% patients recorded renal derangement; 8.5% with renal calculi. Co-existing diseases were as follows: hypertension (21.7%), diabetes mellitus (5.6%), hyperlipidemia (58.2%), and ischemic heart disease (2.9%). An unusual co-occurrence of gout and rheumatoid arthritis was diagnosed in 22 patients. The basis of diagnosis was predominantly clinical; only 35% showed hyperuricemia (8 mg% or more). Acute attacks were mostly managed with nonsteroidal anti-inflammatory drug and/or oral colchicines; steroids were infrequent. Approximately 70% of patients required long-term allopurinol, with <10% patients on febuxostat. There were several limitations including retrospective design and missing and insufficient data, and hence we could not calculate body mass index.

Conclusion: Though seemingly familiar to the classical text, there were phenotypic differences in gender, tophi, articular pattern, and comorbidity. We ought to seek and treat modifiable risk factors.


  OPC0033: A study of correlation of serum Vitamin D3 level with hematological involvement in systemic lupus erythematosus Top


Dharmishtha Ashis Basu, Chayanika Dutta, Sanjeeb Kakati, Lahari Saikia1; Departments of Medicine and 1Microbiology, Assam Medical College and Hospital, Dibrugarh, Assam, India

Background: Deficiency of Vitamin D is prevalent worldwide and more so in some autoimmune diseases such as systemic lupus erythematosus (SLE) as per studies. It may be due to photosensitivity and sunscreen-use led less sun exposure, early involvement of the kidneys in the disease process, or chronic use of certain drugs. Studies also point that there is an inverse correlation between Vitamin D level and hematological manifestation of SLE.

Objective: The objective was to the study the presence of Vitamin D deficiency among SLE patients with special reference to hematological involvement.

Method: In order to check the Vitamin D status and any possible correlation of Vitamin D with the disease activity of SLE, we had undertaken a hospital-based cross-sectional case–control study of 109 SLE patients and 109 age- and sex-matched healthy volunteers from the same geographical area. Vitamin D level was assessed by MINI VIDAS®, 25-hydroxyvitamin D and total enzyme-linked fluorescent assay. Hemoglobin of <10 g/dL was taken as the cutoff for anemia. White blood cell count of <4000/mm3 was taken as the cutoff for leukopenia and platelet count of <100,000/mm3 was taken as the cutoff for thrombocytopenia.

Results: The healthy population had inadequate level of Vitamin D (23.52 ± 8.95 ng/ml), but the inadequacy was higher among SLE patients (20.19 ± 9.49 ng/ml). Vitamin D levels of 90.8% of SLE patients and 77.9% of healthy controls were below the normal range. The difference of Vitamin D level was significant among healthy population, newly diagnosed cases, and on treatment group (P < 0.01). Anemia was found to the most common hematological involvement (50.4%). Nearly 57.1% of patients with thrombocytopenia and 100% of those with leucopenia were seen to be Vitamin D deficient.

Conclusion: Vitamin D has a great role in immunosuppression and, being easily available and relatively nontoxic, it can be of great help as an immunomodulator in the treatment of hematological involvement in SLE.


  OPC0036: A rare case of young girl presented with autoimmune haemolytic anaemia with haemolytic crisis in known case of SLE with secondary APLA, treated successfully with RITUXIMAB Top


A Panday, J Oak, S Tulpule, M Mane, S Sharma, R Mathur, V Hirapara; Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Andheri, Mumbai, Maharashtra, India

A 15-year-old girl presented with a history of recurrent anemia, breathlessness at rest, severe abdominal pain, and fever spikes for 7 days. On examination, the patient had tachypnea, tachycardia, and hypotension. She had pallor with icterus and severe abdominal tenderness with hepatosplenomegaly with splenic enlargement of 10 cm. In view of suspected hemolytic anemia, investigations showed hemoglobin (Hb) – 3.1 g/dl, white blood cell (WBC) – 2640/mm3, platelet count – 128,000/mm3, reticulocyte count – 17.4%, and lactate dehydrogenase – 1071.2 U/l. Peripheral smear showed marked anisocytosis with macrocytosis, few polychromatic cells, spherocytes, and microspherocytes [Figure 1]. Direct and indirect Coombs test showed positivity. Anticardiolipin IgG was 16.65 GPL/ml (normal range – <15.0 GPL/ml) and immunoglobulin M was 36 GPL/ml (normal range – <12.5 GPL/ml). Activated partial thromboplastin time was 33.6 s (normal range – 23.2–28.3 s), total bilirubin was 3.36 mg/dl, direct bilirubin was 0.36 mg/dl, and indirect bilirubin was 3.0 mg/dl. Antinuclear antibody (immunofluorescence assay) was positive (+++) (1;320) and ds-DNA was positive. Her two-dimensional echocardiography and X-ray chest were normal. Computed tomography abdomen showed gross splenomegaly with chronic infarcts and hepatomegaly. The patient was started on iv methylprednisolone 1 g/day for 5 days. The least incompatible packed red cell unit was infused as a life-saving measure as Hb was 3.1 g/dl. Her Hb remained 6 g/dl after methylprednisolone and red blood cell (RBC) transfusion. She was given injection rituximab (375 mg/m2) weekly for 4 weeks. Her critical blood count reports after infusion showed Hb of 7.1 g/dl, WBC of 3550/mm3, platelet count of 97,000/mm3, and packed cell volume of 22.3%. The patient improved remarkably and stable at the time of discharge. Her recent reports showed Hb of 13.6 g/dl, WBC of 3600/mm3, and platelet of 131,000/mm3.

Discussion: Autoimmune hemolytic anemia is an uncommon disorder caused by RBC-directed autoantibodies. RBC-bound complement may potentiate hemolysis and present with fulminant disease. Therapies are aimed at decreasing antibody production using immunosuppressant and reducing RBC phagocytosis.

Conclusion: Rituximab is a therapeutic alternative to splenectomy and can be used with concurrent corticosteroid treatment.




  OPC0037: The current status of spondyloarthritis patient management services in Gujarat: a patient awareness survey Top


Himanshu Pathak1, Sapan Pandya2, Shabbir Chikani3, Reena Sharma4, Ripal Shah1, Pradeep Prajapati1, Namisha Patel1, Alpana Parmar5, Rakesh Tank3 on behalf of Rheumatology Association Gujarat; 1Consultant Rheumatologist, Vadodara, 2Consultant Rheumatologist, Ahmedabad, 3Consultant Rheumatologist, Rajkot, 4Consultant Rheumatologist, Ahmedabad, 5Consultant Rheumatologist, Surat, Gujarat



Subject: Spondyloarthritis.

Background: Spondyloarthritis (SpA) is one of the most common rheumatological condition affecting the young group of population. In recent years the understanding about the condition has increased markedly. Previous studies, predominantly in western countries, have shown that patient education about disease and management, plays an important role in effective care.

Objectives: To know the perceptions of the patients, suffering from SpA, about their condition and care given by rheumatologists.

Methods: The survey questionnaire was formulated by a core group of rheumatologists who are members of Rheumatology association of Gujarat. The survey was conducted over the period of 12 weeks at the urban hospitals and private clinic settings. The survey questions (total 26 questions) were in the local language (Gujarati), to be filled at the time of clinic visit. The questions comprised of multiple choices or free text options. Adult patients age > 14 years with an established diagnosis of SpA, after verbal consent, were included in the survey.

Results: Total 199 patient filled the survey forms.78 %(n=155) respondents were male and 22% were females(n=44). The mean age was 33 years (15-68). Not all patients responded to all survey questions. The result highlights are shown in Table 1. Only 25 %(n=50) were given a diagnosis of ankylosing spondyloarthritis before coming to rheumatologist. Only 20%(n=38) of patients ever heard of term axial spondyloarthritis.

Conclusion: We believe this survey is the first attempt in India, to know the patient perspectives about SpA care. High response rate and questionnaire in local language are an important strength of our survey. Predominantly urban population and non-involvement of other physicians of the region who are caring for SpA patients are the main limitation of our survey. Nonetheless, the current survey gives us an important information about the significant gap in patient knowledge about SpA.


  OPC0190: A comparative study of nail-fold capillaroscopic changes in idiopathic interstitial pneumonia with idiopathic interstitial pneumonia autoimmune features Top


Kunal Kishore, V Vasdev, Hegde Arun, Ashwani Kumar, Ramakant; Department of Rheumatology, Army Hospital Research and Referral, New Delhi, India

Background: Lung involvement, especially interstitial lung disease (ILD), can be the first manifestation of an underlying connective-tissue disorder (CTD). About 25% of ILD occurs in the context of an “undifferentiated” CTDs, characterized by signs and symptoms that are not specific for any of the described CTD entities, now known as interstitial pneumonia with autoimmune features (IPAFs). Nail-fold capillaroscopy (NFC) is an important tool, which helps us in the early recognition of microvascular changes in patients with ILD.

Objectives: The objective was to study the various patterns on NFC in patients of IPAF and compare them with those having idiopathic interstitial pneumonia (IIP).

Methods: The study population consisted of fifty patients each of IIP and IPAF who fulfilled ERS/JRS/ALAT 2011 revised diagnostic criteria for IIP and ERS/ATS classification criteria for IPAFs, respectively. The study also included fifty age- and sex-matched controls, having normal respiratory examination clinically, normal chest X-ray, and normal pulmonary function tests. All patients underwent NFC at room temperature and the following parameters were recorded: capillary density, presence of megacapillaries, tortuosity, avascular areas, disarrangement, and neo-angiogenesis.

Results: This study included 23 (46%) female patients and 27 (54%) male patients in IPAF group and 19 (38%) female patients and 31 (62%) male patients in IIP group. The mean capillary density was significantly reduced in IPAF group and also had the presence of abnormal capillary morphologic patterns (microhemorrhages, neoangiogenesis, and megacapillaries).

Conclusion: This single-center study found that NFC is an important adjunct to differentiate between patients of IPAF and IIP, demonstrating higher frequencies of abnormalities (microhemorrhages, megacapillaries, and reduced capillary density) among patients with IPAF.


  OPC0038: Airway involvement in granulomatosis with polyangiitis patients Top


Nupoor Acharya, Saket Jha, Rajiv Ranjan, Sakshi Mittal, Shankar Naidu, Ritambhra Nada1, Manphool Singhal2, Mahesh Prakash2, Varun Dhir, Ranjana W Minz3, Sanjay Jain, Aman Sharma; Department of Internal Medicine, Division of Rheumatology, PGIMER, Departments of 1Histopathology, 2Radiodiagnosis and 3Immunopathology, PGIMER, Chandigarh, India

Background: Granulomatosis with polyangiitis is a small-vessel vasculitis predominant upper respiratory tract, pulmonary and renal involvement. Involvement of lower respiratory tract is also seen with granulomatosis with polyangiitis (GPA), which could be potentially life threatening.

Objective: The objective was to analyze the patients presenting with lower airway involvement in GPA.

Methods: A retrospective analysis of patients diagnosed as GPA presenting with lower airway involvement to PGIMER, Chandigarh, India, was done. All patients diagnosed as GPA presenting with laryngeal or tracheobronchial involvement were included. Their demographic, clinical, laboratory, and radiological parameters with treatment and outcome were collected from hospital records.

Results: Medical records of 181 GPA patients were screened. Seven out of 181 patients (3.86%) met the inclusion criteria. All patients tested positive for anti-neutrophil cytoplasmic antibodies on indirect immunofluorescence. Six patients tested positive for anti-PR3 and one for anti-myeloperoxidase. Subglottic stenosis was the most common involvement seen in six patients followed by tracheal and bronchial involvement. Sinonasal involvement was prominent and seen in six patients. Stridor and respiratory distress were the presenting manifestations in all the seven cases. All patients received steroids; six received cyclophosphamide and one patient received rituximab on presentation. Four patients eventually received rituximab. Four patients underwent tracheostomy and two patients additionally received airway dilatation and stenting. One patient underwent only airway stenting. Mortality was seen in two patients, while five patients showed improvement with therapy. One patient developed unilateral lung collapse with subsequent actinomycosis.

Conclusion: Lower airway involvement in GPA is a severe and life-threatening condition requiring urgent immunosuppression and surgical intervention to maintain ventilation. Resolution of inflammation and reversal of stenosis are seen in most patients with therapy. Repeated dilatations or stenting may be required in some patients.


  OPC0043: A study of autoantibody profile in systemic lupus erythematosus Top


Alal Uddin, Naman Jain, S Kakati, L Saikia; Assam Medical College, Dibrugarh, Assam, India

Background: Once it has been established that antinuclear antibodies (ANAs) are present, it is important to determine which particular nuclear antigens may be the target of the autoantibodies because some of these antigen-specific responses provide great diagnostic specificity.

Aims and Objectives: The aim and objective was to study autoantibody profile in systemic lupus erythematosus (SLE).

Methods: This is a hospital-based observational study carried out on 129 cases of SLE who fulfilled American College of Rheumatology criteria (1997) attending rheumatology outpatient department (OPD), other OPDs, or admitted in various wards at the Department of Medicine, Assam Medical College, Dibrugarh, Assam, India, during July 2016–June 2017, in order to know the prevalence of autoantibody profile. In our study, ANA assay was done via immunofluorescent assays, and various autoantibody relativities were studied by line immunoassay.

Results: All the 129 SLE patients were ANA (immunofluorescent assay) positive (100%). The frequency of different autoantibodies studied by line immunoassay is as follows: anti-dsDNA (58.91%) which is the commonest followed by anti-Ro (60 kDa) (44.9%), anti-histone (38.76%), anti-Ro (52 kDa) (37.98%), anti-SmD1 (27.13%), anti-ribosomal Po (27.13%), anti-nucleosome antibody (23.26%), anti-U1snRNP (16.28%), anti-La/SS-B (13.95%), anti-PCNA (5.43%), AMA M2 (3.1%), anti-Pm-Scl (3.1%), anti-Mi-2 (1.55%), anti-Scl 70 (1.55%), anti-Ku (1.55%), and anti-CENP B (0.78%). Anti jo-1 was not found in any patient.

Conclusion: As there is a paucity of similar studies, especially in this part of the world, the present study is an attempt to find out the prevalence of various autoantibodies in SLE patients, which may aid us to detect other associated connective-tissue disease disorder in SLE patients, and prognosticate the disease.


  OPC0044: Assessment of pulmonary function in rheumatoid arthritis patients attending rheumatology clinic Top


Kunal Dip Saha, Bhaskar Thakuria, Nilakshi Goswami; Gauhati Medical College, Guwahati, Assam, India

Background: Pulmonary involvement is a frequent and among the most severe extra-articular manifestations of rheumatoid arthritis (RA), ranking as the second cause of mortality in this patient population. RA can affect the lung parenchyma, airways, and pleura. Pulmonary complications are directly responsible for 10%–20% of all mortalities in RA patients. Spirometry is a tool in monitoring pulmonary function abnormalities in patients with RA. Abnormalities detected by pulmonary function tests may precede symptoms by years and lead to early diagnosis of pulmonary fibrosis in RA and hence intervention.

Objective: The objective was to identify and describe the pattern of pulmonary function abnormalities in RA patients attending rheumatology clinic in Gauhati Medical College and Hospital.

Methods: Spirometry was performed in fifty patients of RA who met with American College of Rheumatology criteria. Pulmonary function tests were performed using Spirolab III according to the American Thoracic Society recommendations. Lung parameters such as forced vital capacity, forced expiratory volume in 1 s, forced expiratory flow in 25%–75%, and peak expiratory flow rate were measured.

Results: Patient recruitment is not yet been completed.

Conclusion: Pulmonary affection in various spectra does involve a sizeable percentage of people with RA and can have significant bearing on treatment regimen and overall quality of life of these patients. Screening of RA patients with spirometer helps in the early detection of pulmonary involvement.


  PC0021: Preeclamptic toxemia due to antiphospholipid syndrome and evolving lupus: A catastrophe in pregnancy Top


Ratul Ghosh, Kaushik Basu; Medical College and Hospital, Kolkata, West Bengal, India

Case Report: A 36-year-old elderly primi (32nd week of gestation) presented with hypertensive emergency and absent fetal movement. Her antenatal period was uneventful until the 6th month when she was found to be hypertensive. From the 7th month onward, she experienced orthopnea, gradually progressive pedal swelling, which was followed by facial puffiness, and eventually anasarca. On examination, she had features of preeclamptic toxemia. Initial investigations showed evidence of hemolysis, elevated liver enzymes, and thrombocytopenia, suggestive of hemolysis, elevated liver enzymes, and low platelet syndrome. Hypertensive emergency was managed with injection labetalol and emergency cesarean section was performed. She delivered a stillborn fetus. Anemia, thrombocytopenia, and elevated liver enzymes improved after lower-segment cesarean section without active intervention. Further investigations showed immunoglobulin M B2 glycoprotein 1 and lupus anticoagulant positivity. Her antinuclear antibody was positive, and C3 and C4 levels were low. Anti-dsDNA titer was high, and various extractable nuclear antigen components were positive. 24-h urinary protein was in nephritic range. Kidney biopsy was suggestive of preeclamptic renal involvement.

Discussion: Antiphospholipid syndrome (APS) is a disorder of recurrent vascular thrombosis and/or pregnancy losses associated with persistently elevated antiphospholipid protein antibodies. It can be primary or secondary. Pregnancy morbidity due to preeclampsia has been included in revised Sapporo Classification Criteria for APS. Pregnancy loss, fetal distress, premature birth, and preeclampsia are frequent complications in women with APS, mostly in the 2nd/3rd trimester (50% cases).

Conclusion: Preeclamptic toxemia carries a poor fetal prognosis but relatively good maternal outcome with timely intervention, as evident in our case. In India, the incidence of preeclampsia is reported to be 8%–10% among pregnant women. Here, it was due to APS (probably secondary to lupus) and timely intervention saved the mother's life.


  OPC0045: Epstein–Barr virus infection mimicking autoimmune diseases require greater clinical suspicion: A case series Top


Shivputra ghanti, S Chandrashekara, Devaraj Kori, Bharathraj, P Renuka; ChanRe Rheumatology and Immunology Center, Bengaluru, Karnataka, India

Introduction: Autoimmune diseases have complex multifactorial etiology comprising of genetic, hormonal, and environmental factors. The clinical presentation of Epstein–Barr virus (EBV) acute infection or its reactivation is highly variable and may often mimic an autoimmune disease. The present case series discusses various incidences of EBV infection, noted at our institution, with similar clinical presentations as that of autoimmune diseases.

Methodology: Patients suspected with autoimmune diseases and also showing clinical signs and symptoms of probable viral infection, including EBV virus, were considered. Diagnosed autoimmune cases with abnormal natural course of disease on treatment were also included. Antinuclear antibody profile, EBV virus serology, and nuclear antigen studies were carried out for all the recruited patients and the results were analyzed.

Results: The case series considered patients recruited between August 2017 and 2018. Eighteen patients were suspected with EBV infection. Among these, nine patients had convalescent phase of EBV infection and one had active phase of infection. The following were the diagnoses made: two with rheumatoid arthritis (one had acute cardiac tamponade); one with primary biliary cirrhosis, presented as acute interstitial pneumonia; two with Sjogren's syndrome (one presented as acute interstitial pneumonia, active-phase EBV); one with erythema nodosum; and two patients each with lupus and dermatomyositis-like presentation. Disease-modifying antirheumatic drugs were withdrawn for all the patients. Two patients with acute interstitial pneumonia and one with dermatomyositis were treated with valganciclovir for 21 days. All the three patients demonstrated significant improvement following valganciclovir treatment. Other patients had complete resolution of their clinical features including regression of the antibodies.

Conclusion: Patients having atypical presentation of autoimmune diseases and shorter duration and unnatural disease course should be screened for EBV virus infection. Immunosuppression should be deferred in these patients.


  OPC0051: Correlation of Functional index, Metrology index, and radiological findings in spondyloarthritis Top


Subhankar Das; Srimanta Sankaradeva University of Health Sciences, Guwahati, Assam, India

In this study group of patients those have high Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI) and Bath Ankylosing Spondylitis Metrology Index (BASMI) scores reflecting high disease activity with greater functional limitations, whereas BASMI scores is more sensitive than BASDAI and BASFI. Those having high BASMI scores having more m-MASSS scores and sacroiliitis. On comparison, total m-MASSS is 27.90 ± 18.66 in human leukocyte antigen (HLA)-B27-positive patients and 19.76±16.40 in HLA-B27–negative patients. The m-MASSS index is higher in lumbar spine (57.80%) when compared with that of cervical spine. Bilateral sacroiliitis was found in 75.38% of cases when compared to unilateral sacroiliitis. Highest sacroiliitis incidence was found in ankylosing spondylitis patients. Highest sacroiliitis (67.69%) incidence was present when BASMI score is ≥6. On comparison, five patients with BASMI score <6 had magnetic resonance imaging (MRI) evident of sacroiliitis, whereas eight patients with BASMI score >6 had MRI evident of sacroiliitis. Longer duration of disease resulted in a worse BASDAI, BASFI, and BASMI with high m-MASSS index score.


  OPC0049: A rare case of systemic lupus erythematosus with Broca's aphasia and insular infarct Top


Swati Parida, Soumya Ranjan Tripathy1, Manoj Parida, Bidyut Kumar Das1; Departments of General Medicine and 1Rheumatology, SCB Medical College, Cuttack, Odisha, India

Case Report: Neuropsychiatric disorders are important clinical manifestations of systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). The prevalence of stroke in SLE varies from 2% to 19%. Association of SLE with antiphospholipid antibodies determines an increased risk of ischemic cerebrovascular events (CVEs). We report the case of a38-year-old female patient who presented with an inability to speak for 5 days associated with deviation of angle of mouth to the right side. She was a known case of SLE nephritis, arthritis, mucocutaneous for the last 1 year on stable treatment. She had a spontaneous abortion 15 days before at 12 weeks of gestation. General examination revealed a pulse rate of 88/min and blood pressure of 130/80 mmHg. Examination of the central nervous system revealed Broca's motor aphasia. Comprehension was intact. She also had right upper motor neuron-type facial palsy. Examination of cardiovascular, respiratory, and abdominal systems was unremarkable. Laboratory investigations revealed the presence of lupus anticoagulant-normalized ratio of 1.79 (normal <1.2), positive β2-glycoprotein 1 immunoglobulin (Ig)M of 27.32 SMU (normal <20 SMU), cardiolipin antibody IgM of 19.14 MPL (normal <12.5 MPL), and positive direct Coomb's test; antinuclear antibody profile showed 4+ speckled pattern and extractable nuclear antigen profile showed RNP/Sm++, Sm++, dsDNA+, and nucleosomes++. C3 and C4 were reduced. dsDNA was >300 U/ml. Routine urine microscopy revealed 1–2 red blood cells/high-power field. Echocardiography showed mild pulmonary arterial hypertension with tricuspid regurgitation. Erythrocyte sedimentation rate was 50 mm at the 1st hour and C-reactive protein was 3.1 mg/L (normal <6 mg/L). Computed tomography scan of the brain revealed left insular cortex infarct. She was treated with prednisolone 30 mg/day, warfarin 5 mg/day, low-molecular-weight heparin, and hydroxychloroquine. She recovered completely.

Discussion: Risk of CVE in SLE increases with the presence of vasculitis, APS, hyperviscosity, and hypertension. Recent studies have shown that damage to left anterior insula due to occlusion/narrowing of middle cerebral artery causes apraxia of speech.

Conclusion: We report an uncommon clinical presentation of SLE with secondary APS. A target international normalized ratio of 3–4 is aimed in patients with ischemic stroke and APS.


  OPC0059: Evaluation of reticulocyte haemoglobin for assessment of anaemia in rheumatological disorders Top


Bhandari Gurbir Singh, Lalit Duggal, Ved Chaturvedi, Jyoti Kotwal, Neeraj Jain, Jeet Patel, Mayank Gupta; Departments of Clinical Immunology and Rheumatology and Pathology, Sir Ganga Ram Hospital, New Delhi, India

Objective: The aim of this study was to evaluate the role of reticulocyte hemoglobin (Ret He) estimation in subtyping of anemia and to find the correlation of Ret He with the severity of anemia in patients suffering from rheumatic disorders.

Methods: A total of 94 patients of rheumatic diseases with anemia were enrolled. Blood samples were taken to determine various laboratory parameters. Patients were divided into three groups, i.e., iron deficiency anemia (IDA), anemia of chronic disease (ACD) with iron deficiency, and ACD groups, depending on the iron status and inflammatory markers. Analysis of variance test was used to find out the significance regarding various variables among different groups. Pearson's correlation coefficient was used for studying the relationship. Receiver operating characteristic analysis was used to evaluate the accuracy of the parameters in differentiating anemia.

Results: Statistically significant difference among different groups was seen with regard to parameters such as erythrocyte sedimentation rate, C-reactive protein, serum ferritin, total iron-binding capacity, transferrin saturation, transferrin receptor protein, serum transferrin receptor/log ferritin, and Ret He. Ret He correlates with the subtype of anemia in patients with rheumatic disorders, but it does not correlate with the severity of anemia. Serum tryptophan /log ferritin, Ret He, and serum ferritin values were the best parameters to differentiate between various groups. Ret He (pg) values of <24, 24–26.5, and >26.5 and serum ferritin levels (μg/l) of <35, 35–178, and >178 were highly sensitive and specific for IDA, ACD with iron deficiency, and ACD groups, respectively.

Conclusion: In a country like ours where cost constraints are the foremost issue, a simple and quick investigation like Ret He alone or with serum ferritin can help us to diagnose and differentiate between the different types of anemia accompanying rheumatological disorders without doing other serum iron studies and expensive tests such as transferrin receptor protein which are not readily available.


  OPC0191: Lupus pneumonitis mimicking as tuberculosis in a case of cutaneous lupus erythematosus: A diagnostic and therapeutic challenge Top


Taraknath Chattopadhyay, Pramit Maji, Syamal Kundu; Department of General medicine, Bankura Sammilani Medical College and Hospital, Bankura, West Bengal, India

Lupus erythematosus (LE) is a chronic autoimmune disorder that can have varied clinical presentations ranging from localized skin lesion of cutaneous LE (CLE) to systemic LE (SLE), which causes severe multisystem involvement. Nearly 5% of CLE patients can have SLE. On the other hand, 20% of SLE patients have cutaneous manifestation of LE. Lupus pneumonitis (LP) is a rare and life-threatening complication of SLE usually not found to be associated with cutaneous variety. We report the case of a 27-year-old female patient of known cutaneous LE who was on treatment for the last 2 years presented with features of pneumonia after stoppage of her therapy. Repeated negative sputum staining and culture along with rapid deterioration of patient's clinical condition despite aggressive antimicrobial implementation renders us to proceed for a presumptive antitubercular therapy due to high incidence of the same in our country. In view of the progressive nature of disease course and interstitial pattern of pneumonia, we kept LP as the closest differential. After failure of antitubercular therapy to produce any significant improvement, we commenced pulse methyl prednisolone in which the patient showed dramatic improvement both clinically and radiologically. In a country like ours where the prevalence of tuberculosis is high, diagnosis of LP becomes extremely difficult due to the stark similarities between the two. Moreover, the treating physician often faces the dilemma in commencing immunosuppressive therapy due to its potential of catastrophic consequences in case the clinical picture is of infective origin. Therefore, a high index of suspicion is required to diagnose LP to prevent its dreadful outcome.


  OPC0193: Physicians' perception of rheumatology practice and training in India Top


Vikas Agarwal, Durga Prasanna Misra, Vinod Ravindran1, Aman Sharma2, Anupam Wakhlu3, Vir Singh Negi4, Ved Chaturvedi5; Department of Clinical Immunology, SGPGIMS, 3Department of Rheumatology, KGMU, Lucknow, Uttar Pradesh, 1Center for Rheumatology, Kozhikode, Kerala, 2Department of Internal Medicine, Clinical Immunology and Rheumatology Services, PGIMER, Chandigarh, 4Department of Clinical Immunology, JIPMER, Puducherry, 5Department of Rheumatology and Clinical Immunology, GRIPMER, New Delhi, India

Objective: The objective was to assess physicians' perception and their felt competence in dealing with patients with rheumatic complaints.

Methods: We assessed the quantum of rheumatological disorders seen by physicians in India, their felt competency in dealing with such patients, and their perceived adequacy of undergraduate and postgraduate medical training in rheumatology by means of an anonymized questionnaire conducted at the annual national conference of internal medicine specialists.

Results: Our analysis of 333 respondents revealed that while they saw an average of ten patients with rheumatic complaints every month, the felt competence in dealing with such cases was only a median of 6/10 (interquartile range: 5–7). About 75% professed little or no exposure to rheumatology as undergraduates, whereas only 20% perceived adequacy of training during internal medicine residency to treat such diseases confidently. Nearly 78.37% and 67.7% perceived an inadequacy of rheumatology training at undergraduate and postgraduate levels, respectively [Figure 1], and 83% felt the need for further training or sensitization in rheumatology.



Conclusion: There remains an unmet need to enhance the existing undergraduate and postgraduate internal medicine curricula in India to impart greater skills in the diagnosis and management of rheumatic diseases.

Initiatives and government funding to establish short-term training courses in rheumatology for established internal medicine faculty, to enable them to provide basic rheumatology services at their respective hospitals, are urgently needed.


  OPC0047: A study of insulin resistance in patients with systemic lupus erythematosus Top


John Kumar Das, S M Baruah Assam Medical College, Dibrugarh, Assam, India

Background: Insulin resistance (IR) is an important contributor to the increased cardiovascular risk attributed to metabolic syndrome, a constellation of cardiovascular risk factors that includes central obesity, dyslipidemia, hypertension, and disturbed glucose metabolism, in patients with systemic lupus erythematosus (SLE).

Objectives: The aim of the study was to study IR in patients with SLE and its association with disease activity.

Methods: The study included 100 SLE cases and 100 healthy age- and sex-matched controls. Disease activity (Systemic Lupus Erythematosus Disease Activity Index score), disease duration, and 24-h proteinuria were determined in cases, and body mass index (BMI), waist–hip ratio, blood pressure (BP), C-reactive protein, erythrocyte sedimentation rate, fasting lipid profile, fasting plasma glucose (FPG), and fasting serum insulin (FSI) were measured in cases and controls. The Homeostasis Model of Assessment was used to evaluate IR.

Results: The case and control groups were similar in baseline parameters such as age, sex, BMI, and waist–hip ratio. SLE patients had higher BP, high-grade systemic inflammation, and hypertriglyceridemia compared with controls (P < 0.05). The mean ± standard deviation of FPG and FSI in cases was 92.89 ± 12.95 mg/dl and 15.44 ± 14.89 μIU/ml and that in controls was 88.00 ± 1.41 mg/dl and 9.00 ± 5.66 μIU/ml, respectively. The difference was statistically significant. Around 47% of the SLE cases were having IR compared to 16% of the healthy population. The correlation coefficient of IR with SLEDAI score was 0.6053 (P < 0.05).

Conclusion: The present study demonstrated that SLE patients in this part of the country had higher IR compared to healthy population. There was a positive correlation of IR with disease activity but no correlation with disease duration. No difference was found in IR in cases those who were newly diagnosed not on medication and the diagnosed cases those who were on medication.


  PC0020: Coffee decreases methotrexate intolerance and increases its compliance in autoimmune inflammatory rheumatic diseases: A study by rheumatology nurse counselors Top


S S Baghel, R Thakran, C Messi, V Yadav, S Kapoor, S R Garg, Vivekanand, Q Zaheer, A N Malaviya1; Indian Spinal Injuries Centre, Super Speciality Hospital, 1Department of Rheumatology, Indian Spinal Injuries Centre, Super Speciality Hospital, New Delhi, India

Abstract: Background: Methotrexate (MTX) is universally regarded as the “anchor drug” in the management of autoimmune inflammatory rheumatic diseases (AIRDs). It is usually administered orally or subcutaneously in once-weekly doses. Intolerance is the commonest cause of noncompliance. Adding coffee with MTX increases its compliance by reducing intolerance.

Objectives: The objective was to assess the effect of coffee on MTX intolerance in AIRDs.

Methods: A total of 700 AIRD patients on weekly MTX willing to participate were enrolled in this study. Patients who had MTX intolerance were counseled and explain to follow the coffee schedule. The coffee schedule advised was as follows: 1st dose – 2 strong cups of coffee early in the morning on the day of MTX, the 2nd dose was given late evening 1 h before MTX, and the 3rd dose was repeated the next morning; this schedule was repeated every week synchronized with the weekly dose of MTX. All the information were collected in a predesigned form.

Results: A total of 700 patients were enrolled in this study. All the patients were treated with weekly dose of MTX. Among 700 patients, 385 (55%) patients did not have any MTX intolerance and 315 (45%) had MTX intolerance. Out of the 315 patients, 150 (47.61%) patients had minimal intolerance not requiring any intervention, 165 (52.38%) patients had moderate or severe MTX intolerance were added coffee.

Result after coffee intervention:

  • 75 (45.45%) participants became asymptomatic
  • 35 (21.2%) had partial improvement
  • 20 (12.1%) showed slight improvement
  • 15 (9.09%) had no relief with coffee
  • 20 (12.1%) did not like coffee.


If the intolerance symptoms disappeared completely over time, the patient was advised to discontinue coffee unless the patient liked coffee and preferred to continue taking it.

Conclusion: Coffee relieved the symptoms of MTX intolerance in 45.5% and partial relief in 21.2% of the patients. With the help of intense counseling by the specialist rheumatology nurses about coffee treatment in MTX intolerance reduces intolerance and increases its compliance in patients.


  OPC0192: Tuberculosis: Mimicker of vasculitis Top


Vijaya Prasanna Parimi; Consultant Rheumatologist

Infective causes have been recognized more recently as an important differential diagnosis in patients presenting with features of systemic vasculitis. We report a case of a young female who presented with skin lesions, claudication pains of upper limbs, and recent onset of fatigue. On examination, she had nontender, nonblanchable purpuric rash over the upper and lower limbs, absence of upper-limb pulses, bruit over subclavian arteries, high blood pressure, cervical and axillary lymphadenopathy, and high blood pressure. Chest radiograph revealed bilateral upper-lobe cavitary lesions with strong Mantoux positivity. Biopsy of skin lesions showed leukocytoclastic vasculits, and lymphnode biopsy showed acid-fast bacilli. Computed tomography aortogram depicted involvement of subclavian arteries. She was started on antituberculous therapy. Three weeks after treatment, her skin lesions improved completely and also had symptomatic improvement. This is one of the rare presentations of tuberculosis involving skin, lymph nodes, lungs, and large vessels. This serves us to learn the need to consider infection as an important differential as a mimicker of vasculitis before starting immunosuppression in such patients.


  PC0064: Methotrexate route of administration: Perception of the patient Top


R Thakran, S S Baghel, C Messi, V Yadav, S R Garg, S Kapoor, A N Malaviya1 Indian Spinal Injuries Centre, 1Department of Rheumatology, Indian Spinal Injuries Centre, New Delhi, India

Introduction: Methotrexate (MTX) is an anchor drug in the treatment of rheumatoid arthritis. It is usually administered orally or subcutaneously once weekly either oral or injectable.

Objective: The objective was to understand the patient's perception of oral MTX versus injectable and impact on its adherence.

Methods: A total of 200 rheumatoid arthritis (RA) patients in rheumatology outpatient department were inquired about their present route of MTX administration by asking the following questions at follow-up visit:

  • Benefits and drawbacks of their current route of administration
  • Their preference of oral versus injection route
  • Adherence
  • Safety and efficacy expectation.


Results: A total of 135/200 (67.5%) patients preferred oral route, while 65 (32.5%) patients preferred injectable route. The easy oral administration is the commonest reason for the majority of patients to prefer oral route. Patients are reluctant toward injectable route as they have injection phobia and have to go to nearby clinics or hospital for administration. Nearly 70% of patients are more compliant with oral MTX as 30% of patients left injectable form due to the above-mentioned reasons. If given a choice, 87% of patients would prefer switching their current route.

Conclusion: Reasons for which patients prefer oral over injectable route are quite obvious. However, for effective management, it is of utmost importance that we spend a few extra minutes to counsel the patients regarding the benefits of injectable MTX over the oral route. Furthermore, the gastrointestinal adverse effects are much lower with injectable route. The disease gets control better with moderate-to-high disease activity. Thus, a culture of counseling the patients regarding injectable MTX to improve the outcome of treatment of RA should be adopted by the rheumatology clinics worldwide.


  OC0044: Clinical predictors of response to methotrexate monotherapy in rheumatoid arthritis Top


Vikas Gupta, Pradeepta Sekhar Patro, Ramnath Misra, Amita Aggarwal; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: About one-third of patients with rheumatoid arthritis (RA) do not respond to methotrexate (MTX), the first-line therapy in RA. Early identification of responders may allow the use of alternative disease-modifying antirheumatic drugs (DMARDs) in patients unlikely to respond, thus preventing long-term damage.

Objectives: We aimed to identify the clinical predictors of response to MTX in RA with disease duration <5 years.

Methods: This study is a post hoc analysis from three previous studies in which we aimed to identify various biochemical and genetic biomarkers of MTX response in RA. Patients with active RA (fulfilling 2010 American College of Rheumatology/ European League Against Rheumatism (EULAR) classification criteria and having Disease Activity Score [DAS] 28-erythrocyte sedimentation rate [ESR] [3] >3.2) who were naive to DMARDs and had disease duration <5 years were enrolled. Baseline characteristics studied as predictors of MTX response included age, gender, disease duration, rheumatoid factor (RF) positivity, tender joint count (TJC), swollen joint count (SJC), ESR, and disease activity by DAS28-ESR[3]. After 4 months of MTX monotherapy, patients were classified into two groups based on EULAR response criteria: responders (moderate/good response [MTX-R]) and nonresponders (MTX-NR). The data were analyzed using nonparametric tests.

Results: A total of 211 patients were enrolled (167 females, median age: 40 years and median disease duration: 24 months). Median DAS28-ESR[3] was 6.18 (interquartile range, 5.57–6.93). After 4 months of follow-up, 165 patients were classified as MTX-R and 46 as MTX-NR. Among all the parameters studied, age was the only parameter associated with response to MTX. MTX-R group had significantly lower age as compared to MTX-NR (39 [31–46] years vs. 44.5 [36–51] years, P < 0.05). None of the other variables including gender, disease duration, RF positivity, TJC, SJC, ESR, and DAS28-ESR[3] were predictive of MTX response.

Conclusion: Younger patients have higher likelihood of good response to MTX in RA.


  PC0022: Human leukocyte antigen-B27 and ankylosing spondylitis in a tertiary care center in North-East India Top


Madhumita Priyadarshini Das, Ashish Agarwalla; Department of Medicine, Gauhati Medical College and Hospital, Guwahati, Assam, India

Introduction: Ankylosing spondylitis is an inflammatory disorder of unknown cause that primarily affects the axial skeleton; peripheral joints and extra-articular structures are also frequently involved. The diagnostic criteria of this disease have given a lot of weightage to human leukocyte antigen (HLA) B27 positivity. As such, we tried to look into the prevalence of HLA B27 positivity in patients of ankylosing spondylitis in this part of country, where there is no report till date as per our knowledge in the literature.

Aim: This study aimed to see the HLA-B27 positivity in ankylosing spondylitis patients in a tertiary care hospital in Northeast India. This was a hospital-based observational study and the setting was Rheumatology Clinic of Gauhati Medical College and Hospital, Guwahati, Assam, India.

Materials and Methods: A total of 100 patients who were diagnosed to have ankylosing spondylitis, as per the Assessment of Spondyloarthritis International Society criteria were included in the study and the following data were collected: age, gender, clinical profile, X-ray findings/magnetic resonance imaging, and HLA-B27 positivity.

Results: Of the 100 ankylosing spondylitis patients studied, 77 were male and 23 were female. HLA-B27 positivity was in 81 patients, of which 70 patients were male and 11 patients were female. Prevalence of HLA B27 positivity in male was 90.91% and female was 47.83%, which is statistically significant.

Conclusion: The present study shows a quite lower HLA positivity in females with ankylosing spondylitis than reported. The mechanism behind this observation is unclear, so there is a need for more epidemiological research of ankylosing spondylitis, especially in female patients with a large sample size to arrive at a definite conclusion regarding the association of HLA B27 positivity and ankylosing spondylitis in this part of country and its correlation with disease severity.


  PC0025: Rheumatoid arthritis with nodular myositis: A case report Top


Ramakant, Vivek Vasdev, Arun Hegde, Ashwani Kumar, K Kishore, G P S Gehlot, S Bhatt; Army Hospital Research and Referral, New Delhi, India

Case Report: A 50-year-old woman with long-standing seropositive rheumatoid arthritis (RA) for the last 10 years presented with a history of progressive proximal muscle weakness and mild joint pain. She had been on irregular medical follow-up and chronic alternative medications. There was no history suggestive of any other connective-tissue disorder (CTD). Clinically, she had bilateral wrist synovitis and proximal muscle weakness of all the four limbs. Investigations showed raised muscle enzymes, and electromyogram was suggestive of myopathy. Her magnetic resonance imaging thighs showed inflammatory changes in muscles bilaterally, and the percutaneous muscle biopsy showed features of nodular myopathy with few areas of necrosis. Autoantibody profile including myositis-specific autoantibodies was negative. Nail-fold capillaroscopy was also normal. She was diagnosed as a case of RA with myositis and treated with methotrexate and high-dose steroids with normalization of muscle enzymes and improvement in power.

Discussion: RA is primarily considered a disease of the joints, but abnormal systemic immune responses are evident and can cause a variety of extra-articular manifestations. A good number of patients of RA develop loss of muscular strength and endurance, yet muscle involvement is one of the less studied entities among the extra-articular manifestations of RA. There have been few reports of inflammatory myopathy presenting with muscle weakness and increased muscle enzymes in cases of RA.

Conclusion: Occurrence of myositis in RA is a known but rare phenomenon, with few cases reported worldwide. Ours is one such case where the patient had no features of CTDs other than RA with rheumatoid factor and anticitrullinated peptide antibodies positivity in high titers and muscle biopsy showing features of myositis classically described in cases of RA.


  PC0024: A clinical study on neuropsychiatric systemic lupus erythematosus in a tertiary care hospital in Northeast India Top


Bikramaditya Deb, Chitralekha Baruah, Niharika Kutum; Department of Medicine, Gauhati Medical College and Hospital, Guwahati, Assam, India

Introduction: Neuropsychiatric systemic lupus erythematosus (NPSLE) is a disease with central and peripheral nervous system manifestations. The diagnosis of NPSLE is often a diagnostic challenge though the criteria for NPSLE have been well established. Therefore, this study was undertaken to evaluate the pattern of neurological involvement in systemic lupus erythematosus (SLE) and its correlation with disease activity.

Objective: The objective was to study the prevalence and pattern of neuropsychiatric manifestations in SLE.

Materials and Methods: This hospital-based observational study was carried out from November 2017 to June 2018. A total of 58 cases of SLE were assessed clinically and investigated accordingly. Special emphasis was given to look for neurological involvement. Mini–Mental State Examination, Hamilton Anxiety Rating Scale, and Hamilton Depression Rating Scale scales were used for the assessment of cognitive dysfunction, anxiety, and depression, respectively. The disease activity was measured by the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI).

Results: In a total of 58 patients with SLE evaluated, 92% were female. The most common age group was 21–30 years. The mean age was 25.6 years. Nervous system involvement was found in 37 (63.79%) patients. Headache was the most frequent manifestation, present in 32 (55.17%) patients followed by cognitive dysfunction in 8 patients (13.79%). Seizure disorder was present in six (10.34%) patients, acute confusional state in two (3.44%), depression in four (6.89%), anxiety in two (3.44%), and psychosis in two (26.31%) patients. Aseptic meningitis and peripheral neuropathy were found in two (3.44%) and one (1.72%) patients, respectively. Many of the patients had more than one neurological involvement. SLEDAI score was high in SLE patients with neurological manifestations.

Conclusion: The frequency of neuropsychiatric involvement was found in the majority of the patients with SLE, with headache being the most common manifestation. Patients with NPSLE showed high disease activity.


  PC0026: Scleroderma renal crisis: A case report Top


Ramakant, Vivek Vasdev, Arun Hegde, Sonia Badwal, Ashwani Kumar, K Kishore, S Bhatt Army Hospital Research and Referral, New Delhi, India

Case Report: A 73-year-old male, chronic smoker, presented with rapidly progressive skin tightening, breathlessness, proximal muscle weakness, and significant weight loss for 3 months. Clinically, he had pallor, Rodnan score (modified) of 36/51, bibasilar crackles in the chest, symmetrical proximal muscle weakness, and scleroderma pattern on nail-fold capillaroscopy. Investigation showed anemia, normal renal and liver parameters, raised muscle enzymes and interstitial lung disease on high-resolution computed tomography chest, myopathic pattern on electromyogram, and features of myositis in magnetic resonance imaging thighs. The evaluation for malignancy was negative. He was treated with mycophenolate mofetil and high-dose steroids with significant improvement. Three months later, he presented to the emergency department with acute-onset breathlessness, raised blood pressure, and high creatinine levels. X-ray chest showed features of pulmonary edema. He was managed with angiotensin-converting enzyme inhibitors and supportive measures. Incidentally, on examination, he was found to have right submandibular lymph node which turned out to be poorly differentiated metastatic adenocarcinoma. The patient continued to have worsening course despite treatment and finally succumbed to his illness. The renal biopsy done postmortem showed features suggestive of thrombotic microangiopathy.

Discussion: Scleroderma renal crisis (SRC) is the most recognized renal complication. SRC occurs mostly in patients with diffuse scleroderma usually within the first 2–4 years from the disease onset. The incidence quoted in Western literature ranges from 5% to 10% of patients; however, it appears to be very low in India with very few cases reported.

Conclusion: SRC is an uncommonly reported incidence in India. The occurrence of a concomitant malignancy is even rare. We report a case of SRC in an elderly individual who also was coincidentally detected to have poorly differentiated metastatic adenocarcinoma.


  OPC0195: Granulomatosis with polyangiitis: Clinical manifestations, laboratory parameters, and outcome of 26 patients from a single center in Mumbai, Maharashtra, India Top


Singh S R, Oak J.L, Mahin O S, Mathur R; Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Mumbai, Maharashtra, India

Background: Granulomatosis with polyangiitis (GPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated small-vessel vasculitis with a high mortality. Limited data are available on GPA in Indian population.

Objectives: The present study describes the clinical manifestations, laboratory parameters, treatment, and outcome of 26 patients from a single center in western part of India.

Methodology: From our database, patients with a diagnosis of GPA over 8-year period (June 2010–June 2018) were included. Clinical manifestation, laboratory features, treatment, and follow-up data of these patients were analyzed.

Results: Twenty-six patients with GPA were identified. The median age was 50.5 (27–70 years), with a male-to-female ratio of 4:1. Systemic variety of GPA was found in 19 (73.07%) and limited variety in 7 patients (26.9%). Renal involvement was established in ten (38.4%) patients. Pulmonary involvement was seen in 18 (69.2%) patients. Ear, nose, and throat (ENT) manifestations were seen in ten (38.4%) patients. Articular manifestations were seen in five (19.23%) patients. Skin manifestations were seen in three (11.53%) and eye involvement was seen in seven (26.9%) patients. All patients were positive for cANCA/ANCA PR3 except two patients with pANCA positivity. At presentation, anemia was present in 15 patients, leukocytosis in 10 patients, and thrombocytosis in 8 patients. Thirteen (50%) patients showed proteinuria and 17 (65.38%) had hematuria. All patients required steroids and immunosuppressants. Cyclophosphamide was used for induction in 13 patients. Rituximab was used in eight patients. Mean duration of follow-up was 25.6 months (1–72 months). Twenty-three patients are on follow-up and 3 (11.5%) patients expired due to multiorgan dysfunction. Relapse of disease occurred in 11 (42.3%) patients.

Conclusion: GPA, though uncommon, should be suspected in patients with specific ENT, pulmonary, and renal manifestations. Pulmonary involvement is the most common manifestation. Most patients respond to steroids and immunosuppressants. Relapses are common and rituximab is an effective therapy in this group of patients.


  OPC0058: 2 Young Boys with Neck Swellings Top


Taral Parikh, Sapan Pandya1, Sunil Sharma1; Department of ENT, Gujarat Cancer Society Medical College, 1Vedanta Institute of Rheumatic Diseases, Ahmedabad, Gujarat, India

Case 1: A 17-year-old boy presented with the complaint of recurrent painless parotid, submandibular, and anterior neck swellings for 3 years. Multiple soft, fluctuant, nontender swellings were palpable in bilateral retro-auricular and parotid regions. Fine-needle aspiration cytology (FNAC) of the mass was reported to be reactive.

Case 2: A 21-year-old male presented with 12 years of recurrent head-and-neck swellings. In 2005 and 2012, he had painless left postauricular swellings. FNAC showed mixed inflammatory infiltrate. He was symptomatic for 1 year, with a persistent swelling in the bilateral posterior cervical and retro-auricular regions. Soft fluctuant swellings in both retro-auricular regions were palpable. In both patients, investigations were normal; eosinophilia of 20% was noticeable. Chest X-ray, viral serology, antinuclear antibody (IF), and anti-neutrophil cytoplasmic antibody (IF) were negative. Both responded to steroids. Excisional biopsy showed germinal center formation with follicular hyperplasia, eosinophilic infiltrate, and eosinophilic abscesses. No vasculitis, necrosis, granuloma, or malignancy was seen. There were no systemic features, lymphoma, infectious symptoms, connective-tissue disease features or atopy, allergies, or asthma. The diagnosis was consistent with Kimura disease. The patient was started on 0.5 mg/kg prednisolone and methotrexate 15 mg. At 6 months, the first patient is in remission and the second is well on the first follow-up.

Discussion: Kimura disease is a chronic inflammatory disorder presenting as painless lymphadenopathy or subcutaneous masses in the head-and-neck region. Most cases are seen in Asia, between 20 and 30 years, with a male predominance. Biopsy is characteristic and necessary for diagnosis.

Conclusion: In our setting, this etiology should be entertained in young patients with unexplained masses in the head-and-neck region.

References

  1. Kimura T, Yoshimura S, Ishikawa E. On the unusual granulation combined with hyperplastic changes of lymphatic tissues. Trans Soc Pathol Jpn 1948;37:179-80.
  2. Chen H, Thompson LD, Aguilera NS, Abbondanzo SL. Kimura disease: A clinicopathologic study of 21 cases. Am J Surg Pathol 2004;28:505-13.



  OPC0196: Is there a need to relook at the cutoffs of rheumatoid factor in Indian population? Top


Vivek Vasdev, Saroj K Patnaik, Ramakant Singh, Ashwani Kumar, Arun Hegde, Kunal Kishore; Department of Rheumatology and Clinical Immunology, Army Hospital Research and Referral, New Delhi, India

Background: Population-specific cutoffs of titers of rheumatoid factor (RF) in the diagnosis of rheumatoid arthritis (RA) and the role of anticitrullinated peptide antibody (ACPA) remain unknown.

Objectives: The objective was to define cutoffs for RF titers in the diagnosis of RA in Indian population.

Methods: RF titers of consecutive adult RA patients fulfilling the American College of Rheumatology (ACR) 1987 as well as ACR/ European League Against Rheumatism (EULAR) 2010 criteria were compared with that of healthy normal and diseased non-RA controls encountered in the rheumatology outpatient department using receiver operating characteristic (ROC)-area under curve (AUC) analysis. Reclassification of disease phenotype as seropositive or seronegative RA using various cutoffs was analyzed.

Results: Overall, 589 cases of RA (range: 18–69 years; 29.9% females) were compared with age- and sex-matched 192 non-RA and 51 controls. Mean (+standard error) RF titers in RA disease, non-RA diseases, and healthy controls were 107.7 IU/L (+6.17), 29.3 IU/L (+6.08), and 14.7 IU/L (+0.43), respectively. ROC analysis revealed a cutoff titer of 20.3 IU/L (AUC 0.705 [95% confidence interval: 0.66–0.74]), with the best combination of sensitivity and specificity for a diagnosis of RA from non-RA and healthy controls. With the currently used cutoffs of 60 IU/L in our center as well as high-titer RF as per the ACR/EULAR 2010 criteria, seropositivity was found in 286/589 (48.5%) cases. Cutoffs of 40 IU/L and 20 IU/L led to a label of seropositivity in 322 (54.7%) and 396 (67.2%) cases, respectively. ACPA positivity in RA cases was 75.6%. Using a RF cutoff of 60, 40, and 20 IU/L, ACPA positivity was noted in 86%, 86.3%, and 84.3%, respectively.

Conclusions: For this cohort of Indian population, a cutoff of 20 IU/L of RF titers has the best performance for a diagnosis of RA with an additional 18.7% cases labeled as seropositive as against the current ACR high titer cutoffs.


  OPC0062: Safety of biosimilar adalimumab in real-life scenario in Indian patients with rheumatoid arthritis Top


Ajit Nalawade, Shashank Akerkar, Smruti Ramteke, Bimlesh Pandey, Vinod Ravindran Sancheti Institute, Pune, Maharashtra, India

Background: This is a retrospective observational study to assess the safety of adalimumab (ADA) biosimilar in patients with rheumatoid arthritis (RA) in clinical practice.

Methods: RA patients fulfilling 2010 American College of Rheumatology (ACR) classification criteria on ADA biosimilar therapy for at least 3 months were included. Patients were analyzed with a questionnaire. Past and present records from the clinic were reviewed. Adverse events (AEs) were tabulated by three time windows after the first ADA injection and analyzed per 100 patient-years of ADA exposure.

Results: The cumulative exposure to ADA biosimilar was 33.3 patient-years. Of the total 64 patients, 38 (59.4%) were on methotrexate, 10 on sulfasalazine, 2 on LEF, and 14 on combination disease-modifying antirheumatic drugs (DMARDs) along with ADA biosimilar. Twenty-four (37.5%) patients were on concomitant low-dose steroids, seven patients discontinued ADA due to serious adverse events (SAEs), one patient had acute myocardial infarction at 2 months of ADA therapy resulting in death, and six patients had severe infections needing hospitalization, of which one was diagnosed to have tuberculosis. All the seven patients with SAE had disease duration >4 years and were on small-dose glucocorticoids. Eight patients had mild AEs, mostly infections. AEs within 1 month of ADA were infrequent and included headache and raised blood pressure. The incidence of SAE was 21/100 patient-years of ADA exposure. The cumulative risk of AE was 45/100 patient-years of ADA biosimilar exposure.

Conclusion: Initial therapy with ADA biosimilar was well tolerated. Most AEs were mild-to-severe infections. The exact causality of SAE was difficult to ascertain due to other risk factors such as long disease duration, concomitant steroids, comorbidities, and other DMARDs. The study has limitations since it is a retrospective observational study with small patient number and short duration of treatment. In our study with ADA biosimilar treatment, no new safety concerns were identified.


  OPC0136: A study to know the prevalence of metabolic syndrome in patients with rheumatoid arthritis Top


Rubi Kumari, P Dihingia, A Chakraborty, P Goswami; Assam Medical College and Hospital, Dibrugarh, Assam, India

Background: Rheumatoid arthritis (RA) is a systemic inflammatory disorder characterized by chronic symmetric and erosive synovitis. The interest in identifying metabolic syndrome in patients with RA has emerged recently, justified by the need to better understand the determinant factors of cardiovascular disease (CVD) in these patients.

Objectives: (1) To assess the prevalence of metabolic syndrome according to the NCEP-ATP III definition in patients with RA. (2) To assess the relationship between disease activity, duration of RA, and presence of metabolic syndrome.

Materials and Methods: The study was conducted in Assam Medical College and Hospital, Dibrugarh, over a period of 1 year from May 2011 to April 2012. Seventy-two patients of RA classified by the American College of Rheumatology/European League Against Rheumatism 2010 Criteria and 72 age- and sex-matched controls (1:1) were included in the study.

Results: Of 72 patients, 66 were female and six were male with a ratio of 11:1. Majority of the cases (52.8%) had moderate disease activity, i.e., 3.2–5.1 according to the disease activity score 28 (DAS28). Based on the NCEP-ATP III Criteria, 12 (16.7%) patients and 5 (6.9%) controls have metabolic syndrome (P = 0.07). Metabolic syndrome was found to be more common among the RA cases with disease duration <5 years. Taking DAS28 as categorical variables, no significant relationship was found between disease activity and metabolic syndrome.

Conclusion: The study observations show that although there is no significant prevalence of metabolic syndrome among the cases of RA in comparison to age- and sex-matched controls, there is definite earlier presentation of metabolic syndrome among the cases, which has some implications as RA patient has less life expectancy than general population and the most common cause of death is CVD. In this study, any relationship of disease activity or duration of the disease with metabolic syndrome cannot be established, due to the limitation of the study.


  PC0039: Clinical profile of sarcoidosis at a tertiary health care center in Kerala Top


B Harikrishnan, C B Mithun, Jyothi Srikanth; Department of Clinical Immunology and Rheumatology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Background: Sarcoidosis is a systemic inflammatory disease of unknown etiology. In India, sarcoidosis was an underdiagnosed disease. All ethnic groups in the Indian subcontinent are affected by sarcoidosis.

Objective: The objective of the study is to describe the clinical profile of sarcoidosis at a tertiary care center in Kerala.

Results: Thirty-four patients (9 males and 25 females) who were diagnosed with sarcoidosis over a period of 4 years were studied. The mean age was 54.9 years. The mean duration of symptoms was 13.03 months. Of these patients, 14.7% were previously treated with antituberculous treatment. The average duration of symptoms was 13.03 months. The common presenting complaints were cough 43.75%, dyspnea on exertion 37.5%, weight loss 53.12%, fever 31.25%, joint pain 43.75%, skin rash 25%, and eye symptoms 40.6%. Two patients presented with altered behavior and two patients had history of Bell's palsy. 21.8% had peripheral lymphadenopathy and 34.3% had arthritis. Of them, ankle joint was commonly involved. 21.8% of patients had uveitis; lung signs were present in 12.5% of patients. Two patients had parotid gland enlargement and one patient had lacrimal gland enlargement. Hypercalcemia was found in 25%, raised angiotensin-converting enzyme (ACE) in 53.12%, and raised alkaline phosphatase in 34.3% of patients. On X-ray, Stage 1 sarcoidosis was common (37.5%). Biopsy was done in 24 (75%) patients. 23 (95.8%) were consistent with noncaseating granuloma and one was inconclusive. All patients with hepatic sarcoidosis had raised ACE levels; three out of four patients with neurological manifestations had raised ACE. Thoracic sarcoidosis was diagnosed in 35.2%, lymph node sarcoidosis in 29.4%, and arthritis in 32.2%. Five patients had hepatic sarcoidosis, four had neurological involvement, two had cardiac sarcoidosis, and one had choroid granuloma.

Conclusion: The overall clinical profile of sarcoidosis in the present cohort was similar to the series reported from other parts of India and the West.


  OPC0197: POEMS syndrome in a young male successfully treated with stem cell transplantation Top


Shah I, Tulpule S, Oak J; Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Mumbai, Maharashtra, India

A 24-year-old male presented with lower limb weakness for 3 weeks which had progressed over the last 3 days and started involving upper limbs. He had preceeding history of undergoing radiotherapy for right iliac bone plasmacytoma and was declared as cured. On general examination, there was wasting of masseter and temporalis muscles, bilateral gynecomastia, and hyperpigmentation over the chest and lower limbs with enlarged axillary and iliac lymph nodes. On central nervous system examination semidilated right pupil with preserved extraocular movements and light reflex. Lower limb power was 0/5 and hand grip was weak; deep tendon reflexes were absent in the lower limbs and plantars were mute.

Magnetic resonance imaging revealed progression of plasmacytoma and lymph node biopsy revealed infective etiology and lymphoma. Nerve conduction velocity showed demyelinating neuropathy in the upper limbs and axonal neuropathy in the lower limb. Ophthalmology examination showed right eye papilledema. Cerebrospinal fluid was normal. Blood investigations showed B2 microglobulin-3.07 mg/ml (0.81–2.19), IgG-2010 mg/dl (751–1560), free kappa-40 mg/L (3.3–19.4), free lambda-61.34 mg/L (5.71–26.30), ratio 0.65 (0.26–1.65), serum electrophoresis-M band, estradiol E2-101 pg/ml (11–44), and testosterone-130 ng/dl (142–923). Chemotherapy with bortezomib, cyclophosphamide, and dexamethasone was started. After four cycles, he underwent stem-cell transplant and his lower limb power improved to 5/5 and blood investigations also showed improvement.

Discussion: This case report is an atypical presentation of a POEMS syndrome as the classical syndrome is seen in the fifth or sixth decade. The clinical symptom is ascending symmetrical demyelinating polyneuropathy. Vascular endothelial growth factor is responsible for papilledema. Organomegaly can present as hepatosplenomegaly or lymphadenopathy. Endocrinopathy involves four major axes (gonadal, thyroid, glucose, and adrenal). The M protein most frequently seen is immunoglobulin A and G. Skin changes are in the form of hyperpigmentation resembling scleroderma.

Conclusion: POEMS syndrome is a paraneoplastic syndrome associated with clonal plasma cell neoplasm. Misdiagnosis is common due to its rarity and complexity. Patients present with multisystemic manifestations. Early diagnosis is vital to improve survival rate.


  OPC0109: Non ischemic cardiomyopathy:role of immunology work-up and cardiac mri in etiologic diagnosis Top


Deepak Malgutte, Sameer Sarda, Irawati Waghmare, Abhilasha Manwatkar, Lalna Kalekar, Yojana Gokhale; Department of Medicine, Lokmanya Tilak Medical College, Mumbai, Maharashtra, India

Introduction: Etiology of nonischemic cardiomyopathy (NICMP) is not studied in detail.

Objective: The objective is to study the etiology of NICMP and the role of cardiac magnetic resonance imaging (MRI) in diagnosis.

Methods: This was a prospective observational study. Inclusion criteria: (1) Clinical feature of cardiac failure, (2) 2D echocardiography (2DE) systolic dysfunction, ejection fraction (EF) <45% OR diastolic dysfunction without regional wall motion abnormality, (3) absent ischemic ST-T changes on electrocardiography and/or coronary angiography. Exclusion criteria: Valvular and congenital heart disease, cor pulmonale, renal failure. Patients were subjected to complete blood count with absolute eosinophil count, erythrocyte sedimentation rate (ESR), urine-r/m, NT-Pro-BNP, antinuclear antibody (ANA), antineutrophil cytoplasmic antibody (ANCA), angiotensin-converting enzyme (ACE), bone marrow, amyloid fat pad biopsy, chest X-ray, and 2DE. High-resolution computed tomography chest and coronary angiography, cardiac MRI by 3 Tesla MRI machine. Patients were treated with antic-failure drugs and as per the etiology and followed at 6 weeks clinically (NYHA) and 2DE.

Results: Thirty patients, with a mean age of 36 years having a female:male ratio of 17:13, were included; 22 patients had other features such as Raynaud's phenomenon (2), joint pain (4), oral ulcer (3), leg ulcer (1), and recent delivery (4). ESR elevated in 19 patients (63%), urine r/m abnormal-8 patients (26%), eosinophilia-3 patients (10%), hypothyroidism-5 (16%), ANA positive-3 (10%), and ANCA-2 (7%) patients. ACE in 1 out of 22 patients Diagnosis established in 23 patients as per table. Raised ESR with P = 0.0001 and abnormal urine r/m predicted systemic cardiomyopathy. Cardiac MRI detected 2 HOCMP missed on 2DE, 1 sarcoid (abnormal delayed enhancement, nonnecrotic mediastinal lymph node), and scarring (predictor of poor prognosis). Out of 30 patients, 17 received additional specific treatment with clinical NYHA grade symptom (P = 0.001) and EF improvement (P = 0.0023) by 20% in 6 and >10% in 5, as compared to idiopathic dilated cardiomyopathy patient on antifailure treatment only.

Conclusion: (1) For etiology of cardiac failure features such as fever, joint pain, Raynaud's phenomenon, oral/leg ulcer, and high ESR, abnormal urine microscopy points toward systemic immune disease. (2) In cardiomyopathy patient with treatable cause-specific treatment in addition to antifailure drugs give significant clinical and EF improvement.


  OPC0108: Outcome of ILD in patients with scleroderma: 5 year prospective cohort study Top


Akash Khune, Rohini Samant, Girish Kakade, Yathish GC, Ashok Mahashur; P.D. Hinduja National Hospital and MRC, Mumbai, Maharashtra, India

Background: Systemic sclerosis is a multisystem disorder characterized by a disturbance in fibroblast function, microvascular disease, and immune system activation, resulting in fibrosis of the skin and internal organs.

Objectives: The objective was to study the changes in lung function in patients with scleroderma with interstitial lung disease (ILD) on treatment followed up prospectively over a period of 5 years.

Methodology: In this study, 73 patients with scleroderma with ILD were prospectively followed up from July 2013 to July 2018. Patients' clinical features, investigations, treatment, and complications were recorded at regular interval.



Results: Of 73 patients, 68 were females and five were males. Four had only one visit and 11 had incomplete data. Baseline characteristics are shown in [Table 1]. Mean pulmonary arterial hypertension at baseline was 35.95 ± 14.26 mmHg and at the end of the study was 39.44 ± 14.44 mmHg. Immunosuppressants used were mycophenolate mofetil (MMF) in 52, cyclophosphamide in 15, azathioprine in 13, and methotrexate in 17 patients. Sixty-one patients received oral prednisolone with mean dose of 6.46 ± 7.10 mg/day during maintenance and 15 ± 8.95 mg/day during exacerbation of ILD. There was no statistically significant difference in mean forced vital capacity (FVC) of 59 patients at baseline (59.76 ± 16.91) and at end of the study (57.03 ± 16.77) and in subgroup analysis of patients who received MMF (P > 0.05). Change in FVC in all patients and in patients on MMF is shown in Figures 1 and 2, respectively. Mean 6-min walk distance at baseline was 370 ± 58.83 m and at the end of study was 377 ± 88.96 meters. Comorbidities seen were hypertension in 15, DM in 6, osteoporosis in 23 (one had vertebral fracture), avascular necrosis in 3 patients. Five patients died because of ILD. 13 patients needed home O2.



Conclusion: Lung function either improved or remained stable over 5 years in 78% of all patients and 83% of the patients treated with MMF, suggesting beneficial effect of immunosuppressants, especially MMF, in reducing progression of ILD in scleroderma.


  OPC0202: Economic impact of systemic lupus erythematosus on patients: Study from a government tertiary care hospital Top


Suvrat Arya, Ramnath Misra, Amita Aggarwal; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Systemic lupus erythematosus (SLE) is a chronic disease associated with frequent flares, thus requires regular follow-up and treatment. In a recent systematic review, annual direct costs per patient ranged from US$ 2214 to 16,875, and the mean annual indirect cost from US$ 2239 to 355,401. There are no data from India.

Objective: The objective was to study the annual cost of medical treatment of SLE patients treated at a government hospital in India.

Methods: Patients with SLE with at least 1-year follow-up were enrolled. Besides demographic data and baseline, SLE disease activity index (SLEDAI) information about direct (hospitalization, drugs, investigations, and doctor fees) and indirect cost (travel to hospital, engaging house help, loss of wages, and cost of staying outside the hospital) of medical treatment was collected.

Results: One hundred SLE patients (age 33.8 ± 10.9 years, disease duration 7.9 ± 5.1 years, duration of follow-up 6.5 ± 4.6 years) were enrolled. Mean SLEDAI at baseline was 18.6 ± 7.2. Only 16% of patients were covered by health insurance. The mean annual cost of drugs was Rs. 18,588 ± 15,666 whereas the mean cost of investigations was Rs. 4982± 3762. Among indirect cost, maximum amount was spent on travel to hospital. Mean annual cost of travel was Rs. 6774 ± 6698. Mean total annual direct cost was Rs. 38,867.5 ± 8449.4. Mean total annual indirect cost was Rs. 13,337.9 ± 2370.2. The mean annual cost of drug therapy in patients with nephritis (n = 56; Rs. 21,095 ± 17,298) was higher than those without nephritis (n = 44; Rs. 15,397 ± 12,750; P < 0.05).

Conclusion: SLE causes a significant economic burden on the family, especially in patients with nephritis. Lack of trained workforce in vicinity increases indirect cost related to travel.

Reference

  1. Meacock R, Dale N, Harrison MJ. The humanistic and economic burden of systemic lupus erythematosus: A systematic review. Pharmacoeconomics 2013;31:49-61.



  OPC0087: Correlating disease activity in psoriatic arthritis and Bath Ankylosing Spondylitis Activity Disease Activity Index in axial psoriatic arthritis Top


Nibha Jain, Dhaiwat Shukla, Puja Srivastava, Sapan Pandya; Sheth V S Hospital and Smt. NHL Municipal Medical College, Ahmedabad, Gujarat, India

Introduction: Disease activity in psoriatic arthritis (DAPSA) is a widely used outcome measure in psoriatic arthritis; however, it does not take into account the axial pain and enthesitis. Bath Ankylosing Spondylitis Activity Disease Activity Index (BASDAI) is a patient-reported outcome measure for axial spondyloarthritis (AxSpA) and does not use erythrocyte sedimentation rate or C-reactive protein (CRP). Our objective is to correlate DAPSA and BASDAI in axial psoriatic arthritis (AxPsA).

Methods: This is a prospective longitudinal study of 8-month duration (January 2018 to August 2018) done at a government medical college outpatient department. All patients of AxPsA (CASPAR criteria) were enrolled after informed consent, and baseline and follow-up demographic data were collected along with the calculation of BASDAI and DAPSA. All data analysis was done using SPSS software.

Results:

  • Total number: 24
  • Mean duration of AxPsA (median): 4.6 + 4 (3) years
  • Mean age (median): 40 + 12 (42) years
  • Mean CRP (mg/dl): 12.6+5
  • Mean DAPSA: 24 + 16
  • Mean BASDAI: 4.6 + 1.6
  • BASDAI versus DAPSA correlation coefficient (r) = 0.88.


Conclusion: Our data show good correlation between DAPSA and BASDAI in AxPsA. While BASDAI is a simple tool that has been validated well, it just classifies patients (cut-off 4). DAPSA stages the patients according to disease activity and can be used for follow-up and management of disease even in AxSpA.


  OPC0101: SLE disease activity remains low in patients receiving standard care of treatment and adjunct Vitamin D therapy Top


Mohapatra S, Tripathy R ,Panda AK, Parida M,Tripathy S, Das BK; 1S.C.B Medical College, Cuttack, Centre for Life Sciences,Ranchi, Jharkhand, India

Introduction: Vitamin D deficiency has been associated with various autoimmune disorders (multiple sclerosis, autoimmune type 1 diabetes, rheumatoid arthritis, systemic lupus erythematosus [SLE], etc.). Previous studies have shown negative correlation between serum Vitamin D levels and SLE disease activity.

Aims and Objectives: The objective of the study is to assess disease activity in SLE patients receiving adjunct Vitamin D in addition to standard care of treatment.

Methodology: A total of 251 patients who fulfilled SLICC criteria for SLE, on standard care of treatment and adjunct 1000 IU Vitamin D daily, were included. One hundred healthy controls not receiving Vitamin D were included for comparison. Serum Vitamin D was measured using competitive enzyme-linked immunosorbent assay. SLE disease activity was measured using systemic lupus erythematosus disease activity index-2K (SLEDAI-2K), serum C3 and C4, and serum dsDNA.

Results: Out of 251 patients, 10 were males and 241 females. History of lupus nephritis was present in 64.5%, neuropsychiatric systemic lupus erythematosus was present in 20%, and cardiovascular involvement was present in 4%. The mean duration of the disease was 56.1 ± 41.2 months. The mean serum Vitamin D level in SLE patients (21.3 ± 13.8 ng/ml) was higher than in controls (12.4 ± 10.2 ng/ml) (P < 0.001). The mean SLEDAI-2K score was 2.1 ± 2.43. Serum C3 and C4 levels were 93.3 ± 45.8 mg/dl (normal 75–180 mg/dl) and 18.3 ± 10.97 mg/dl (normal 10–40 mg/dl), respectively. The mean serum dsDNA was 97.95 ± 123.2 IU/ml (normal <100 IU/ml).

Conclusion: Adjunct Vitamin D therapy in SLE helps them to maintain a higher serum Vitamin D level and to suppress disease activity.


  PC0065: Prevalence of thyroid dysfunction in patients with systemic lupus erythematosus Top


Rup Jyoti Sarma, S K Baruah, R P Das; Department of Medicine, Gauhati Medical College and Hospital, Guwahati, Assam, India

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease in which organs and cells undergo damage initially mediated by tissue binding autoantibodies and immune complexes, affecting mostly joints, skin, blood vessels, heart, lungs, kidneys, and nervous system. The course of SLE is variable with flares alternating with periods of remission. SLE is associated with many other autoimmune diseases and autoimmune thyroid disorders too.

Objectives: The objectives were (1) to find out the prevalence of thyroid dysfunction in SLE and (2) to find out the prevalence of thyroid autoantibody (antithyroid peroxidase [TPO]) in SLE.

Methods: A cross-sectional observational study is done to find out the prevalence of thyroid dysfunction in SLE patients admitted in the medicine ward from January to October 2018 and a control group. The patients are submitted to a clinical and laboratory evaluation; the tests included anti-TPO antibody, thyroid stimulating hormone, and free T3 and T4.

Results: High prevalence of subclinical hypothyroidism, clinical hypothyroidism and are detected in the group of patients with SLE when they are compared with the control group. The hyperthyroidism occurs in a similar frequency in the two groups. The positivity of anti-TPO antibodies is higher in SLE patients.

Conclusion: The thyroid disorders and antithyroid antibodys () are more prevalent in patients with SLE than in general population.


  OPC0071: Prevalence of cardiovascular risk in Indian rheumatoid arthritis patients Top


Hafis Muhammed, Sarit Sekhar Pattanaik, Sujata Ganguly, Saurabh Chaturvedi, Harshit Singh, Mohit K Rai, Anamika Anuja, Namita Mohindra1, Neeraj Jain1, Vikas Agarwal, Durga P Misra; Departments of Clinical Immunology and 1Radio Diagnosis, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Rheumatoid arthritis (RA) patients have increased cardiovascular risk (CVR). Prevalence of traditional CVR factors and performances of various CVR scores in Indian RA patients are not yet studied.

Objectives: The study was designed to study the prevalence of traditional CVR factors in RA patients. In addition, the performance of various CVR scores with respect to each other and against subclinical atherosclerosis as measured by carotid intima-media thickness (CIMT) was evaluated.

Type of Study: This was a cross-sectional study.

Methods: Patients fulfilling the 2010 American College of Rheumatology/European League Against Rheumatism criteria for RA were included. Presence of traditional CVR factors was recorded. Ten-year CVR was predicted using five different calculators including Framingham risk scoring using lipids (FRS-lipids), FRS using body mass index, QRISK-3, and the algorithm recommended by 2013-ACC/AHA guidelines (atherosclerotic cardiovascular disease [ASCVD]). CIMT was measured on the far-wall of the common carotid artery at least 5 mm below its end. CIMT value of >0.90 mm or presence of plaque was chosen as a marker of subclinical atherosclerosis.

Results: A total of 111 patients of RA were enrolled in the study. The mean age was 46.45 ± 12.12 years and disease duration was 7.44 ± 6.04 years. A total of 101 were seropositive for either rheumatoid factor or anti-cyclic citrullinated peptide. 59.5% had erosive arthritis. Mean disease activity score (3); erythrocyte sedimentation rate was 3.99% ± 1.3. 49.5% of patients were on corticosteroids, 73% on methotrexate, and 59.5% on hydroxychloroquine at time of the study. The prevalence of various CVR factors is given in [Table 1]. CVR scoring was done in all patients. A Friedman's test for global variance of various CVR scores showed a significant difference among them. Post hoc analysis done with Wilcoxon signed-rank test showed significant variability between each of them. When CIMT was taken as reference, CVR scores with maximum sensitivity were ASCVD (area under the curve [AUC] = 0.739) and QRISK-3 (AUC = 0.729) (n = 62).



Conclusions: CVR scores cannot be used indiscriminately in Indian RA patients. ASCVD and QRISK-3 scores had the maximum sensitivity in predicting subclinical atherosclerosis.


  PC0027: Vasculitis or malignancy or both? Top


Ashish Sharma, Mohammad Ali, Vivek Arya; Fortis Flt. Lt. Rajan Dhall Hospital, New Delhi, India

Behçet's disease is a multisystemic vasculitis which is characterized by recurrent oral and genital ulceration with positive pathergy test. These features may also be seen in various hematological malignancies. In patients with leukemia who present with Behçet's disease-like features, it is often difficult to ascertain whether the clinical manifestations are due to leukemia, or there is coexisting Behçet's disease too. Although there are a few reports in the literature showing coexistence of leukemia with Behçet's disease, the diagnosis of Behçet's disease in those cases is debatable since there is no confirmatory test for the same. We report an Indian farmer who presented with clinical features suggestive of Behçet's disease with profound neutropenia. On bone marrow examination, he was found to be having acute monocytic leukemia -M5.


  OPC0076: Obesity as a confounding factor in raised ESR & CRP levels in clinically inactive RA Top


Ashish Sharma, Ashok Kumar, Alka Jha, Anunay Agarwal; Fortis Flt. Lt. Rajan Dhall Hospital, New Delhi, India

Background: Obesity is associated with raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) in the general population. Hence, it can potentially interfere with the assessment of disease activity in rheumatoid arthritis (RA).

Objective: The objective of the study is to assess the magnitude of confounding effect of obesity on raised ESR and CRP in patients with RA.

Methods: Adult RA patients (2010 American College of Rheumatology criteria) aged more than 18 years and with clinical disease activity index (CDAI) <10 were consecutively recruited from January to July 2018. Low disease activity was defined by CDAI of 2.8–10 and remission at CDAI below 2.8. Patients with RA were divided into two groups: Group A - obese patients (body mass index [BMI] >25), Group B - nonobese patients (BMI <25). A third control group comprising obese individuals without RA was also included. CRP levels and ESR were analyzed in all patients. Patients with any evidence of infection currently or in the past month, major surgery in the past 3 months, another inflammatory disorder (e.g., Sjögren's syndrome, interstitial lung disease, scleritis/episcleritis, rheumatoid vasculitis, overlap with another connective tissue disorder, pregnancy, anemia (hemoglobin <10 g/dL), current smoking, polycythemia, sickle cell anemia, liver disease, malignancy and those on tocilizumab therapy were excluded. Percentage of individuals with raised ESR and CRP in each group was calculated.

Results: A total of 68 obese and 54 nonobese patients with RA and 52 obese controls were included in the study. Among obese RA patients, 47% had raised ESR (>30 mm in the 1st h) and 15.8% had elevated CRP levels (>10 mg/L). In contrast, 25.9% of nonobese RA patients had raised ESR and 4% had raised CRP. Among non-RA obese controls, elevated ESR and CRP were found in 24% and 50% of the patients. Thus, confounding by obesity was observed in 12%–21% of patients with RA.

Conclusion: Obesity may falsely elevate inflammatory markers in as many as 12%–21% of RA patients with low disease activity.


  OC0023: Evaluation of Temporomandibular Joint in children with Juvenile Idiopathic Arthritis by Magnetic Resonance Imaging Top


Zubenthung L. Kithan, T. P. Yadav, Namrita Sachdev; P.G.I.M.E.R. & DR. Ram Manohar Lohia Hospital, New Delhi, India

Background: Juvenile idiopathic arthritis (JIA) is the most common autoimmune musculoskeletal disease occurring in children causing a wide range of disability. Temporomandibular joint (TMJ) arthritis remains one of the most underdiagnosed and undertreated conditions in JIA. Magnetic resonance imaging (MRI) is regarded as the most sensitive imaging modality of diagnosis.

Objectives: The objectives of the study were to access (1) the delineate changes in TMJ by magnetic resonance imaging in children with JIA using juvenile arthritis MRI scoring system (JAMRIS) and (2) the correlation between MRI changes with disease duration and juvenile arthritis disease activity score (JADAS) in JIA patients.

Methods: Thirty patients of JIA were evaluated for clinical involvement of TMJ arthritis, and all the 60 joints underwent contrast-enhanced MRI to look for changes (bone marrow edema, synovial hypertrophy, cartilage erosions, and bone erosions) as per the JAMRIS system.

Results: Of the 18 symptomatic joints, 12 (66.7%) joints showed MRI changes – bone marrow edema in two joints, synovial hypertrophy in 10 joints, and bone erosions in eight joints. Of the 42 asymptomatic joints, only 3 (7.1%) joints showed synovial hypertrophy. Synovial hypertrophy and bone erosions had a significant association with clinical TMJ involvement. The sensitivity, specificity, positive predictive value, and negative predictive value of clinical examination to diagnose TMJ arthritis as compared to MRI taken as gold standard was 80.0%, 86.7%, 66.7%, and 92.7%, respectively. Clinical evaluation of swelling, tenderness, and decreased range of motion of TMJ could detect TMJ arthritis with 85% accuracy as compared to MRI.

Conclusion: JAMRIS system could be used to detect TMJ involvement and evaluate disease activity in JIA patients. No significant association was found between duration of disease activity and JADAS with total MRI score. However, more studies would be required to clearly elucidate the role of JAMRIS of TMJ and its correlation with composite scoring like JADAS in JIA patients.


  OPC0198: A case report of drug-induced hypersensitivity syndrome mimicking as adult-onset Still's disease Top


Mayank Gupta, Lalit Duggal, Ved Chaturvedi, Neeraj Jain, Jeet Patel, Bhandari Gurbir Singh; Department of Clinical Immunology and Rheumatology, Sir Ganga Ram Hospital, New Delhi, India

Introduction: Drug-induced hypersensitivity syndrome (DIHS), also called drug rash with eosinophilia and systemic symptoms (DRESS), is a severe reaction characterized by fever, rash, and multiorgan failure, occurring about 1–8 weeks after drug administration. It is an immune-mediated reaction involving macrophage and T-lymphocyte activation and release of various cytokines, although no consensus has been reached as to its etiology. The skin, hematopoietic system, liver, and kidney are frequently involved. DIHS can mimic severe sepsis, viral infections, adult-onset Still's disease (AOSD), or lymphoproliferative disorders.

Discussion: Here, we describe a case of acute febrile illness with rash, initially suspected as AOSD due to very high level of serum ferritin, raised liver enzymes (>3 times upper limit), sore throat, and lymphadenopathy and with clinical and laboratory parameters fulfilling the Yamaguchi criteria of AOSD. Furthermore, on positron emission tomography-computed tomography scan, there is a diffusely increased uptake of multiple lymph nodes suggestive of a lymphoproliferative disorder. After extensive interrogation, a history of dapsone intake was found 4 weeks before symptoms. In addition, there is persistent eosinophilia with lymph node and bone marrow biopsy was reactive with marked increase in eosinophilic precursors. Finally, diagnosis of DRESS due to dapsone was made. Patient symptoms were improved with intravenous steroids and other supportive treatment. While on steroids, relapsing of symptoms occurred after which N-acetyl cysteine (NAC) was started in twice daily dose. The patient responded dramatically to NAC with decrease in frequency of relapse had occurred.

Conclusion: DRESS can mimic several diseases including AOSD. Careful history taking and clinical examination are useful in diagnosis of such diseases. One should not have relied much on laboratory parameters for making a diagnosis.


  OPC0199: Unusual culprit of septic arthritis Top


Deepak Rath, Parasar Ghosh, Alakendu Ghosh; Department of Rheumatology, IPGMER and SSKM Hospital, Kolkata, West Bengal, India

Case Report: Mr. SG, 67-year-old male is a diagnosed case of diabetes mellitus and hypertension and had undergone coronary artery bypass graft in 2013. He had developed gradual progressive renal failure, necessitating the need for thrice weekly hemodialysis. He had presented with complaints suggestive of monoarthritis of the right knee for the past 1½ months. There was no history of preceding trauma, fever, and swelling of other joints. He had a functioning arteriovenous fistula in his left forearm and had otherwise normal systemic examination. Local examination of the joint had revealed features of inflammation. Ultrasonography evaluation did not show double contour sign. Synovial fluid examination showed the presence of Gram-negative bacteria which on culture was identified as Ralstonia mannitolilytica. The patient was treated with intravenous ciprofloxacin for 2 weeks followed by oral ciprofloxacin for another 4 weeks as per the sensitivity profile. He also underwent daily joint lavage during the 1st week of hospital admission.

Discussion: R. mannitolilytica is nonpathogenic soil microbe, and clinical infection with this pathogen is very rare. However, immunosuppressed individuals remain at risk. Case reports are mentioned of this organism causing bloodstream infection, meningitis, catheter-related infections, and septic arthritis in the patients.

Conclusion: Immunosuppressed individuals are always at risk of developing infections with atypical organism at typical sites. Septic arthritis should be treated on clinical suspicion with intravenous antibiotics, after having sent the fluid for cultures.


  OPC0077: Coexisting ankylosing spondylitis and lupus Top


Deepak Rath, Pradyot Sinhamahapatra, Alakendu Ghosh; Department of Rheumatology, IPGMER and SSKM Hospital, Kolkata, West Bengal, India

A 14-year-old male was diagnosed ERJIA in 2014 after presenting with complaints of large joint polyarthritis and inflammatory low back pain. He had dactylitis, enthesitis, and sacroiliitis on X-ray pelvis and human leukocyte antigen (HLA-B27) positivity by polymerase chain reaction. He was treated with nonsteroidal anti-inflammatory drugs and sulfasalazine. Methotrexate was added from January 2016 but stopped from September 2016 in view of anemia.

He was admitted again in January 2018 with complaints of fever of 1 month. He was evaluated outside for prolonged pyrexia, was had pancytopenia and pyuria, and was treated with broad-spectrum antibiotics and antifungals. He developed prerenal failure and transaminitis. After admission, all drugs were stopped and hydration status corrected. His urine examination showed active sediments with granular casts. His serological profile showed antinuclear antibody (ANA) 4+ homogenous, and ANA profile showed RNP/Sm 3+, Sm 3+, SS-A 3+ and Ro-52 3+. He had hypocomplementemia (C3, C4: 23, 2.15) along with high titers of dsDNA (466). He underwent kidney biopsy which was reported as lupus nephritis (LN) Class 2. Considering high disease activity of systemic lupus erythematosus (SLE) with components – hematological (pancytopenia) and renal (LN Class 2), he was put on oral steroid at 0.5 mg/kg (prednisolone 20 mg/day) along with hydroxychloroquine (200 mg/day) and his condition improved.

Only few case reports of coexisting lupus and ankylosing spondylitis has been described. Genetic causation of SLE and ankylosing spondylitis is due to different haplotypes, which are not commonly seen together.[1] One of the proposed mechanisms of the development of SLE in ankylosing spondylitis may be due to low-grade chronic inflammation. Another mechanism proposed is that HLA-B27 antigen predisposes to certain microbial infection and later infections may trigger the development of SLE.[2]

This patient meets the American College of Rheumatology/SLICC criteria for diagnosis of lupus as well as ASAS criteria for spondyloarthritis. The coexistence of these two diseases is very rare.


  OPC0078: Evaluation of subclinical gut inflammation using fecal calprotectin level and colonic mucosal biopsy in psoriasis and psoriatic arthritis patients Top


M B Adarsh, Saroj K Sinha1, Sunil Dogra2, Kim Vaiphei3, Chetana Vaishnavi1, Aman Sharma; Departments of Medicine, 1Gastroenterology, 2Dermatology and 3Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: The association between gut inflammation and ankylosing spondylitis is well-established, not so in psoriatic arthritis (PsA).

Objective: The objective was to study the prevalence of subclinical gut inflammation in PsA and psoriasis (PsO) patients using fecal calprotectin levels and colonic mucosal biopsies.

Methods: Fifty consecutive patients with active PsA and one hundred with PsO were recruited. Fecal calprotectin levels of more than 43.2 μg/gm by ELISA were considered positive. Sigmoidoscopy and multiple colonic mucosal biopsies were done in 20 PsA patients and 8 PsO. Thirty consecutive patients diagnosed to have irritable bowel syndrome (IBS) were disease control population for fecal calprotectin assay.

Results: Baseline characteristics are given in [Table 1]. Fecal calprotectin level was elevated in 29 (58%) PsA patients and 26 (26%) PsO patients (P = 0.000). The mean value of fecal calprotectin was higher in PsA patients than PsO (86.6 ± 81.5 μg/g vs. 32.9 ± 48.1 μg/g, P = 0.000). The odds for a positive fecal calprotectin level in PsA was 3.9 (95% confidence interval 1.9–8.0) in comparison to PsO. Fecal calprotectin levels were significantly higher in PsA patients with high body surface area and PsO area and severity index scores. Those with axial phenotype had higher calprotectin levels and the levels correlated with Bath Ankylosing Spondylitis Activity Disease Activity Index. Mean fecal calprotectin level was 22.0 ± 18.5 μg/g min IBS patients which was significantly lower than PsA patients (P = 0.002) but was comparable with that of PsO patients (P = 0.8). Sigmoidoscopy was normal in all PsO patients, while two PsA patients had mucosal erythema. Fifteen PsA and all PsO patients showed increased lymphoplasmacytic infiltration of lamina propria in biopsy. Evidence of active colitis with cryptitis was seen in two and collagenous colitis was seen in seven PsA patients [Figure 1]. No PsO patient had active colitis or collagenous colitis.



Conclusion: Subclinical gut inflammation was significantly higher in PsA patients in comparison to PsO patients and is more prevalent among those with axial phenotype.


  OPC0081: Clinical study of rheumatoid arthritis and its association with anti-cyclic citrullinated peptide antibodies with special reference to the first-degree relative Top


Trinayani Barua, P. Dihingia, Pramod G.R, S. M. Baruah, T. K. Das, D Das; Assam Medical College and Hospital, Dibrugarh, Assam, India

Background: Rheumatoid arthritis (RA) is a systemic inflammatory disorder characterized by chronic symmetric and erosive synovitis. The presence or absence of antibodies to citrullinated peptides (ACPA) is an important diagnostic parameter of early RA to initiate treatment.

Objectives: The objectives were (1) to study the prevalence of anti-cyclic citrullinated peptide (anti-CCP) antibodies in RA patients and their asymptomatic first-degree relatives (AFDRs) and (2) to study the correlation of symptoms of RA with respect to anti-CCP antibodies.

Materials and Methods: This study was conducted in Assam Medical College and Hospital, Dibrugarh, over a period of 1 year from June 2013 to May 2014. Eighty-five patients of RA classified by the American College of Rheumatology/European League Against Rheumatism 2010 Criteria and their 105 AFDRs were included. Serum anti-CCP antibody was assessed by chemiluminescent microparticle immunoassay.

Results: Out of the total of 85 patients, 67 were female and 18 were male with a ratio of 1:3.72. Majority of the patients (32.94%) belonged to the age group 40–<50 years. Anti-CCP was found to be positive in 71 (83.5%) patients and rheumatoid factor (RF) in 61 (71.7%) patients. Erosion on X-rays were present in 48 (56.47%) patients. Mean tender joint count and swollen joint count was more in anti-CCP positive patients than anti-CCP negative patients. Out of the total 105 AFDR, 22 (20.95%) patients showed positivity for anti-CCP antibodies out of which 13 (59.09%) were male and 9 (40.91%) were female.

Conclusion: Anti-CCP was present in 83.5% patients of RA compared to RF positivity in 71.7% only and was associated with more active, erosive and severe form of RA, with higher number of tender and swollen joints. The higher seroprevalence of anti-CCP in AFDR suggests that they may have a high risk of development of RA in the near future.


  OPC0082: Longitudinally extensive transverse myelitis as initial presentation of systemic lupus erythematosus Top


Harish Soni, B. C. Kalita; Assam Medical College and Hospital, Dibrugarh, Assam, India

Introduction: Lupus myelitis is a rare but serious condition reported in 1%–2% of patients with systemic lupus erythematosus (SLE). The rapidly progressive course involving motor, sensory, and autonomic functions makes transverse myelitis a medical emergency. The pathophysiological mechanism of lupus myelitis is uncertain although one of the two mechanism, i.e., primary vascular injury or primary inflammatory injury, is suggested.

Case Presentation: The patient was a 20-year-old female, presented with acute-onset symmetrical flaccid paraplegia with sensory involvement with early bladder and bowel involvement resulting in urinary retention and constipation. The patient also gave a history suggestive of inflammatory arthritis involving both small and large peripheral joints of body with associated mild intermittent fever for the last 6 months and a history of erythematous papular rashes over her trunk 1 month back which subsided spontaneously. Neurological examination revealed hypotonia, absent deep tendon reflexes, and muscle power to be Grade 0/5 in both lower limbs. Plantar reflex was indeterminate bilaterally. There was loss of all modalities of sensation below T8 spinal level and abdominal reflexes were absent. Examination of the upper limbs revealed normal findings. The most relevant laboratory abnormalities were hemoglobin – 7.9 g/dl and erythrocyte sedimentation rate – 150 mm AEFH. The patient had urinary tract infection. Cerebrospinal fluid examination revealed 10 cells/cumm predominantly polymorphs, protein 55 g/dl, sugar 60 mg/dl, and arthritis disease activity 7.21 U/L. She was tested negative for HIV. Antinuclear antibody and anti-dsDNA were positive. Antineutrophil cytoplasmic antibody, rheumatoid factor, and anti-cyclic citrullinated peptide antibody were negative. Magnetic resonance imaging showed intramedullary cord signal abnormality from D1 to conus medullaris involving central circumference of the cord showing inhomogenous hyperintensity on T2/STIR with mild long segmental cord swelling.

Conclusion: Having in mind acute transverse myelitis as a possibility in any patient with SLE allows us for early diagnosis and timely intervention.


  OPC0084: Adult Onset Still's disease- Study of series of 28 cases experience from tertiary care rheumatology units Top


Arun Kumar Gupta; Norvic International Hospital and Arthritis Care Center, Kathmandu, Nepal

Background: Adult-onset Still's disease (AOSD) is a rare chronic systemic inflammatory disease of unknown etiology that features prolonged fever and polyarthritis and is difficult in diagnosis.

Aims and Objective: The study aims to see clinical feature and outcome in AOSD patients.

Methods: This is prospective study of patients with AOSD, observed over 7 years from January 2010 to 2018 at a large tertiary care hospital.

Results: Twenty-eight patients (20 females) with median age at onset of 20 (18–25) years were seen. The clinical features include fever in all 28, inflammatory polyarthritis in 22, arthralgia in 28, sore throat in 15, and typical rash in 20 patients; lymphadenopathy was present in 22, hepatosplenomegaly in 25, anemia in 18, serositis in 15, neutrophilic leukocytosis in 25, and thrombocytosis in 25 patients. Acute-phase reactants were elevated in all. Rheumatoid factor, anti-cyclic citrullinated peptide, antinuclear antibody, and anti-dsDNA were observed negative in all patients. Serum glutamate-pyruvate transaminase, serum glutamic oxaloacetic transaminase, and serum ferritin were high in all cases. On median of 18 months, three patients had self-limited monocyclic pattern, 10 had polycyclic, and 17 had chronic articular pattern. All patients received nonsteroidal anti-inflammatory drugs, whereas 20 patients received methotrexate. All patient received steroid; eight patients received combined hydroxychloroquine and methotrexate.

Conclusion: AOSD is uncommon disease with varied presentation. Chronic articular pattern is the most common course. It has poor outcome with significant morbidity and most needing long-term therapy with steroids and disease-modifying antirheumatic drugs.


  OPC0153: Comparison between Routine Assessment of patient data index 3 (RAPID3) and Clinical disease activity index (CDAI) as a measure of disease activity in urban Indian population with Rheumatoid arthritis Top


Swetal Pandey, P D Rath, S Bhasin; Max Super Speciality Hospital, New Delhi, India

Background: Disease activity score 28, clinical disease activity index (CDAI), and simplified disease activity index are already validated outcomes to assess disease activity in rheumatoid arthritis. Routine assessment of patient data index 3 (RAPID3) is a questionnaire which is very easy and simple.

Objective: The objective was to assess RAPID3 and CDAI in rheumatoid arthritis patients and to correlate them.

Methods: Clinically diagnosed 200 patients of rheumatoid arthritis as per the American College of Rheumatology/European League Against Rheumatism 2010 criteria were selected including patients with comorbid illness. Patients were divided into two groups based on the presence or absence of comorbid illness.

Results: The mean age was 52 years, and 84.46% were females. Median score of RAPID-3 was 8 (interquartile range [IQR] 18–10) while of CDAI was 14.5 (IQR 21–6.6). Spearman's rank order correlation coefficient for CDAI with RAPID3 was 0.757 (P < 0.001). In individuals with no comorbid illness, it is slightly higher 0.817 (P < 0.001) in comparison to patients with comorbid illness 0.677 (P < 0.001); 61.17% of patients who met CDAI moderate/high activity criteria met similar RAPID3 severity criteria (κ: 0.480, P < 0.001). One patient with moderate activity in CDAI had low severity in RAPID3. 18% who met CDAI remission/low activity criteria also met similar RAPID3 criteria. In 21.6%, RAPID3 overestimated remission or low activity as moderate-to-high activity, while in patients with comorbid illness, it was more (26.5%). CDAI and RAPID3 showed disagreement in group with comorbid illness (odds ratio 2.528, 95% confidence interval 0.853–7.48).

Conclusions: RAPID3 correlates well with CDAI in all patient groups, and correlation is better in group with no comorbid illness. RAPID3 overestimated remission or low activity as moderate-to-high activity as 26.5% and 21.6%, respectively, in patients with and without comorbid illness.


  OPC0152: Prepulseless Takayasu's arteritis: An important cause of fever of unknown origin Top


A. Raut, P. Joshi, C. Balakrishnan, A. Khune, R. Nanavati; P.D. Hinduja National Hospital and MRC, Mumbai, Maharashtra, India

Introduction: Takayasu's arteritis (TA) affects the aorta, its main branches, and the pulmonary arteries. It is difficult to detect in the prepulseless stage as patients present with nonspecific symptoms. Fever can be the presenting feature of TA. We present three patients of prepulseless TA who presented with fever and discuss the importance of imaging in the diagnosis.

Case Report: All three patients presented with fever of unknown origin (FUO) of more than 1 month duration with weight loss. All patients were above 50 years. Routine investigations were normal including liver function test, renal function test, and viral markers except raised acute-phase reactants. Myeloma workup was negative. Antinuclear antibody, antineutrophil cytoplasmic antibody, and other serological markers were negative. Chest radiograph, ultrasound, and 2D echocardiography were normal. Blood and urine culture done multiple times were showing no growth. In the view of nonresolving fever and high erythrocyte sedimentation rate, considering the age of the patients, positron emission tomography-computed tomography (PET-CT) scan was done in all three patients. It showed active uptake in the aorta and its branches. Although PET scan is sensitive in TA, it is important to remember that active uptake in the vessel wall is also seen with atherosclerosis, vascular remodeling, or fibrosis. Since the three patients did not have any clinical evidence of TA and a tissue diagnosis was not possible, we thought it fit to document the vessel wall inflammation by another imaging method magnetic resonance (MR) angiography. MR angiography showed diffuse wall thickening in the aorta and its branches without stenosis, after which the diagnosis of TA was made. All three patients became asymptomatic after treatment with steroids and appropriate steroid sparer.

Conclusion: TA, especially in the prepulseless stage, can present as FUO. PET-scan is an important screening imaging modality in those with FUO. It is advisable to do an MR angiography to confirm vessel wall inflammation as PET-CT can be false positive in certain situations.


  OPC0085: Clinicopathological characteristics of renal involvement in systemic lupus erythematosus patients with antineutrophil cytoplasmic antibody Top


Sonali Dey, Sanjeeb Kakati; Assam Medical College and Hospital, Dibrugarh, Assam, India

Background: Lupus nephritis is one of the most serious complications of systemic lupus erythematosus (SLE). Vasculitis in SLE patients is a well-documented phenomenon; however, the antineutrophil cytoplasmic antibody (ANCA) (antineutrophil antibody [ANA]) role in the disease pathogenesis is still under discussion.

Objectives: The objective was to study ANCA prevalence in SLE patients and its association with renal manifestations, disease activity, and other autoantibodies.

Methods: The study was a hospital-based cross-sectional study carried out in 200 SLE patients. Disease activity was assessed by SLE disease activity index (SLEDAI) score. The detection of ANCA and ANA were done by indirect immunofluorescence. The other antibodies were detected by line immunoassay and C3, C4 by nephelometric immunoassay. Renal involvement was assessed based on clinical features and laboratory parameters.

Results: In a total of 200 cases, the prevalence of ANCA was 27.5%; predominant pattern was p-ANCA in 99% cases. Renal involvement was present in 79% of cases. The incidence of oliguria/anuria, facial puffiness/edema, raised serum creatinine, and proteinuria and the presence of renal cast were significantly higher in ANCA-positive group as compared to ANCA-negative group (P = 0.016, 0.04, 0.00, 0.001, and 0.016, respectively). In our study, the positive rate of anti-dsDNA, antinucleosome, and antihistone antibodies was higher significantly in ANCA-positive group than in ANCA-negative group (P = 0.04, 0.00, and 0.003, respectively). Patients with ANCA had significantly low C3 and C4 and high SLEDAI score (P = 0.03, 0.02, 0.00, respectively).

Conclusion: The present study of ANCA in SLE showed that patients with ANCA positivity presented with more severe clinicopathological injuries. ANCA can be a useful complementary parameter to predict vasculitis in SLE and is a risk factor for poor renal outcome.


  OPC0086: Efficacy of tocilizumab in patients with Takayasu's arteritis: Results from a single center prospective observational study Top


S Karthikeyan, S Balameena, R Ramesh, S Mythili, V A Sowndhariya, N Sujatha, H Raghavendra, Anusha, Aravind, Rajavel, Anuja, Sreedevi; ???, Institute of Rheumatology, Rajiv Gandhi Government General Hospital, Madras Medical College, Chennai, Tamil Nadu, India

Background: Takayasu's arteritis (TA) is a large-vessel vasculitis that affects predominantly the aorta and its main branches. Interleukin-6 is a key cytokine. Steroids remain the cornerstone of therapeutic management. Tocilizumab may be an effective, steroid-sparing agent for rapid control of disease activity in patients with life-threatening indications. Recently reported multicenter studies demonstrated the benefit of tocilizumab in TA, for event-free survival.

Objectives: The objective was to assess the clinical profile and the efficacy of tocilizumab in patients with TA.

Methods: This is a prospective observational study conducted between February 2018 and July 2018. Thirty patients with active disease (ITAS >1) presenting first time to the clinic are given 6 monthly infusions of tocilizumab (8 mg/kg intravenous). Pregnant women, lactating women, and those with active tuberculosis/HIV and renal failure are excluded. Baseline clinical characteristics, acute-phase reactants, and findings of computed tomography angiogram are recorded. ITAS 2010/ITAS-A is assessed on admission and 3rd and 6th month of tocilizumab therapy.

Results: Mean age, median disease duration, and mean ITAS 2010 are 32 ± 9.2 years, 9 months, and 13.3 ± 4.3, respectively. Clinical presentation included limb claudication (60%), hypertension (47%), constitutional (fever/weight loss) (20%), stroke (13%), cardiomyopathy (13%), aortic regurgitation (13%), myocardial infarction (7%), and carotidynia (3%). Type V (30%) is the predominant type seen in angiography. Mean starting dose of steroids is 45.8 ± 9.7 mg, and the dose is effectively reduced to 7.58 ± 2.9 mg at the end of 6 months of therapy. An ITAS score of 0 is seen in 90%, 100% of patients at the end of 3rd and 6th month of tocilizumab infusion. All patients achieved a reduction in erythrocyte sedimentation rate and C-reactive protein at 3 months.

Conclusion: Tocilizumab may be an effective steroid-sparing option for rapid control of disease activity in TA. However, relapse-free survival after the cessation of tocilizumab needs to be studied.


  OPC0200: A rare case of rheumatoid arthritis with concurrent multicentric reticulohistiocytosis Top


Sujata Devi, Anupama Behera; All India Institute of Medical Sciences, Bhubaneswar, Odisha, India

Introduction: Multicentric reticulohistiocytosis (MRH) is a rare multisystem macrophage disorder of unknown etiology characterized by papulonodular skin and mucosal lesion, rapidly progressive erosive symmetric polyarthritis and inflammation of the internal organ. Most often, it is misdiagnosed as rheumatoid arthritis (RA).

Case Report: We report a case of a 60-year-old female found to have features of both RA and MRH concomitantly, confirmed by histopathology of the skin lesion which showed diffuse histiocytic infiltrates with the multinucleated giant cell. She had positive RA factor and anti-cyclic citrullinated peptide antibody in the serum. She was being advised with methotrexate, hydroxychloroquine, corticosteroids and nonsteroidal anti-inflammatory drugs, and bisphosphonate.

Discussion: Coexistence of RA and MRH is very rare in the field of rheumatology. Few cases have been reported worldwide. Hence, owing to its rarity, the diagnosis can be challenging. Differential diagnoses of nodular RA or multicystic reticulohistiocytosis or coexistence of both diseases have to be kept in mind.

Conclusion: Clinicians need to have a high index of suspicion if a patient presenting with erosive polyarthritis associated with papulonodular skin lesions.


  PC0028: Mycoplasma pneumoniae infection-associated thrombotic state in children: A case series Top


Rajesh Kanumuri, Suma Balan; Department of Clinical Immunology and Rheumatology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Case Report: We report three pediatric cases of around 5 years of age, who presented with thrombotic manifestations in different anatomical sites with a concomitant history of lower respiratory tract infection. Out of three, two had arterial thrombosis, among which one presented with thromboses in multiple arterial territories and the other had cardiac thrombi and left main coronary artery infarct. The third case had deep venous thrombosis of the left lower limb. All the three cases were evaluated thoroughly and were found to have four-fold rise in titers of IgM Mycoplasma antibodies. Out of the three, two had positivity for antiphospholipid antibodies, which were undetectable at 1 year of follow-up. All the three cases improved with macrolides, short course of steroids, and anticoagulation. Oral anticoagulants were stopped successfully without any further thrombotic events in all the patients by the end of 1 year of follow-up.

Discussion: Mycoplasma pneumoniae is a common cause of community-acquired pneumonia in children and adolescents. Thrombosis is not very commonly seen in M. pneumoniae infection. The mechanism underlying thrombosis is poorly understood. The possibilities proposed were a direct invasion mechanism and autoimmune modulations. To the best of our knowledge, there are <30 case reports of Mycoplasma infection-related thrombosis in the literature. In one study, antiphospholipid antibodies were found to be positive in 50% of cases of Mycoplasma-induced thrombosis. These antibodies were undetectable by 1 year after occurrence of the thrombosis.

Conclusion: Infection-related thrombosis is not very common and should always be suspected in pediatric age group when there is associated clinical history suggestive of infection. The thrombosis associated with Mycoplasma infections in pediatric age group has relatively a good prognosis if treated promptly with appropriate antibiotics and anticoagulants. There may be transient production of antiphospholipid antibodies which does not require lifelong anticoagulation or immunosuppression.


  OPC0090: Acute respiratory failure as an antisynthetase syndrome Top


Nahar Naisar, Nahar Prachi; Arham Rheumatology Center, Nashik, Maharashtra, India

Antisynthetase syndrome is a major subgroup of inflammatory myopathies seen in a minority of patients with dermatomyositis and polymyositis. Interstitial lung disease is the main pulmonary manifestation and may be severe, thereby determining the prognosis. We report the case of a 35-year-old female admitted to intensive care for acute respiratory failure complicating rapid interstitial lung disease. The presence of Raynaud's phenomenon and finger hyperkeratosis associated with the positivity of anti-Jo1 antibodies allowed the diagnosis of antisynthetase syndrome. Our case emphasizes the importance to search for anti-Jo1 antibodies in the presence of interstitial lung disease. Early diagnosis and immunosuppressive therapy would prevent disease progression and results in a good outcome.


  OPC0091: Breastfeeding and the risk of systemic lupus erythematosus: Registry data Top


Keerthi Talari; Consultant Rheumatologist, Yashoda Hospitals, Secunderabad, Telangana, India

Background: Human breast milk has been found to be essential in development of an infant's immunity by influencing intestinal microbiome, thymus, and T-cell repertoire development and is also a complete arsenal of immune components (lactoferrin, lactoadherin, sIgA, and cytokines). The absence of breastfeeding has been implicated in diabetes mellitus, coeliac disease, asthma, multiple sclerosis, rheumatoid arthritis, and juvenile idiopathic arthritis with no or decreased breastfeeding causing increased risk of disease development. Studies assessing risk of systemic lupus erythematosus (SLE) in nonbreastfed infants are very few with controversial results.

Objective: We sought to assess whether patients with SLE were breastfed during their infancy and their duration of breastfeeding.

Methods: Data were derived from our lupus registry. Patients with SLE aged <30 years were included. Mothers of the patients were interviewed regarding whether the patient was breastfed or not and the duration of breastfeeding.

Results: Out of 82 SLE patients in the registry, 54 were <30 years and their mothers were interviewed of which 45 answered. Except for one patient, all were breastfed. Two patients were breastfed for 6 months, 4 for 1 year, 15 for 2 years, 13 for 3 years, and 10 for more than 3 years.

Conclusion: We found very few patients of lupus who were not breastfed; hence, nonbreastfeeding may not be a risk for development of SLE in the future for a child.


  OPC0155: Ataxic hemiparesis as a rare manifestation of polyarteritis nodosa: A report of two cases and review of literature Top


Siddharth Jain1,2, Manish Modi3, Manphool Singhal4,

Sanjay Jain1,2, Aman Sharma1,2; 1Division of Clinical Immunology and Rheumatology, PGIMER, Departments of 2Internal Medicine, 3Neurology and 4Radiodiagnosis, PGIMER, Chandigarh, India

Introduction: Central nervous system (CNS) involvement in polyarteritis nodosa (PAN) is rare (4%–8%) and can present with stroke (3%–24%), seizures, or encephalopathy. Lacunar strokes are the most common PAN-associated strokes; however, ataxic hemiparesis is rarely reported in PAN.

Case 1: A 49-year-old female diagnosed as PAN (hepatitis B negative) at the age of 35 years when she presented with mononeuritis multiplex, digital gangrene, hypertension, weight loss, and inflammatory polyarthritis. She recovered completely with pulse cyclophosphamide. 14 years after diagnosis, she had acute left-sided ataxic hemiparesis with the left sensorineural hearing loss. Magnetic resonance imaging (MRI) brain showed T2/FLAIR hyperintensities in the lower pons, pontomedullary sulcus, and ventral medulla [Figure 1] with multiple chronic lacunar infarcts. Magnetic resonance (MR) angiography did not show microaneurysms. She symptomatically improved with cyclophosphamide with resolution of hyperintensities on follow-up MRI. She is now doing well on immunosuppression.



Case 2: A 51-year-old gentleman diagnosed as PAN (hepatitis B negative) when he presented with livedo reticularis, hypertension, and mononeuritis multiplex (nerve biopsy showing vasculitis) 19 years back. He improved with immunosuppression but 7 years into the disease developed acute left-sided ataxic hemiparesis. MRI brain showed an acute lacunar infarct in the right pontomedullary junction. MR angiography was normal. He recovered completely with steroids and azathioprine; however, there were intermittent exacerbations in ataxia on tapering steroids, which were steroid responsive. He is currently doing well on immunosuppression.

Discussion: Ataxic hemiparesis in PAN seems to have a delayed onset (long latency from disease onset to stroke) and good prognosis, with near-complete recovery of neurodeficits with immunosuppression. Recurrent relapses on steroid tapering understate the relapsing nature of disease and the need for maintenance immunosuppression. Intracranial microaneurysms are relatively rare in CNS-PAN. Timely recognition and early aggressive immunosuppressive therapy are critical.

Conclusion: Ataxic hemiparesis is a rare manifestation of PAN and behaves differently from other PAN-associated stroke phenotypes.]


  OPC0159: Rheumatological manifestations of HIV Top


Vikram Londhey; HBT Medical College and RN Cooper Hospital, Mumbai, Maharashtra, India

Introduction: Rheumatological manifestations can develop during HIV infection at any stage of the disease. These disorders can cause significant morbidity in the patients affecting their quality of life. With the advent of highly active antiretroviral therapy (HAART), many of the side effects of HAART can also present as rheumatological manifestations.

Methods: This prospective observational study was conducted in HBT Medical College and RN Cooper Hospital between November 2015 and May 2018. The patients were followed up for 2 years. Adult HIV-positive patients (those on antiretroviral therapy [ART] and those not on ART) having rheumatological problems attending the ART outpatient department (OPD) or rheumatology OPD or admitted in the wards were included in the study. Institutional Ethics Committee approved the project and consent was taken from each participant.

Objectives: The objectives were (1) to study the rheumatological manifestations in HIV patients who are on HAART and not on HAART, (2) to correlate the rheumatic manifestations with CD4 count, and (3) to study the correlation of duration since detection of HIV and development of rheumatological manifestation.

Results: Of 3802 patients suffering from retroviral disease, 3007 were on ART. Of 32/3802 (0.84%) patients, 26 were on ART and 6 were not on ART. 132 patients had arthralgias and fibromyalgias. Males 23, females 9; patients were in the age group of 18–52 years. Mean CD4 count was 428 (37–977).

Diagnosis: Reactive arthritis was diagnosed in 9, psoriatic arthritis 4, HIV arthropathy 2, rheumatoid arthritis 2, vasculitis 2, PH 1, cerebral venous sinus thrombosis (aCL) 1, painful articular syndrome 1, zidovudine myopathy 1, cutaneous lupus 1, antinuclear antibody +ve 2, hyperuricemia 4, and monoarticular involvement Koch's 2.

Conclusion: Rheumatological manifestations can occur at any stage of the disease. They do not correlate with the CD4 count. In some patients, the rheumatological manifestation and HIV were detected simultaneously. We did not see DILS in our study. Disease-modifying antirheumatic drugs and short course of corticosteroids are safe with regular CD4, complete blood count, liver function test, and renal function test monitoring.


  PC0030: Clinical characteristics and treatment outcome of eosinophilic granulomatosis with polyangiitis patients in tertiary center of PGIMER Top


Rajiv Ranjan Kumar, Saket Jha, Sakshi Mittal, Arghya Chatopadhya, Adhaar Dhooria, Kusum Sharma1, Ritambhra Nada2, Ranzana Minz3, Varun Dhir, Sanjay Jain, Aman Sharma; PGIMER, Chandigarh, India

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is the rarest of all antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. It was first described by Churg and Strauss in 1951. Ten-year mortality in retrospective studies is up to 30%. The most important risk factor for death is cardiomyopathy and older age as shown by French cohort.

Aim: To investigate the clinical characteristics and long-term outcomes of EGPA patients in a single tertiary center of North India.

Methods: This was a retrospective study, data were collected from January 2011 to December 2017, and 13 patients of EGPA were found. All patients fulfilling the American College of Rheumatology 1990 or Chapel Hill definitions of EGPA were included.

Results: The mean age was 41 years (20–75 years) with slight male preponderance (1.4 times). Asthma was seen in 84.6% of patients. The mean duration from asthma to EGPA diagnosis was 43 ± 30.8 months. ANCA was positive in 46% of patients (MPO-ANCA) except one patient having PR3-ANCA. Constitutional symptoms were seen in 77% of cohort, pulmonary involvement in 69%, and peripheral neuropathy in 59%, skin and cardiac in 30.7% of patients. Renal, upper respiratory tract, and joint involvement were found in 23% of patients. Lung and renal involvement was seen more in ANCA-positive patients while cardiac and gastrointestinal tract involvement was predominant in ANCA-negative patients. All patients were treated with oral steroids. Cyclophosphamide induction was given in 9 (69%) patients, while rituximab induction in 2 (15%) patients. Azathioprine maintenance therapy was given in nine patients (69%) while two patients were on rituximab maintenance. Remission was seen in all patients. No mortality was seen over a follow-up period of 48 ± 35.2 months. Relapse was seen in six patients. Adverse events were urinary tract infection, superficial fungal infection, and cellulitis of the right foot.

Conclusion: EGPA was rare but had a good treatment outcome in our cohort. ANCA status predicts organ involvement.


  PC0031: Cytophagic histiocytic panniculitis with macrophage activation syndrome: A case report Top


Rajesh Kanumuri, Suma Balan; Department of Clinical Immunology and Rheumatology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Case Report: We report an 8-year-old male child who was referred to us with 7-month history of painful erythematous-indurated skin lesions mainly over the face, thigh, and lower back associated with daily spiking fever. He was evaluated elsewhere for these symptoms, leukopenia, and thrombocytopenia with infective and autoimmune workup being negative and with inconclusive diagnosis on the biopsies of the bone marrow and cutaneous lesions. Repeat deep skin biopsy of the indurated lesions at our center showed features of cytophagic histiocytic panniculitis (CHP)/subcutaneous panniculitis-like T-cell lymphoma (SPTCL). Considering his age, indolent course, and dense inflammation in the subcutaneous fat with large areas of fibrinoid necrosis, he was labeled as probable CHP after a multidisciplinary meeting involving rheumatologist, dermatologist, hemato-oncologist, and pathologist. He also had features of macrophage activation syndrome (MAS). He was treated initially with pulse doses of intravenous methyl prednisolone followed by oral prednisolone along with cyclosporine. After initiation of treatment, he remained afebrile and skin lesions gradually resolved, thus favoring the diagnosis of CHP.

Discussion: CHP is rare panniculitis in childhood, associated either with nonmalignant conditions or with SPTCL and often also associated with MAS. Differentiating these conditions is therapeutically important because nonmalignant CHP often improves with prednisolone and cyclosporine, whereas most cases of SPTCL may require aggressive cytotoxic therapy. The differentiation between CHP and SPTCL is difficult as both can have similar features on the biopsy and immunohistochemistry. A collaborative discussion among interdepartmental meetings would help differentiate these two conditions.

Conclusion: Patients with CHP may have varied clinical outcomes. Some patients may rapidly progress to death, and some may have a normal life. Differentiating CHP from SPTCL is very important to avoid complications related to cytotoxic therapy and financial and emotional burden to the patients and their family.


  OPC0220: A questionnaire-based survey of common knowledge about rheumatologic diseases amongst patients Top


Pandya S, Solanki R, Shah R, Patel N, Shukla A, Shah N, Chikani S, Desai B, Sanap A, Bavaliya M, Jain N, Shukla D, Tank R, Prajapati P, Usadadiya J, Srivastava P, Sharma R, Parmar A, Sharma V, Kotecha M, Pathak H, Lapsiwala M, Deewanji S, Parikh T; (Rheumatology Association Gujarat) Vedanta Institute of Medical Sciences, Ahmedabad, Gujarat, India

Background: We wanted to look at whether patients coming to our outpatient departments know about some common issues related to rheumatologic diseases.

Objective: The objective was to find out existing knowledge among patients about rheumatologic diseases.

Materials and Methods: This was a cross-sectional survey of patients presenting to about 15 centers across the state of Gujarat. A questionnaire in the local language consisting of ten questions related to most common issues, to be answered in a yes/no format was circulated. The patients were asked to fill these while sitting in the waiting area.

Results: The ten questions were related to whether they knew about “rheumatology,” that rheumatoid arthritis can affect any age, that it can involve other organ systems apart from the joint, whether it can be transmitted to the child, that it can cause permanent damage to the joint, any relation to foods (e.g., curd, sour food), whether they think allopathy can control them well, alternative therapy, about corticosteroids and its adverse effects and the role of their family doctor in treatment decisions.

A total of 1244 patients filled the questionnaires. Except for question number 4 ( if they thought it is transmitted to the child) where about 80% said “no” and question number 5 (if they knew it damages the joints permanently if not treated) where about 80% said “yes,” the rest of the replies were 60/40 proportion of yes/no. Significantly more in the more educated group (67% vs. 52%) was aware of “rheumatology” as a specialty (question number 1) and about corticosteroids (question number 8). Significantly, more patients on follow-up (47.5% vs. 33%) knew that allopathy can control the disease.

Conclusion: Awareness about facts related to rheumatologic diseases needs to be increased by doing more such surveys and intervening with counseling and education.


  OPC0114: Palindromic rheumatism versus rheumatoid arthritis and regional differences Top


Pandya S, Parikh T, Solanki R, Shenoy P; Vedanta Institute of Medical Sciences, Ahmedabad, Gujarat, India

Background: There are scarce data on palindromic rheumatism (PR) from the subcontinent. We wanted to see if PR is indeed a less severe form of rheumatoid arthritis (RA) and also compare features of our cohort from that from Kerala.

Objective: The objective was to compare clinical and laboratory features of patients of PR presenting to our outpatient department (OPD) to those of RA also from our OPD and those with PR from another center from South of India (Kerala).

Material and Methods: Ours was a cross-sectional study. Descriptive statistics were applied to the data we had on patients with PR and RA (from 2013 January to 2018 July) to look at differences in demographic profiles and autoantibody positivity (rheumatoid factor [RF] and cyclic citrullinated peptide [CCP]) between the two. We also compared our data to a series reported from Kerala.

Results: Data from 3500 patients with RA and 548 with PR were analyzed. Proportion of males were more in PR patients (27.5% vs. 14.6%). PR patients were significantly younger (43.7 mean vs. 47.2 mean) and were early in their disease (3 vs. 6.1 years). The mean erythrocyte sedimentation rate (ESR) of PR patients was significantly lower (43.7 vs. 65.6). Our 548 patients (PR) were compared with 353 from the Kerala group (PR). Our patients were significantly younger (43.7 mean age vs. 45.7 mean age), early in their disease (3 vs. 6.8 years), had much less pain (visual analog scale 0–10, 3.9 vs. 8.3), but had significantly higher ESR (mean 43.7 vs. 22.4). Most of our patients (75%–80%) were seropositive for RF and CCP compared to about half in the Kerala group.

Conclusions: Compared to RA, PR patients were younger and early in the disease with lesser clinical and laboratory inflammation. There are differences between different regions of the subcontinent among PR patients.


  PC0036: Retrospective analysis of cardiovascular risk (using atherosclerotic cardiovascular disease risk score) in patients with fibromyalgia syndrome: A case–control study Top


Sandeep Surendran, C B Mithun; Department of Rheumatology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Background: Routine assessment of cardiovascular risk is of increasingly importance in rheumatological diseases, namely rheumatoid arthritis. However, the knock-down effect of the neuroinflammation in fibromyalgia on cardiovascular risk is not known.

Objectives: The purpose of our study is to retrospectively compare the cardiovascular risk using atherosclerotic cardiovascular disease (ASCVD) risk score in fibromyalgia patients versus normal controls.

Methods: Patients satisfying 2016 modification of American College of Rheumatology 2010/2011 fibromyalgia criteria were included in our study. Baseline details collected included demographics, comorbidities, baseline outcome measures (brief pain inventory average pain and fibromyalgia impact questionnaire revised), blood pressures, and lipid profile. The 10-year and lifetime ASCVD cardiovascular risk score was calculated using the ASCVD calculator. For patients <40 years, only lifetime risk was calculated; while for patients >60 years, only 10-year risk was calculated. Chi-square test and modified Fisher's exact test were used to compare risk scores between patients and normal controls.

Results: The age-stratified distribution of ASCVD risk scores between the patients (n = 113) and controls was calculated and attached (refer to the attached image). The lifetime cardiovascular disease (CVD) risk of fibromyalgia patients in the age group 40–59 years was higher than controls (P = 0.002).

Conclusions: Our study shows that fibromyalgia patients within the age range of 40–60 years have a statistically higher lifetime risk of CVD. Identification of higher CVD risk is important as the lifestyle modifications and other preventive measures should be started at the earliest in these fibromyalgia patients.


  OPC0179: Kawasaki disease shock syndrome with macrophage activation syndrome, severe myocarditis, and coronary artery abnormalities: All in one patient Top


Anjani Gummadi, Rakesh Kumar Pilania, Deepti Suri, Anju Gupta, Amit Rawat, Surjit Singh; Postgraduate Institute of Medical Education and Research, Chandigarh, India

Case Report: A 5-year-old male child presented with fever for 20 days, irritability, bilateral conjunctival injection, strawberry tongue, red, cracked lips, left-sided cervical lymphadenopathy, and perianal erythema. Investigations revealed anemia (92 g/L), neutrophilic leukocytosis (20.6 × 109/L with 99% neutrophils), thrombocytosis (platelet count up to 850 × 109/L), C-reactive protein (120 mg/L), elevated erythrocyte sedimentation rate (ESR) (67 mm in 1st h), sterile pyuria, and high N-terminal Pro B-type natriuretic peptide (80,000 pg/ml; normal <125 pg/mL). Clinical possibility of Kawasaki disease (KD) was considered and intravenous immunoglobulin (IVIg) (2 g/kg) was administered. He continued having high-grade fever and spikes and developed skin and gastrointestinal bleed. Repeat investigations revealed worsening anemia (hemoglobin 7.5), severe leukopenia (480/L with absolute neutrophil count of 20), thrombocytopenia (minimum 4.0 × 109/L), elevated transaminases, hypofibrinogenemia, hypertriglyceridemia, and hyperferritinemia. Bone marrow examination showed hemophagocytosis. His clinical status deteriorated, and he developed respiratory failure and severe myocardial dysfunction requiring inotropic support and mechanical ventilation. Two-dimensional echocardiography (2DE) revealed severe myocardial dysfunction (ejection fraction [EF] 20%) with ectatic left main, left anterior descending, and right coronary arteries. Diagnosis of KD with severe myocarditis with coronary artery aneurysm with macrophage activation syndrome (MAS) was proffered. He was initiated on IV pulse methylprednisolone at 30 mg/kg/day along with second doses of IVIg and infliximab. His MAS and myocarditis were refractory. Perforin expression on NK cells revealed decreased expression (stimulation index 1.44; control 3.76). He required 7 pulses of IV methylprednisolone followed by tapering doses of oral prednisolone along with cyclosporine. 2DE done at 6 weeks of illness revealed normalization of coronary artery diameters with a normal EF.

Discussion: This case highlights the difficulties in the management of myocarditis and MAS in children with KD, especially in countries where treatment with interleukin-1 antagonists is not readily available.

Conclusion: Severe myocarditis and MAS can occasionally present together and complicate the clinical course of KD.


  PC0032: Coexistence: Rheumatoid arthritis and sarcoidosis Top


Nahar Naisar, Nahar Prachi, Shah Samkit; Arham Rheumatology Center, Nashik, Maharashtra, India

Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized by erosive arthritis. Sarcoidosis is a chronic disease characterized by the formation of noncalcified granulomas. Our case, a 53-year-old woman, is a known case of RA for 5 years and on regular treatment with disease-modifying antirheumatic drug (DMARD) (leflunomide and hydroxychloroquine). She is also known case of hypertensive, diabetes mellitus, and hypothyroidism. Suddenly, she developed dyspnea and multiple cranial nerve palsy. Computed tomography (CT) chest suggestive of hilar lymphadenopathy and endobronchial biopsy showed noncaseating granuloma. Laboratory tests showed elevated acute-phase responses and serum angiotensin-converting enzyme levels. The patient was diagnosed with RA and sarcoidosis. High-dose corticosteroids were started and DMARD was continued, and on her 6th month of follow-up, her clinical and laboratory findings and lymphadenopathies on CT had regressed. The clinical follow-up continues; the patient appears to be in clinical remission. The purpose of this report is to describe a patient who developed sarcoidosis after the 1st-year history of RA.


  OPC0093: Contact dermatitis with nut extract of Semecarpus anacardium Top


Keerthi Talari; Consultant Rheumatologist, Yashoda Hospitals, Secunderabad, Telangana, India

In this era, where prevention of rheumatoid arthritis is becoming a realizable target, we report an extremely appalling practice in patients with rheumatoid arthritis. Many patients with rheumatoid arthritis and those with other causes of joint pains use the oily extract of nut Semecarpus anacardium commonly called jeedi in the local vernacular language. It is believed that pain at any site in the body can be relieved with the application of this nut extract. However, its local application is known to cause severe contact dermatitis. Within few hours or sometimes as late as 3 days, erythema, itching, bullae, and at times cutaneous necrosis with ulceration develop at the site of application. We report images of contact dermatitis with S. anacardium in patients of rheumatoid arthritis due to application of nut extract on joints.

Discussion: The chemical components of the seed are mainly bhilwanols, phenolic compounds, biflavanoids, sterols, and glycosides. Multiple formulations made out of this nut extract have been used in Siddha and Ayurvedic System of Medicine. The nut extract is believed to have anticarcinogenic, hypoglycemic, antiatherogenic, and anti-inflammatory properties. However, its local application has been known to cause severe contact dermatitis. The oil extract of this seed has a strong vesicant property. The chemical component responsible for this is identified as urushiol, an oleoresin.

Conclusion: While we focus on achieving treat to target in patients with rheumatoid arthritis, educating the general population regarding the available treatment options for rheumatoid arthritis is also of utmost importance as these time-worn practices still plague our society.


  PC0049: NA Top


Sandesh Guleria, Johnson Nameirakpam, Sagar Bhattad, Avinash Sharma, Anju Gupta, Amit Rawat, Prateek Bhatia, Vignesh Pandiarajan, Deepti Suri, Surjit Singh; Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: Systemic juvenile idiopathic arthritis (SJIA) is now considered as an autoinflammatory disorder secondary to innate immune dysfunction with a propensity to develop macrophage activation syndrome (MAS). sCD25 has been used as a sensitive biomarker for the diagnosis of hemophagocytic lymphohistiocytosis.

Objective: The objectives were to assay serum-soluble CD25 in children with SJIA and to compare levels of sCD25 in children with active disease and inactive disease and those with MAS.

Method: This prospective study was conducted in a tertiary care referral center in North India from January 2017 to June 2018. All patients fulfilling the International League of Associations for Rheumatology criteria for SJIA were enrolled in the study. Appropriate investigations were carried out, and sCD25 was analyzed using commercially available sCD25/IL-2R ELISA kit. The data were analyzed using the Statistical Package for the Social Sciences, version 20.

Results: A total of 35 children (1–18 years) with 43 events were included in the study. There was male predominance (71.4%) with mean age at enrolment of 7.3 ± 3.59 years. Based on clinical features and investigations, events were categorized into three groups; SJIA with inactive disease (15; 34.9%), SJIA with active disease (15; 34.9%); and SJIA with MAS (13; 30.2%). sCD25 levels significantly (P = 0.0001) varied across three groups. On the basis of receiver operating characteristic curve, sCD25 cutoff level at 10,385 pg/ml was found to have sensitivity of 100% and specificity of 96.7% in differentiating MAS in SJIA from disease flare and inactive disease.

Conclusion: sCD25 is a useful biomarker in differentiating MAS in SJIA from disease flare and inactive disease with sensitivity and specificity of 100% and 96.7%, respectively, at a cutoff level of 10,385 pg/ml. Coupling sCD25 with other laboratory parameters would be ideal for early diagnosis and management of MAS complicating SJIA.


  OPC0095: Clinical characteristics of 181 granulomatosis with polyangiitis patients Top


Saket Jha, Godasi S R S N K Naidu, Sakhsi Mittal, Vikash Sharma, Manish Rathi1, Roshan Verma2, Manish Modi3, Benzeeta Pinto, Kusum Sharma4, Varun Dhir, Manphool Singhal5, Mahesh Prakash5, Ritambhra Nada6, Naresh K Panda2, Ranjana W Minz7, Sanjay Jain, Aman Sharma; Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: The published Indian data on granulomatosis with polyangiitis (GPA) are sparse. The aim of the study is to analyze the clinical characteristics of GPA patients at a single tertiary care center.

Methods: A retrospective analysis of all consecutive patients diagnosed to have GPA patients from 2005 to March 2018 was done. The demographic, clinical profile, laboratory, and imaging findings along with treatment outcome were noted from the medical records. The disease activity at presentation was assessed with Birmingham Vasculitis Activity Score (BVAS v3).

Results: A total of 181 patients (101 females and 80 males) were diagnosed to have GPA at a mean age of 41.5 ± 14.6 years. PR3 and MPO positivity was seen in 84.4% and 7.8%, respectively. 7.8% were antineutrophil cytoplasmic antibody negative by ELISA. The mean BVAS v3 score was 17.4 ± 8.3. The classical triad of upper respiratory tract (URT), lung, and renal involvement was seen in 61.6%, 58%, and 50.8% patients, respectively. Constitutional symptoms were noted in 63.9% of patients and joint symptoms were present in 43.6% of patients. Localized disease was seen in 24, early systemic disease in 49, generalized disease in 55, severe disease in 49, and refractory disease in four patients. Cyclophosphamide was given in 137 patients. Rituximab was used for remission induction in 25 and for maintenance in 31 patients. Four patients required both for refractory disease. Forty-four (24.3%) patients required renal replacement therapy at admission, and 12 (6.6%) patient required plasma exchange. A total of 33 patients died. Among the patients who died, 5 were categorized as early systemic, 10 generalized, 15 severe, and 3 had refractory disease.

Conclusion: This is the largest case series from India. Indian population gets affected by GPA at an earlier age and has less frequent URT and renal involvement as compared to the Western cohort.


  OPC0096: Cardiovascular manifestations in patients with rheumatoid arthritis and its association with anti-cyclic citrullinated peptide antibodies Top


Nancy Garg, L. A. Gauri, Kuldeep Saini, Rohitash Kularia; Sardar Patel Medical College, Bikaner, Rajasthan, India

Introduction: Rheumatoid arthritis (RA) is an independent risk factor for cardiovascular events that account for a significant proportion of mortality among these patients. Anti-cyclic citrullinated peptide (anti-CCP) antibodies are independent predictors of radiological damage and progression and are markers of poorer outcome in RA.

Aims: The aim was to determine the role of anti-CCP antibody as an independent risk factor for developing cardiovascular complications as documented by carotid intima-medial thickness and abnormal echocardiography in RA patients.

Materials and Methods: One hundred patients of RA having disease duration of at least 3 years participated in this hospital-based, cross-sectional, and observational study. Fifty patients were anti-CCP antibody positive and rest fifty were anti-CCP antibody negative. Patients of RA with known cardiovascular risk factors such as known heart disease, diabetes, hypertension, dyslipidemias, thyroid disorder, or family history of premature heart disease were excluded.

Results: Average intimated medial thickness of common carotid arteries was significantly higher among anti-CCP-positive group (P < 0.05). Lower left ventricular ejection fraction and left ventricular diastolic dysfunction was more commonly dispersed among the anti-CCP antibody-positive group with P = 0.01 and 0.034, respectively. Mild pericardial thickening was documented among 10% patients of anti-CCP antibody-positive group, while none of the anti-CCP antibody-negative group had similar findings on echocardiography.

Conclusion: This study stressed the important role of anti-CCP antibody in predicting cardiovascular manifestations such as atherosclerosis, pericardial, myocardial, and endocardial involvement due to inflammation in RA patients. Hence, patients with high titer of anti-CCP antibody should be evaluated for cardiovascular morbidity more vigilantly.t


  OPC0209: Correlation of ASDAS and BASDAI in Ankylosing Spondylitis Top


Nibha Jain, Dhaiwat Shukla, Puja Srivastava, Sapan Pandya; Sheth V S Hospital and Smt. NHL Municipal Medical College, Ahmedabad, Gujarat, India

Background/Purpose: To study correlation and agreement between the Ankylosing Spondylitis Disease Activity Score (ASDAS) with Bath Ankylosing Spondylitis Activity Index (BASDAI) and their trend on follow up.

Methods: Prospective longitudinal study including all consecutive patients of Ankylosing Spondylitis(AS) (November 2017 to August 2018) visiting the government medical college OPD after informed consent.

Baseline and follow-up demographic data with ESR, CRP, BASDAI, ADSAS, Patient and physician global assessment (PTGA, PHYGA) was noted and appropriate statistical analysis performed using SPSS software.

Results: The sample consisted of 134 patients, mostly men (80%), with a mean age of 30+12 years(Median = 27 yrs) and disease duration of 3.5+ 5.2 years.

  • Correlation coefficient between ASDAS CRP and BASDAI is r=0.75
  • The cutoff point of ASDAS with the best agreement with BASDAI was 3.5 (global agreement 82%, kappa 0.54).
  • Convergent validity shows a higher correlation of BASDAI with PT GA than with PhYGA (0.82 vs 0.7)
  • ASDAS had a similar correlation with PTGA and PhyGA (r=0.74).
  • The discriminant validity analysis showed that both indices were able to discriminate patients above and below the median PTGA(>4.5) (Cohen δ 1.3 vs 1.1)
  • On followup study of 52 patients with mean follow-up duration of 4.2+2.1 months, both ASDAS CRP and BASDAI were sensitive to change but ASDAS CRP showed better results as measured by effect size (δ 0.8 vs 0.6)
  • Baseline and follow-up data along with comparison with other national and international data shown in [Table 1] and [Table 2].



Conclusion:

  • Both ASDAS and BASDAI correlate well with each other and show similar trend on follow-up.
  • Agreement of BASDAI (>4) was more with high ASDAS in our study.
  • ASDAS has higher effect size on follow-up compared to BASDAI



  OPC0097: A case of psoriatic spondyloarthropathy presenting as unilateral sternoclavicular joint arthritis Top


Paras Kathuria, Pulin Kumar Gupta; RML Hospital, New Delhi, India

Case Report: A 24-year-old man presented with pain and swelling of the right sternoclavicular joint along with low backache for 3 months. There was no history of fever, trauma, polyarthritis, and early morning stiffness. Magnetic resonance imaging (MRI) of the right sternoclavicular joint done outside was suggestive of osseous edema with effusion; joint aspiration was noncontributory. He was started on antituberculous treatment (ATT), but there was no relief, and after 15 days, he developed pain and swelling of left middle finger along with swelling of left second, third, and fourth toe. He was again evaluated and based on his high uric acid levels (8.5 mg %), he was started on zycolchicine and allopurinol along with nonsteroidal anti-inflammatory drugs, but the patient remained symptomatic. The right sternoclavicular joint was swollen but nontender. Hand and foot examination revealed left middle finger and left second to fourth toe dactylitis. Nail examination revealed pitting along with onycholysis of both toes and left second/third finger. His modified Schober's test was negative. Skin examination was normal. Antinuclear antibody, human leukocyte antigen B27, and rheumatoid factor were negative. MRI sacroiliac joint revealed bilateral sacroiliitis He was started on methotrexate, sulfasalazine, and steroids. His ATT and allopurinol were stopped. The patient showed dramatic improvement in his symptoms within 2 weeks.

Discussion: Psoriatic arthritis is a seronegative inflammatory joint disease which occurs on an average in 26% of patients with psoriasis. Hyperuricemia is seen in 20% due to the increased metabolic activity and may be mistaken as gouty arthritis sternoclavicular joint is involved in 90% of the patients with severe psoriatic arthropathy but is rarely a presenting complaint.

Conclusion: We hereby present a case of unilateral sternoclavicular joint arthritis treated as tubercular and gouty arthritis in the past and which later turned out to be psoriatic arthritis and that too without any obvious dermatological involvement.


  OPC0099: Sleep quality and fatigue in rheumatoid arthritis: Unanswered questions Top


A Aravindan, S Balameena Kumar1, R Ramesh1, Mythili Seetharaman1, Karthikeyan, Sujatha; Institute of Rheumatology, RGGGH, MMC, Chennai, Tamil Nadu, India

Background: Fatigue is common in rheumatoid arthritis (RA).[1] The complex interplay of sleep quality on fatigue has not been studied much.

Objective: This is a cross-sectional study for evaluating the correlation between the quality of sleep and the fatigue scale, in RA patients in remission.

Methods: Inclusion criteria: Patients (n = 46) who fulfilled American College of Rheumatology/European League Against Rheumatism 2010 RA classification criteria, and in remission were recruited. Exclusion criteria: Patients aged <16, serious comorbidities, patients on biologicals, and pregnant and lactating mothers.

Procedure: After written consent, patients were examined; disease activity (disease activity score 28, simplified disease activity index, and clinical disease activity index) were recorded. Fatigue was evaluated with FACIT-F and sleep quality was assessed using Pittsburgh Sleep Quality Index (PSQI). There were 32 (70%) females and 14 males (30%). The mean age was 36 ± 8.2 (interquartile range 28). The mean disease duration was 5.66 ± 2.9. 37 (80%) patients were on double disease-modifying antirheumatic drugs (DMARDs) methotrexate and hydroxychloroquine. Six (13%) patients were on triple DMARDs. Three (6.5%) patients had received biological in the form of injection etanercept 50 mg/every week for 12 doses. The mean FACIT-F score was 28.6 ± 8.9. The mean PSQLI score was 9.12 ± 3.8. The score of <5 was taken as poor sleep quality and >5 as poor sleepers. In poor sleepers (33 patients [71%]) the mean score was 2.2 ± 1.3. The fatigue scale among the poor sleepers was 24 ± 4.8. The mean fatigue scale among the good sleepers was 32 ± 3.7. The mean fatigue scale was 22.2 ± 3.4 in females and 38 ± 2.9 in males. Spearman's correlation coefficient was 0.27 (P < 0.03).

Conclusion: Fatigue is common in RA. This study was able to document fatigue even in patients in remission. Fatigue was more common in female patients than males. Sleep might be important in the multifaceted etiology of fatigue.

References

  1. Katz P. Causes and consequences of fatigue in rheumatoid arthritis. Curr Opin Rheumatol 2017;29:269-76.



  OC0031: Real-world use of tofacitinib in rheumatoid arthritis: Data from a single North India tertiary care hospital Top


Prasan Deep Rath, Swetal Pandey; Max Super Speciality Hospital, New Delhi, India

Background: Tofacitinib is an oral Janus kinase inhibitor for the treatment of rheumatoid arthritis (RA). Although there are multiple trials published illustrating efficacy and safety, real-world data are limited.

Objectives: The objective was to evaluate real-world efficacy and safety in patients not responding to conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) or biological disease-modifying antirheumatic drugs (bDMARDs) in single North Indian center.

Methods: Data of RA patients treated with tofacitinib 5 mg BID from a tertiary hospital in New Delhi were collected from February' 2017 to August' 2018 using standardized formats at baseline and 3 and 6 months. Efficacy was evaluated by clinical disease activity index (CDAI), visual analog scale for patient global assessment, safety by listing of adverse events (AEs)/serious AEs. Analysis was based on observed values without imputation of missing data.

Results: Among 39 patients, females outnumbered (n = 29, 74%). Mean age/disease duration mean (range), 51 (34–72) years, 7.38 (1–25) years respectively, 87% of patients rheumatoid factor positive/anti-citrullinated peptide antibody positive. Patients were at various stages of therapy. Tofacitinib was given in post-csDMARD-inadequate response (all triple csDMARD-IR) in 87% patients and after >1 bDMARD (all with biologics post triple csDMARD-IR) in 13% patients. Used mostly combined with csDMARD in 87% patients (mostly with methotrexate [MTX]/or double/triple csDMARD combination) as monotherapy in 13%. Average MTX dose was 17.5 mg/week. At 3 and 6 months, the percentage of patients in CDAI remission/low disease activity/moderate disease activity was 0/58%/42% and 0/80%/20%, respectively. All patients were off steroids at 3 months. 7.6% of patients diagnosed with latent tuberculosis (TB) infection. Six adverse events (AEs) were noted; mild transaminitis (2 patients), facial skin pigmentation (1 patient), bilateral (B/L) knee heaviness (1 patient), B/L pedal edema (1 patient), death (1 patient, duration of therapy 2 days). No cases of tuberculosis/herpes zoster were observed. Most AEs were mild and reversible. Eleven patients discontinued, 5 lost to follow-up, 3 reasons not specified, 2 IR (2), and 1 AE.

Conclusion: Patients with RA showed good CDAI response at 3 months and sustained through 6 months. No new safety signals were identified. These limited real-world data are reassuring, but it would need validation with prolonged use in larger population.


  PC0034: Clinical profile of adult immunoglobulin A vasculitis in a tertiary care hospital in South India Top


Vishnu S Chandran, C B Mithun; Department of Rheumatology, Amrita Institute of Medical Sciences and Research Centre, Kochi, Kerala, India

Background: Immunoglobulin A-associated vasculitis (IgAV) is an immune complex-mediated small vessel vasculitis which is regarded as a disease of the childhood. It was for the same reason the initial American College of Rheumatology classification criteria was meant for an age group <20 years. However, it has been recently shown that the disease is by no means rare in adults but is different from the presentation in children.

Objective: The main objective was to describe the clinical profile of adult IgAV who presented to our institute.

Methods: Thirty-six adult patients who fulfilled the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitis definition of IgAV at our institute during 2015–2018 were critically reviewed. The continuous variables were expressed as mean ± standard deviation. Categorical variables were expressed in percentage.

Results: The mean age of presentation was 42.72 years with a male:female ratio of 17:19. All the patients had palpable purpura at the time of presentation and 34 patients had histopathologic evidence. Only 29 patients had immunofluorescence positivity.

Conclusion: In comparison with the previous studies, arthritis was found to be significantly lower in our study group. Blood in stools was seen only in a very small percentage of the cohort. The disparity between skin changes and histopathologic features in few was attributable to steroid modifications. Recurrence was seen in eight patients with female predominance despite treatment. None of them had solid organ tumors.


  OPC0103: Still's disease in an elderly female with early joint erosion: Rare presentation in India Top


Rajdeep Basu, Omkarr De Hazra, Srabani Ghosh, Soumitra Ghosh, Sumantro Mondal1; Departments of Internal Medicine and 1Rheumatology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

Case Report: A 65-years-old female patient presented with high-grade (~104°F) fever for 3 months before admission, associated with bilateral multiple small and large joint pain and swelling. Although there was a history of macular rash over body during peak of fever, there was no rash at time of presentation. The patient had bilateral axillary lymph node enlargement and persistently high total leukocyte count along with granulocytosis. Erythrocyte sedimentation rate and C-reactive protein were raised. No source of infection was detected. Rheumatoid arthritis and antinuclear antibody were negative. Ferritin level was 2870 μg/L. The patient was fitting with Yamaguchi criteria for diagnosis of Still's disease. Although no joint deformity was visible [Figure 1], power Doppler US of both wrist joints showed bilateral synovial proliferation and joint erosion. The patient was started with oral steroid and methotrexate. Symptoms were improving even with tapering dose of steroid.



Discussion: Adult-onset Still's disease is a rare systemic inflammatory disorder. Newer studies showed a bimodal age distribution on presentation. In Indian studies, mean age of distribution were 27.8 and 24.6 years. Onset in older people is rarely documented in the literature [Table 1]. Further, early joint erosion is not common.
Table 1: Previous cases of elderly-onset Stillís disease


Click here to view


Conclusion: Still's disease in the elderly, studies are necessary for treatment planning, especially when other comorbidities present and many drugs cannot be used freely.


  OPC0106: Subclinical renal disease in rheumatoid arthritis Top


Pravin Jain, Amita Agarwal; Department of Clinical Immunology, Sanjay Gandhi Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Renal involvement in rheumatoid arthritis (RA) can occur secondary to RA, drugs used for RA, or due to unrelated causes. Although glomerulonephritis (2.9%) and amyloidosis (1%) are rare complications; subclinical renal disease occurs in a significant proportion of patients (COMEDRA cohort study, 8.8%; Haroon et al., 17.5%). In India with the use of over-the-counter drugs and less attention to comorbidities, the prevalence could be higher. Thus, we assessed the prevalence of renal diseases among patients with RA.

Methods: Patients with RA (American College of Rheumatology 1982 criteria) were enrolled during their regular visit. Renal function was assessed by measuring estimated glomerular filtration rate (eGFR) through abbreviated modification of diet in renal disease formula. Dipstick urine was done for proteinuria and if significant quantitation was done. Risk factor association was measured by odds ratio (confidence interval).

Results: There were 217 patients (180 females) with mean age of 46 years; mean disease duration of 9.3 years. 78.7% were rheumatoid factor positive, and 84.4% were anti-citrullinated peptide antibody positive. All were on disease-modifying antirheumatic drugs, and 26.7% were using nonsteroidal anti-inflammatory drugs (NSAIDs) off and on and 40.5% were on low-dose prednisolone. 18 (8.3%) had diabetes whereas 21% were hypertensive (HTN). 35 out of 217 (16%) patients had estimated glomerular filtration rate <60 ml/min and five patients had serum creatinine >1.4 mg/dl. Based on the National Kidney Foundation criteria, 13.3% had Stage 3a, 1.8% Stage 3b, and 0.9% had Stage 4 chronic kidney disease (CKD). Hypertension (HTN) (3.125 [confidence interval (CI) - 1.4–6.7]), male gender (3.9 [CI - 0.9–17.1]), and extra-articular features (2.4 [CI - 1.1–5.2]) were significant (P < 0.05) risk factors for CKD. Two patients were detected to have amyloidosis. Among these 35 patients with CKD, 23 (65.7%) were on methotrexate (15–25 mg), 3 (8.5%) were on leflunomide, and 7 (20%) were on NSAIDs.

Conclusion: The prevalence of CKD in our cohort is 16.2%. HTN, male gender, and extra-articular features are risk factors for CKD. These patients need careful monitoring of nephrotoxic and other drugs excreted through the kidney.


  OPC0133: Aortitis in a case of granulomatosis with polyangiitis: A case report Top


Satyam Bhatt, Gp Capt V Vasdev, Col Arun Hegde, Lt Col Ashwini Kumar, Sg Cdr Ramakant; R & R Hospital, New-Delhi

Case Report: A 30-year-old man presented with prolonged low-grade fever, migratory joint pains, dry cough, and significant weight loss of 5 weeks duration. He also reported a transient self-limiting erythematous nonpruritic rash involving both lower limbs that resolved over a week. Clinical examination revealed bilateral episcleritis, nasal crusting and ulcerations, and oligoarthritis involving both shoulders and right knee joint with normal systemic examination. Laboratory tests revealed normocytic normochromic anemia (Hb 12.2 g/dl), progressive azotemia with active urine sediment, c-antineutrophil cytoplasmic antibody (c-ANCA) immunofluorescence pattern, and elevated anti-PR3 levels. Workup for infective etiology, including tuberculosis, was negative. Contrast computed tomography (CT) chest showed the presence of centrilobular ground-glass opacities. Contrast CT and magnetic resonance imaging of the abdomen showed features of inflammatory concentric wall thickening of abdominal aorta without involvement of its branches. Renal biopsy showed crescentic focal necrotizing glomerulonephritis. He was managed with pulse methylprednisolone followed by oral steroids and cyclophosphamide as per the EUVAS regimen.

Discussion: Granulomatosis with polyangiitis (GPA) is a rare multisystem disorder characterized by necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract and necrotizing vasculitis affecting predominantly small-to-medium-sized vessels. Large vessel involvement in GPA, though rarely reported in medical literature, may present as stenosing large-vessel arteritis, aneurysmal disease, aortic dissection, aortic rupture, aortic regurgitation, and death. Here, we describe a case of GPA with large vessel vasculitis involving the abdominal aorta.

Conclusion: Although rare, large-vessel vasculitis can precede small-vessel vasculitis or occur in the absence of small-vessel involvement in GPA and ANCA-associated vasculitis should be considered in the differential of aortitis.


  OPC0203: Takayasu's arteritis: Experience from a tertiary care hospital in Northern India Top


Arun Hegde, Vivek Vasdev, Ashwani Kumar, Kunal Kishore, Ramakant Singh, Satyam Bhatt; Department of Rheumatology and Clinical Immunology, Army Hospital (Research and Referral), New Delhi, India

Background: Takayasu's arteritis (TA) is a granulomatous vasculitis of large vessels. The disease shows varied demographic spectrum among different population. There is limited number of data regarding the epidemiology of TA in India.

Objectives: This study was intended to study the epidemiology of TA among patients presenting to a tertiary care center in North India.

Methods: A total of 30 angiographically proven TA patients who met the American College of Rheumatology 1990 Criteria for TA were included in this study from 2013 to 2017. Demographic, clinical, laboratory, and imaging variables and treatment profile were observed.

Results: The mean age at presentation was 26.36 ± 11.4 years. Female:male ratio was 5:1. Mean disease duration before diagnosis was 8.7 ± 5.7 years. Upper limb claudication was the most common symptom (79%) followed by syncope (41%), carotidynia (24%), lower limb claudication (17%), chest pain (14%), and stroke (10%). 31% of patients had systemic symptoms at initial presentation. The mean erythrocyte sedimentation rate was 50 mm 1st h and mean C-reactive protein was 30 mg/L. 83% patients underwent computed tomographic angiography as first choice of imaging and 17% underwent magnetic resonance angiography. The left subclavian artery was the most common vessel affected (80%). Left renal artery was involved in 24% and right renal artery in 20% of cases. 40% underwent fluorodeoxyglucose positron emission tomography (FDG PET) scan to assess for disease activity, 16% of whom showed evidence of activity on FDG-PET. The most common angiographic types were Type 1 and Type V (both 33.3%). Methotrexate was used as the first-line steroid-sparing drug in 90% of patients whereas 10% used mycophenolate mofetil. 13.5% patients required tocilizumab for refractory disease.

Conclusion: Our study helps elaborate the epidemiological profile of TA in India. We however found significant variations in gender distribution, presenting symptoms, angiographic types, and management profiles as compared to previous studies in India.


  OPC0204: Secondary renal amyloidosis in a tertiary rheumatology center Top


Akash Khune, C Balakrishnan, Gurmeet Mangat; P.D. Hinduja National Hospital and MRC, Mumbai, Maharashtra, India

Introduction: The incidence of secondary amyloidosis (SRA) in inflammatory arthritis has reduced over the past few decades due to aggressive therapy. With this in mind, we analyzed the cases of systemic amyloidosis we had seen over the last 10 years.

Methods: Over the last 10 years, all patients with inflammatory arthritis who had nephrotic range proteinuria and biopsy-proven renal amyloidosis were analyzed. Epidemiological data, clinical features, and response to therapy were included in the analysis.

Results: In all 6 (5 adults [3 females] and 1 juvenile idiopathic arthritis [JIA] female) patients were seen. The median age of adults was 43 years. Among the adults, four had rheumatoid arthritis (RA) and one had ankylosing spondylitis (AS). Median duration of RA was 14 years and JIA was of 4 years when amyloid was detected. The proteinuria was ongoing for a median duration of 15 months when SRA was diagnosed and median urine protein/creatinine ratio was 4.46. Baseline creatinine was elevated in only one patient. Among RA patients, four were rheumatoid factor positive and two anti-cyclic citrullinated peptide positive. The female JIA patient was antinuclear antibody negative. The AS patient was human leukocyte antigen B27 positive. All were treated with steroids and synthetic disease-modifying antirheumatic drugs (methotrexate 5/6, leflunomide 1, hydroxychloroquine 1, and salazopyrin 1). Biologics were used in four patients (adalimumab in 3, etanercept 1). The median duration of follow-up was 8.5 years. There was significant improvement (reduction by >50%) in proteinuria in only two patients with no change in the rest. The creatinine remained normal in 4 and worsened in 2 patients. The AS patient who had elevated creatinine at diagnosis of SRA developed end-stage renal disease and is on maintenance dialysis.

Conclusions: SRA is a rare entity in our rheumatology unit. Baseline creatinine predicted the long-term outcome of SRA. Aggressive therapy even with biologics may not reverse the proteinuria.


  PC0062: Complex regional pain syndrome: A forgotten entity Top


Pankaj Gupta; Dr. RML Hospital, New Delhi, India

A 25-year-old female presented with complaints of weakness and pain in left hand involving third, fourth, and fifth digits associated with pain in the left wrist, elbow, and shoulder joint associated with swelling and redness along with blackish mottling of the overlying skin. She sought medical consultation at dermatology clinic first for discoloration and skin changes before consulting various other specialties and ultimately being referred to our rheumatology clinic. In our rheumatology clinic, the clinical history was reviewed. There was flexion deformity at proximal and distal interphalangeal joints involving left third, fourth, and fifth digits with severe tenderness. There was associated soft tissue swelling and skin changes in the form of blackish discoloration and dryness and scaling but with no synovitis. Pain was assessed with visual analog scale, the score of which was 9 at presentation. Most of her laboratory reports were normal. Meanwhile, she was started on antifungals and nonsteroidal anti-inflammatory drugs. However, she still complained of persistent pain. Radiograph of hand showed marked regional osteopenia with bone resorption. A technetium-labeled methylene diphosphonate triple-phase bone scan was advised which revealed increase in radiotracer accumulation in the region of left forearm and left wrist, left shoulder joint, left elbow joint and left wrist joint, metacarpophalangeal joint, and proximal interphalangeal joint of third and fourth fingers of the left hand. Hence, a diagnosis of complex regional pain syndrome (CRPS) type II was made according to Budapest criteria. As the patient did not respond to NSAIDs, amitriptyline and pregabalin, left stellate ganglion block was given which resulted in pain resolution.

Discussion: CRPS is a clinical diagnosis based on symptoms and signs elicited. The Budapest clinical diagnostic criteria have a sensitivity of 99% and a specificity of 68% for the diagnosis of CRPS.

Conclusion: CRPS is often missed because of vague symptoms and disease mimickers.


  OPC0110: IgG4-related disease: Experience at a tertiary rheumatology center Top


Akash Khune, C Balakrishnan; P.D. Hinduja National Hospital and MRC, Mumbai, Maharashtra, India

Introduction: IgG4-related disease (IgG4-RD) is a new disease described only a decade ago. We present a series of patients seen at our rheumatology center over the past 5 years.

Methods: A retrospective study was done of all patients who had histopathological evidence of IgG4-RD, including light microscopic features with or without immunohistochemistry (IHC). Relevant differential diagnoses including chronic infections and malignancy were excluded.

All patients had their epidemiological data, clinical features, investigations, and response to treatment analyzed.

Results: In all, 14 patients were seen (male:female = 8:6). The median age was 39.5 years, and the median duration of illness was 12 months. Orbit, sinuses, mediastinum, and retroperitoneum were the most common sites involved [Table 1]. Median serum IgG4 level was 7.5 g/dl. All had typical histological evidence of IgG4. IHC was positive in 10/10 patients in whom it was done. All were treated with steroid, six were also treated with tamoxifen, and six were treated with methotrexate. One was switched from tamoxifen to methotrexate. One patient who was resistant to tamoxifen, and methotrexate was treated with rituximab. There was follow-up of at least 1 year in 12 patients, and there was improvement on imaging in 10 patients and the lesions were static in 2. While on therapy, one developed pulmonary tuberculosis, one had recurrent urinary tract infection, and one needed DJ stenting for ureteric stenosis.



Conclusions: It is one of few case reports showing efficacy of methotrexate in IgG4-RD. IgG4-RD was seen predominantly in the orbit, ear nose throat, mediastinum, and retroperitoneal areas. Rare subglottic involvement was also seen. All had elevated serum IgG4 levels with positive IHC in 20 patients. All were treated with steroids. Tamoxifen and methotrexate are useful steroid-sparing agents. Majority improved.


  PC0066: An interesting case of chronic diarrhea in a young male with pyrexia of unknown origin and acute renal failure Top


Pulin Kumar Gupta, Arvind Chaudhry, Anand Vishal, Balshine Singh, Saurabh Tyagi, Narender Sharma; Department of Medicine, PGIMER, Dr. RML Hospital, New Delhi, India

Case Report: A 21-year-old male presented with fever, abdominal pain, and diarrhea with 8 kg weight loss for 2 months followed by bilateral pedal edema, oliguria, and rash on lower extremities for 5 days. History was unremarkable except that he was on antituberculous treatment for the last 6 weeks empirically for abdominal Koch but to no benefit. His vitals were normal and abdominal examination was unremarkable. Normocytic normochromic anemia with raised erythrocyte sedimentation rate (80 mm/h) and deranged kidney functions (urea-60 mg/dl, creatinine-2.8 mg/dl) along with 2.2 g/day urinary protein were present.

Upper gastrointestinal endoscopy and colonoscopy were normal, and C-reactive protein was high (>32 mg/dl]. An extended autoimmune profile (extractable nuclear antigen) revealed antinuclear antibody (1:640) positive, anti-dsDNA-402 IU/ml, antineutrophil cytoplasmic antibody-negative; anti sm (2+), ssa/ro-(4+), ssa/la-(2+), and antinucleosome-(1+). Interferon-gamma release assay (TB Gold), Mantoux, HIV, hepatitis B surface antigen, and antihepatitis C virus were negative. Skin biopsy revealed leukocytoclastic vasculitis. Complement levels were low. Kidney biopsy revealed grade-4 lupus nephritis. Renal and pulmonary angiogram was normal. Contrast-enhanced computed tomography (CECT) abdomen revealed jejunal and ileal edematous bowel wall thickening with changes more marked in the jejunum and increased vascularity of the mesentery with minimal ascites suggestive of lupus enteritis. High-dose pulse steroids along with hydroxychloroquine and mycophenolate were started. The symptoms and laboratory parameters normalized in 1 week. After 3 months, CECT abdomen is unremarkable and proteinuria decreased to 0.4 g/day with normal kidney function test and he had gained 12 kg weight.

Discussion: The diagnosis of lupus enteritis is based on classical computed tomography findings (bowel wall edema, mesenteric abnormalities, and ascites) and definite histologically proven lupus from any other organ (like nephritis in our case). Typically, lupus enteritis is steroid responsive with an overall excellent prognosis and reversibility.

Conclusion: One should think of lupus enteritis (and not only Kochs) in all patients with systemic lupus erythematosus presenting with pain abdomen. Immunosuppressive treatment is reserved for recurrent enteritis or cases with multiorgan involvement.


  OC0028: Effect of tocilizumab and mycophenolate mofetil in patients with Takayasu's arteritis - interleukin-6, tumor necrosis factor alpha, and C-reactive protein assessment: One-year follow-up study from a tertiary care center Top


Rajeswari Sankaralingam, Bhuvanesh Mahendran, Balaji Chilukuri, Saranya Chinnadurai, Vignesh Mantharam, Euphrasia Latha, Tamilselvam TN, Balameena S, Saravanan M, Therese Mary; Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Takayasu's arteritis (TA) is enigmatic and erratic. Till date, no definite outcome measures are available. An attempt has been made with tocilizumab (TCZ) and mycophenolate mofetil (MMF) treatment with an assessment of interleukin (IL-6), tumor necrosis factor alpha (TNF-α), and C-reactive protein (CRP).

Methods: This is a prospective observational study of 17 TA patients (recalcitrant severe disease, aneurysms, cardiac, strokes, thrombosis, and eye involvement). Nine received TCZ therapy, and MMF therapy was given to eight patients. IL-6, TNF-α, and CRP were assessed at baseline, 6 months, and 1 year after treatment. Twenty age- and sex-matched healthy controls were also included in the study.

Results: Clinical response in TCZ group was dramatic, and response to MMF remained stable. TNF-α and IL-6 levels were higher in the TCZ and MMF groups when compared to healthy controls at baseline. Mean IL-6 levels increased after TCZ and MMF at 6 months but decreased below the baseline levels at 1 year of follow-up as in Figure 1. Mean TNF-α levels increased in both groups at 6 months and 1 year as depicted in [Figure 1]. Mean CRP levels decreased in both groups at 6 months (22.5–6 mg/L in MMF group and 18.61–0.5 mg/L in TCZ group); decreased further in MMF group (1.13 mg/L), and increased in TCZ group to 1.58 mg/L at 1 year. The median levels of IL-6 and TNF-α remained similar at 6 months and 1 year between MMF and TCZ groups (P = nonsignificant, Mann–Whitney test).



Conclusion: Patients with recalcitrant TA showed dramatic response to TCZ, but the patients on MMF remained stable. The reason for increase of TNF levels with treatment despite decrease in IL-6 and CRP levels at 1 year needs to be evaluated further.


  OC0027: A rare case of Salmonella typhi associated sacroiliitis, not so rare Top


Pulin Kumar Gupta, Garyll Tariang Blah, Ulka Kamble, B M S Lamba, Saurabh Tyagi, Manita Khatak; Department of Medicine, PGIMER, Dr. RML Hospital, New Delhi, India

Case Report: A 17-year-old male presented with high-grade fever with diarrhea, abdominal pain, and left hip pain for 10 days. Abdominal examination revealed generalized tenderness but no organomegaly. Rest of the systemic examination was unremarkable. Left sacroiliac joint was tender, and the range of motion was terminally impaired. His routine laboratory parameters were unremarkable except high erythrocyte sedimentation rate (65 mm/h) and alkaline phosphatase. All other investigations as part of fever workup were normal. Widal test was positive in titers of 1:160 and subsequent titers were >1:640. IgM ELISA against Salmonella typhi was positive, blood cultures were sterile, but bone marrow cultures grew S. typhi sensitive to aminoglycosides, azithromycin, and ceftriaxone. X-ray pelvis revealed unilateral left grade II sacroiliitis. Magnetic resonance imaging (MRI) pelvis revealed left sacroiliitis (right normal) with a loculated soft tissue collection in left iliacus muscle with postcontrast enhancement. Ultrasonography aspiration of the joint and collection was unsuccessful. Contrast-enhanced computed tomography (CECT) abdomen showed thickening of terminal ileum with abdominal lymphadenopathy without ascites. Hepatitis B surface antigen, antihepatitis C virus test, and ELISA for HIV were negative.  Brucella More Details serology, Mantoux, human leukocyte antigen-B27, antinuclear antibody, and interferon-gamma release assay (TB Gold) were also negative. The patient was diagnosed as a case of enteric fever with typhoidal sacroiliitis with iliacus collection. He was treated with dual intravenous followed by oral antibiotics for 2 months, and he responded well to that. Contrast MRI pelvis and CECT abdomen after 3 months were unremarkable.

Discussion: Salmonella sacroiliac arthritis can be reactive or infection of the joint per se. Among various findings, periarticular muscle edema was found to be the single most important predictor of infectious sacroiliitis (which was probably seen as iliacus collection in our case).

Conclusion: Pyogenic sacroiliitis due to Salmonella is not unusual. Timely diagnosis and longer duration of treatment can save the affected sacroiliac joint and prevent any future disability.


  PC0069: Scleromalacia perforans in a patient of leprosy Top


Manesh Manoj, Akhil Pawan Goel, Sourav Pradhan, Prashant Bafna, Urmila Dhakad, Siddharth Kumar Das; Department of Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India

Background: Leprosy can present with a variety of ocular manifestations, but scleromalacia perforans has been rarely reported. We report a case of leprosy presenting with scleromalacia perforans and polyarthritis causing a diagnostic dilemma.

Case Report: A 38-year-old female presented with symmetrical polyarthritis for 3 years, pain in both eyes for 6 months, and decreased vision for 2 months. It was associated with redness and photophobia involving both eyes. She also had numbness and tingling sensations over both hands and feet for the last 6 months without motor weakness. On examination, she had symmetrical polyarthritis of small and large joints with correctable swan neck deformities, vague diminution of sensation over both hands and feet, and eye features characteristic of scleromalacia perforans.

Investigations showed Hb: 9.7 g/dl; total lymphocyte count: 7300/mm3; platelet count: 3 lac/mm3; erythrocyte sedimentation rate: 39 mm; C-reactive protein: 1 mg/L; rheumatoid factor: 25 (<20); anti-citrullinated peptide antibody: negative; MPO, PR3: negative; antinuclear antibody: negative; HIV, hepatitis B surface antigen, hepatitis C virus: nonreactive; urine R/M: normal; thyroid-stimulating hormone: 2.68 μg/dl; X-ray hands: mild juxta-articular osteopenia; X-ray pelvis: normal; nerve conduction study: mononeuritis multiplex. Ophthalmology opinion was sought which confirmed scleromalacia perforans along with secondary glaucomatous optic atrophy. Sural nerve biopsy for histopathological examination was done. In view of polyarthritis, scleromalacia perforans with worsening ocular symptoms, and mononeuritis multiplex, a diagnosis of rheumatoid arthritis vasculitis with scleromalacia perforans versus undifferentiated vasculitis was considered and the patient was given pulse steroids followed by 1 mg/kg steroids, and cyclophosphamide was planned. Meanwhile, her sural nerve biopsy report showed lepromatous leprosy with bacillary index 6+. She was started on multidrug therapy, and on follow-up, her arthritis subsided, but her vision remained the same.

Conclusion: Leprosy can present in a multitude of ways and scleromalacia perforans can be a rare but grave manifestation.


  OPC0205: Body composition in psoriatic arthritis and its relationship with the disease activity, cytokine profile, and adipokines Top


K C Shanoj, K G Chengappa, Pooja Belani, V S Negi; Department of Clinical Immunology, JIPMER, Puducherry, India

Background: Psoriatic arthritis (PSA) is associated with increased prevalence of metabolic syndrome (MS) and threefold increase in cardiovascular risk. Insulin resistance is central to the pathogenesis of MS, causing abnormal adiposity and sarcopenia. The association between disease activity, MS, and sarcopenia is not well characterized. This is an interim report of ongoing study exploring the association between these factors in PSA.

Objectives: The objectives were to compare appendicular lean mass (ALM) among PSA and healthy controls (HCs) and to compare anthropometric parameters, ALM, fat distribution, disease activity, cytokine profile and adipokines in PSA with and without sarcopenia.

Methods: Sixty-one patients with PSA and 33 HC were enrolled in the study. Body composition measured using dual-energy X-ray absorptiometry. ALM/body mass index (BMI) £0.789 in males and £0.512 in females were defined as sarcopenia. Circulating cytokines (interleukin-6, tumor necrosis factor alpha, monocyte chemotactic protein-1) and adipokines (leptin, adiponectin, omentin, resistin) were measured by ELISA.

Results: Demographic factors, BMI, and waist circumference were comparable among PSA and HC. ALM/BMI was less in PSA males (0.80 and 0.92, P = 0.004). Total body fat percentage and distribution were similar in PSA and HC. PSA group had higher serum leptin, omentin, resistin, and MCP-1 and lower adiponectin (P ≤ 0.05). PSA with sarcopenia was associated with higher BMI (26.76 ± 3.71 vs. 24.56 ± 4.19, P = 0.045), waist circumference (90.65 ± 9.69 vs. 83.21, P = 0.007), and less disease activity. Total cholesterol was more in PSA with sarcopenia (188.73 ± 40.94 vs. 169.85 ± 31.48, P = 0.048); however, total cholesterol high-density lipoprotein ratio was similar. PSA with sarcopenia had higher total body fat (39.12 ± 6.12 vs. 33.60 ± 7.03, P = 0.003) and android-gynoid ratio. Inflammatory cytokines were similar in PSA with and without sarcopenia.

Conclusion: The appendicular muscle mass was less in male patients with PSA. Sarcopenia in PSA is associated with higher BMI, central obesity, body fat percentage, abnormal fat distribution, and higher leptin but less disease activity.


  PC0067: Rhupus with pyoderma gangrenosum treated with immunosuppression and skin grafting Top


Nupoor Acharya, Arghya Chattopadhyaya, Aman Sharma, Sanjay Jain

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India

A 26-year-old man presented with jaundice and anasarca, fever, and a large nonhealing ulcer on left leg for a month and a history of symmetrical, additive, deforming, inflammatory joint pains and multiple ulceration over both legs, recurrent oral ulcerations and alopecia over the past 4 years. The patient has been taking over-the-counter medications, alternative therapy, and recreational drugs, the records of which were not available. He was pale and icteric and had diffuse alopecia. A large ulcer with a necrotic base covering two-third of his left leg was present with pus discharge [Figure 1]. Multiple healed scars were present on right leg. Multiple noncorrectable deformities involving both hands were present. He had gross ascites with hepatosplenomegaly and bilateral pleural effusion.



On investigations, he had anemia (hemoglobin – 5.7 mg/dl), leukocytosis, and thrombocytopenia which gradually recovered. He had predominant conjugated hyperbilirubinemia with normal transaminases, hypoalbuminemia (1.7 g/L), and deranged renal functions which improved during hospital stay. Urinary examination revealed proteinuria of 2.6 g/day with no active sediments. All cultures were sterile, and serology for HIV, hepatitis B, and hepatitis C were negative. He tested positive for antinuclear antibody, rheumatoid factor (3910 IU/ml), anti-cyclic citrullinated peptide (>3000 μ/ml), and direct Coomb's test (IgG). He also had hypocomplementemia. Skin biopsy was suggestive of pyoderma gangrenosum. Workup for autoimmune hepatitis was negative. Renal biopsy was suggestive of membranous nephropathy with IgG and complement deposits.

A diagnosis of rhupus with pyoderma was made. The patient received oral steroids, intravenous antibiotics, and mycophenolate mofetil. A plastic surgery consultation was taken, and he underwent skin grafting. He responded to the therapy and the ulcer healed well. At 6 months follow-up, he had mild ascites with proteinuria of 600 mg/day, and skin graft was healthy with no new skin lesions.


  OPC0111: A study on clinical and radiological profile in patients of Calcium Pyrophosphate Dihydrate Crystal Deposition Disease (CPPD) Top


Sourav Pradhan, S. K. Das, Urmila Dhakad; King George's Medical University, Lucknow, Uttar Pradesh, India

Backgrounds: Although calcium pyrophosphate dehydrate (CPPD) is considered as the third most common form of inflammatory arthritis, till date there are no data on CPPD in India except some case reports (Part of this study was presented in OACON 2018).

Objectives: The present study evaluates the clinical as well as radiological profile in CPPD patients especially in India.

Methods: A total of 33 patients of CPPD diagnosed by polarized light microscopy synovial fluid crystal examination were included in the study. Thorough history-taking, clinical examinations, X-ray knee, hand, and pelvis, as well as B mode grayscale ultrasonography (USG) at 11–13 MHz were done for both knee and hand to detect chondrocalcinosis (CC). In USG, CC was detected as hyperechoic spots, aggregates, or linear deposits.

Results: In the study, population age ranged from 37 to 70 years. There were 14 males and 19 females. Clinical presentation was acute CPPD in 16 and chronic CPPD in 17 patients. Of these 33 patients, 21 had osteoarthritis-associated CPPD, 6 had pseudogout, and 6 had pseudo-rheumatoid-like presentation; 4 had overlap with rheumatoid arthritis, and one had overlap with psoriatic arthritis. On X-ray, seven patients had CC of hyaline cartilage, 3 had CC of meniscal cartilage of knee, and 4 had anterior cruciate ligament calcification, but none found to have triangular cartilage CC in hand. In USG of the knee, 10 had hyaline cartilage, 30 have medial meniscal, and 6 have lateral meniscal cartilage CC. In hand, 20 had ultrasonographic CC of triangular cartilage.

Conclusion: In this observational study, we found that CPPD is quite common in Indian patients and USG is more sensitive than X-ray in detecting CPPD crystal deposition.


  OPC0115: Polyserositis in a case of scleroderma: A rare entity Top


Anunay Agarwal; Fortis Flt. Lt. Rajan Dhall Hospital, New Delhi, India

Case Report: We present the case of a 49-year-old male presenting with a 6-month history of skin tightening over the face and upper limbs, progressive dyspnea, and Raynaud's phenomenon. Clinical examination showed calcinosis cutis over the fingers and dorsum of feet, sclerodactyly and skin thickening extending up to the mid-forearm, restricted mouth opening, and telangiectasias, and chest examination revealed basal crepitations. Antinuclear antibody was positive at 1:320 (homogeneous + speckled pattern). His anti-Scl-70 was also positive. High-resolution computed tomography showed interstitial lung disease (ILD) (nonspecific interstitial pneumonia pattern). Echocardiography revealed mild pulmonary arterial hypertension (PAH). He was diagnosed as having scleroderma with ILD and PAH and started on methotrexate and low-dose prednisone along with supportive management.

The patient was lost to follow-up for a year when he returned with polyserositis (massive ascites and mild pleural and pericardial effusion) and diffuse skin thickening. He did not have any rash and joint symptoms. Investigations revealed mild hypoalbuminemia, mildly decreased C3, normal C4, and negative anti-dsDNA. Thyroid function test and urine examination were normal. Ascitic fluid showed a total lymphocyte count of 800 (80% L) and total protein of 5 g/dl. A workup for liver cirrhosis, malignancy, and abdominal tuberculosis was negative. He improved after pulse methylprednisolone and intravenous cyclophosphamide.

Discussion: Polyserositis itself has not been reported as a part of scleroderma. Overlap with other rheumatological diseases such as systemic lupus erythematosus (SLE) is well known and polyserositis can occur with this condition. This case highlights the presence of polyserositis in scleroderma and no other features of overlap. There is only one case report (from Japan) of polyserositis in scleroderma.

Conclusion: This case is reported because polyserositis is a rare manifestation of scleroderma. He did not have SLE.


  OPC0116: Neutrophil-to-lymphocyte ratio in SLE is influenced by steroid usage and may not represent disease activity Top


Renuka P, Chandrashekara S, Anupama KR, Renuka BS; ChanRe Rheumatology and Immunology Center and Research, Rajaji Nagar, Bengaluru, Karnataka, India

Aim: The aims of the study were to evaluate the relationship between neutrophil-to-lymphocyte ratio (NLR) and other inflammatory disease activity parameters and to verify the factors influencing NLR in systemic lupus erythematosus (SLE) patients.

Materials and Methods: The cross-sectional study carried out from February to May 2018 involved 117 SLE patients fulfilling the SLICC/American College of Rheumatology criteria (2010). The following clinical and demographic characteristics of the recruited participants were recorded: age, gender, total lymphocyte count (TLC), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), complement C3, and the usage pattern of immunosuppressants/disease-modifying antirheumatic drugs, steroids, and biologics. The Systemic Lupus International Collaborating Clinics damage index and the SLE disease activity index (SLEDAI-2K) were also assessed. Based on the SLEDAI total score (based on safety of estrogens in lupus erythematosus national assessment-SLEDAI groupings), the patients were grouped as follows: minimum disease activity or remission (0–3), mild (4–8), moderate (9–11), and severe (12 and above) disease activity. Patients were reclassified on the basis of NLR values into three groups: ≤2, >2–4, and >4.

Results: The parameters, patient age, and number of immunosuppressants differed significantly among the mild, moderate, and severe SLEDAI disease groups. TLC, CRP, and steroid usage differed significantly among the NLR subgroups. The patients in >4 NLR group had elevated TLC and CRP levels compared to ≤2 and >2–4 NLR groups. NLR correlated with CRP. Mountain plots of CRP, NLR, ESR, and C3 had median (bias) >3 for SLEDAI. Receiver operating characteristic of NLR, ESR, and C3 failed to discriminate severe and mild–moderate disease activity. Multiple linear regression analysis demonstrated that CRP and steroids influenced NLR directly with the weak association.

Conclusion: CRP and steroid use influenced NLR in SLE patients. NLR was found to be not useful in classifying SLE patients based on disease activity. However, NLR demonstrated better agreement with moderate and severe disease activity SLEDAI scores compared to CRP.


  PC0068: Estimation of minimum clinically important difference in fibromyalgia for Fibromyalgia Impact Questionnaire-Revised using brief pain inventory as the anchor measure Top


Sandeep Surendran, C B Mithun; Department of Rheumatology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Background: The Fibromyalgia Impact Questionnaire (FIQ) was highly reliable and recognized as an outcome measure in fibromyalgia. Bennett et al.[1] have shown that 14% change in the FIQ total score represents the minimum clinically important difference (MCID). It was revised to the FIQ-Revised (FIQR) in 2009. However, there has been no study done for assessing the MCID of the FIQR in fibromyalgia.

Objective: The aim of our study is to estimate the MCID for FIQR using anchor-based methodology with average pain score on brief pain inventory (BPI) as the anchor.

Methods: From our prospectively followed cohort of fibromyalgia patients who were treated as per protocol with duloxetine, we used data to estimate the MCID for the FIQR using anchor-based methodology, with average pain score in BPI as the anchor. The MCID was calculated as the difference in the unadjusted mean change in the FIQR scores between the “nonresponder” group and the group with “responder group.”[2]

Results: [Table 1] shows the FIQR scores at baseline, endpoints, and the calculated MCID. The mean change of FIQR score between responders and nonresponders was 31.88 and 4.83, respectively. The MCID was calculated to be 27.04% or 45.5%.



Conclusions: That MCID of 45.5% obtained for the FIQR score is much higher than the 14% which was the MCID obtained for the older FIQ score but is likely a more clinical relevant MCID that should be used in clinical trials. Strengths of this work include the usage of prospectively followed patient population for analysis and protocol-based treatment with duloxetine.

References

  1. Bennett RM, Bushmakin AG, Cappelleri JC, Zlateva G, Sadosky AB. Minimal clinically important difference in the fibromyalgia impact questionnaire. J Rheumatol 2009;36:1304-11.
  2. Mease PJ, Spaeth M, Clauw DJ, Arnold LM, Bradley LA, Russell IJ, et al. Estimation of minimum clinically important difference for pain in fibromyalgia. Arthritis Care Res (Hoboken) 2011;63:821-6.



  OPC0118: CRP is a better marker of bacterial infection and procalcitonin level can supplement the grouping with more specificity Top


S Chandrashekara, K Devraj, P Renuka, G Shivputra, R Bharath, K R Anupama; Chanre Rheumatology and Immunology Center and Research, Bengaluru, Karnataka, India

Aim: The aim of the study is to evaluate the role of C-reactive protein (CRP), procalcitonin, and neutrophil-to-lymphocyte ratio (NLR) in differentiating bacterial infection requiring antibiotics from viral infections in AIRD patients.

Materials and Methods: A retrospective case–cohort study conducted from January 2017 to August 2018 involved AIRD patients with fever. The study excluded individuals with no established AIRD or the cause could be concluded. CRP, NLR, and procalcitonin levels were evaluated. The individuals were classified on the basis of infection, CRP and procalcitonin levels. Analysis of variance, Kruskal–Wallis, Chi-square, and kappa test were used.

Results: Out of the 103 screened individuals, the study recruited 78 individuals with an average age of 51 years and female:male ratio of 1:0.32. Majority of the individuals had rheumatoid arthritis (RA) (n = 30) followed by systemic lupus erythematosus (SLE) (n = 19). Bacterial and viral infections and noninfections/indeterminate causes for fever were noted in 39, 19, and 20 patients, respectively. Primary diagnoses of following autoimmune diseases were noted: RA 30, SLE 19, connective tissue diseases 7, ankylosing spondylitis 4, and other diseases 21. Comorbidities such as diabetes, hypertension, hypothyroidism, and ischemic heart disease were noted in 37 patients. The drug usage patterns noted were as follows: 69 on disease-modifying antirheumatic drugs, 51 steroids, 10 biologics, and 37 immunosuppressants.

Twenty-one with bacterial infection had CRP >100 compared to other groups. Most of the patients in the infection group had procalcitonin >0.25–≤1 ng/ml. The kappa agreement showed that CRP groups have a significant agreement with procalcitonin groups (kappa 0.481 and 95% confidence interval [CI] 0.320–0.642). The procalcitonin >1 had the sensitivity and specificity of 27.78% (95% CI 14.20%–45.19%) and 81.08% (95% CI 64.84%–92.04%), respectively. The CRP >100 mg/dl had the sensitivity and specificity of 55.26% (95% CI 38.30%–71.38%) and 75.68% (95% CI 58.80%–88.23%), respectively.

Conclusion: The present study showed that CRP >100 has better sensitivity in identifying bacterial infection and procalcitonin has higher specificity.


  OPC0119: Ankylosing spondylitis versus nonradiographic spondyloarthritis: A comparative study Top


Pandya S, Solanki R, Parikh T; Vedanta Institute of Medical Sciences, Ahmedabad, Gujarat, India

Background: While there are ample data on differences in clinical features between ankylosing spondylitis (AS) and nonradiographic axial spondyloarthritis (nraxSpA) from the West, data from our country are scarce.

Objective: The objective was to look at differences between clinical and laboratory features in our group of AS and nraxSpA patients.

Materials and Methods: This was a cross-sectional study. Data of all patients fulfilling AS and nraxSpA as per the ASAS criteria who had presented to our outpatient department from January 2013 to July 2018 were retrieved and analyzed for differences.

Results: See accompanying table AS nraxSpA JPG. Patients of nraxSpA were significantly younger, early in disease with lesser Bath Ankylosing Spondylitis Activity Disease Activity Index and Bath Ankylosing Spondylitis Functional Index score.

Conclusions: Male:female ratio was not dissimilar between AS and nraxSpA patients and the latter were much younger, early in disease and had more peripheral arthritis.


  OC0029: NA Top


NA; National Center for Rheumatic Disesases

Background: Gout results from deposition of monosodium urate crystals in joints and tissues. It is commonly associated with comorbidities such as obesity, hypertension, abnormal lipid metabolism, and impaired glucose tolerance. The presence of metabolic syndrome could increase the risk of cardiac morbidity and mortality.

Objective: The objective was to study the prevalence of deranged metabolic parameters in gout.

Methods: An open-label, observational cross-sectional study conducted at NCRD, a tertiary care rheumatology center in Nepal. Patients diagnosed with gout by a rheumatologist were enrolled in and evaluated for the presence of metabolic syndrome. Metabolic syndrome was diagnosed if >3 of five criteria given by the National Cholesterol Education Program are fulfilled: (1) central obesity (waist circumference >90 cm in men and 80 cm in women); (2) concentration of triglyceride >150 mg/dL; (3) concentration of high-density lipoprotein cholesterol <40 mg/dL in men and 50 mg/dL in women; (4) blood pressure >130/85 mmHg or under medical treatment of hypertension; or (5) concentration of serum glucose in fasting state >100 mg/dL or under medical treatment of diabetes.

Results: A total of 523 patients with gout were included among which 97.1% were male. Mean age was 49.12 ± 12.80 years. The mean serum uric acid level was 7.29 ± 2.14 mg/dL. Acute gout was seen in 95.9%; chronic tophaceous gout in 2.1%; hyperuricemia in 1%; and polyarticular gout in another 1%. Most of the patients were overweight with mean body mass index of 27.05 ± 3.68 kg/m2. Around 30.6% of patients fulfilled three out of five criteria for metabolic syndrome whereas 60.6% fulfilled two criteria.

Conclusions: Deranged metabolic parameters are common in gout which might translate to increased cardiac morbidity and hence warrant screening and intervention as a preventive measure.


  OPC0120: Real world experience with Infliximab Biosimilar (BOW015®) in Ankylosing spondylitis – A sub-group analysis of the East India cohort Top


Pradip Sharma, Sukumar Mukherjee1, R N Sarkar2, Santosh Kumar Mandal3, Santa Naorem4, Tanoy Bose5, Kaushik Basu2, Ajit Surin6, Pradeepta Sekhar Patro7, J.R.Parida7, Mohammed Shamil8; Excel care, Guwahati, Assam, 1CMRI, Kolkata, 2Medical college, Kolkata, 3N H Rabindranath Tagore, Kolkata, 4Regional institute of Medical Sciences, Imphal, 5N H Rabindranath Tagore Surgical Centre, Kolkata, 6Apollo hospital, Bhubaneswar, 7Sum hospital, Bhubaneswar, 8Sr Manager- Medical Affairs, Sun Pharma Laboratories, Mumbai, Maharashtra, India

Background: Due to dearth of data on infliximab biosimilar (BOW015®), a pan India registry was planned to capture the efficacy and safety in real-world clinical settings. Here, we analyzed the subgroup data in ankylosing spondylitis (AS) from the East India cohort.

Objectives: The objective was to determine the efficacy and safety of BOW015 in AS patients.

Methods: Data were collected from multiple regions across the eastern region of India. Patients diagnosed with AS, having 4–6 months of follow-up data during the last 1 year, were included in the study. Patients who were given BOW015 for other indications, prior innovator infliximab, or other biologics were excluded out of the study. Primary variable, major clinical improvement, was defined as delta AS disease activity score (ASDAS) >2 from the baseline to 4–6 months of follow-up. For analysis, statistical tests were applied as appropriate.

Results: The cohort consisted of 149 patients, predominantly male (69.8%), with mean age of 36.75 ± 11.11 years and mean body weight of 58.26 ± 154 kg. Of the treated patients, 91 (61.1%) patients administered four doses, 10 (6.7%) patients administered three doses, 37 (24.8%) patients administered two doses, and 11 (7.4%) patients administered single dose of BOW015. In the final analysis set, 81 patients had baseline and visit-4 data. Among them, 74 (91%) patients achieved major improvement, 5 (6%) patients achieved clinically important improvement, and 2 (3%) were nonresponders at visit-4. Bifurcation of cohort according to disease activity from baseline to visit-4 and trends in Bath Ankylosing Spondylitis Activity Disease Activity Index (BASDAI) measurements were also computed. Five patients reported infusion-related reactions.

Conclusions: BOW015 showed significant improvement in ASDAS and BASDAI in patients with AS at the end of 4–6 months of follow-up, and the clinical benefits were apparent as early as the first dose of BOW015.


  PC0070: Prevalence of anti-cyclic citrullinated peptide antibody in asymptomatic first-degree relatives of rheumatoid arthritis patients Top


Ankur Bhattacharyya, P Dihingia, G R Pramod; Assam Medical College and Hospital, Dibrugarh, Assam, India

Background: Rheumatoid arthritis (RA) is a systemic inflammatory disorder characterized by chronic symmetric and erosive synovitis. Antibodies to citrullinated peptides are important marker for diagnosis and prognosis in RA. It is found that antibody seropositivity may precede clinically apparent RA by two decades, hence helpful in early diagnosis and initiation of treatment.

Objectives: The objective was to study the prevalence of seropositivity for anti-cyclic citrullinated peptide (anti-CCP) antibody in asymptomatic first-degree relatives (AFDRs) of RA patients.

Materials and Methods: This was a hospital-based study conducted from June 2013 to May 2014 in Assam Medical College and Hospital, Dibrugarh. A total of 85 patients with RA as per the American College of Rheumatology diagnostic criteria were included. A total of 105 FDRs of those RA patients and 105 controls belonged to the same geographical area having no family history of autoimmune disease were enrolled. FDR and control group completed a questionnaire that asked about any of the symptoms of joint pain, swelling, morning stiffness in hand joints and were excluded if positive. Serum anti-CCP antibody positivity was assessed by chemiluminescent microparticle immunoassay.

Results: Out of 85 patients, seropositivity was detected in 71 patients with male:female =1:3.72. 22 of 105 (20.9%) AFDR and 13 of the 105 controls were found seropositive for anti-CCP. Although a greater prevalence was found in AFDR, it was not significant statistically (P = 0.1378). The serum titer of anti-CCP was found to be higher in RA patients in comparison to AFDR.

Conclusion: The higher seroprevalence of anti-CCP in AFDR may have high risk of development of RA in the near future. Therefore, long-term follow-up is required for anti-CCP-positive AFDRs for early detection of development of RA.


  OPC0125: Comparative study of demographic and clinical features of patients of limited scleroderma, mixed connective tissue disease, and undifferentiated connective tissue disease: Single center Top


Pandya S, Solanki R, Parikh T; Vedanta Institute of Medical Sciences, Ahmedabad, Gujarat, India

Objective: The objective was to study demographic and clinical differences between patients of limited systemic sclerosis (SSc), mixed connective tissue disease (MCTD), and undifferentiated connective tissue disease (UCTD).

Materials and Methods: This was a cross-sectional study. The study period was January 2013 to July 2018. For limited scleroderma, the American College of Rheumatology criteria for MCTD patients, the Alarcon-Segovia criteria, while for UCTD, the revised 2008 criteria by Mosca et al. was used. Appropriate statistical tests were used to compare the features.

Results: See attached table.

Conclusions: Limited scleroderma was most common. These patients were late in the disease course compared to other two. Skin, gastrointestinal, and interstitial lung disease were more in SSc compared to other groups. More patients of MCTD had musculoskeletal manifestations.


  OPC0124: Poncet's disease - Elbow joint tuberculosis presenting with seropositive polyarthritis: A rare entity Top


Swapnil Khose, Jayashree Kharaje; ESIC Model Hospital cum ODC and PGIMSR, Mumbai, Maharashtra, India

Background: Poncet's disease is an aseptic form of arthritis in patients with active tuberculosis. Elbow joint tuberculosis is a rare disease which accounts for 1%–3% of all cases of osteoarticular tuberculosis. As seropositivity for rheumatoid arthritis has been reported in Poncet's disease as well as in tuberculosis, it is rather uncommon. This presentation shows the same rare phenomenon.

Case Report: A 62-year-old female a known case of hypertension presented with a complaint of symmetrical polyarthritis involving wrist, metacarpophalangeal (MCP), proximal interphalangeal (PIP), elbow, and knee for the past 1.5 years. Joint pain was associated with swelling and early morning stiffness. Elbow joint pain and swelling increased further over the last 2 months. There was no history of fever and previous history of pulmonary or extrapulmonary tuberculosis.

Blood investigations revealed erythrocyte sedimentation rate 11 mm, C-reactive protein-positive (6 μg/dl), and rheumatoid factor-positive (8 IU/ml); ultrasonography wrist suggested inflammatory arthritis. Magnetic resonance imaging right elbow revealed joint subluxation, moderate effusion with synovial thickening, periarticular erosions, marrow edema, and septated collection. Elbow joint effusion aspiration was pale yellow, hazy with white blood cell count 4300 cells/cmm, proteins 3.3 g, ADA 75.70 (highly raised), and Mycobacterium tuberculosis (MTB) by GENEXPERT reported as “MTB detected” with NO resistance to rifampicin resistance, and the diagnosis of Poncet's disease was made.

Intervention: She started on CAT 1 antituberculous therapy drugs with directly observed treatment's center.

Outcome: At 2 months of follow-up, joint pain and swelling of wrist, MCP, PIP resolved completely and significant reduction was observed in elbow joint pain and swelling.

Message: It shows the value of Poncet's disease in a patient presenting with seropositive polyarthritis. We can avoid long-term disease-modifying antirheumatic treatment being vigilant.


  OC0039: An uncommon overlap of two common rheumatological disorders: A report of 2 cases Top


Dharmagat Bhattarai, Sandesh Guleria, Ankur Jindal, Rakesh Pilania, Pandiarajan Vignesh, Deepti Suri, Amit Rawat, Anju Gupta, Surjit Singh; Postgraduate Institute of Medical Education and Research, Chandigarh, India

Case Reports: Case 1 - A 5-year-old male child presented with fever, malar rash, oral ulcers, and body aches for 2 months. On examination, he had irritability, malar rash, generalized maculopapular rash, oral ulcers, conjunctival injection, cervical adenopathy, and hepatomegaly. Laboratory investigations showed anemia, leukopenia, transaminitis, elevated erythrocyte sedimentation rate (ESR), high C-reactive protein (CRP), hypocomplementemia, positive antinuclear antibody (ANA) (4+ diffuse pattern), and high anti-dsDNA antibody titer. Diagnosis of systemic lupus erythematosus (SLE) was made, and he was initiated on oral prednisolone and hydroxychloroquine. One week later, he was brought with persistent irritability and periungual peeling of skin in fingers and toes. Laboratory investigations showed anemia, thrombocytosis, persistently elevated ESR, and high CRP. Two-dimensional (2D) echocardiography revealed normal coronary artery “Z” scores. A diagnosis of concomitant Kawasaki disease (KD) was considered. He was treated with intravenous immunoglobulin (IVIG). Case 2 - An 8 year-old female child presented with hair loss, intermittent fever, cough, and abdominal pain. On examination, she had tachypnea, tachycardia with feeble pulses, and hypotension. She also had pallor, diffuse alopecia with predominant frontal involvement, generalized firm lymphadenopathy, hepatomegaly, crepitations, and gallop rhythm on cardiac auscultation.

Laboratory investigations revealed severe anemia, lymphocytopenia, elevated ESR and CRP, and high serum pro-BNP levels. 2D echocardiography showed ejection fraction of 35%. She had hypocomplementemia, positive direct Coomb's test; positive ANA test (4+ diffuse pattern with rim enhancement), and high anti-dsDNA antibody titer.

A diagnosis of SLE with myocarditis, cardiogenic shock, and hematological involvement was made. She was treated with O2 inhalation, antibiotics and inotropes, IVIG, and pulse methylprednisolone. She has initiated on pulse intravenous cyclophosphamide for lupus myocarditis. On day 5 of hospital stay, she developed redness of tongue and lips, chromonychia, periungual, perianal peeling, and thrombocytosis. Possibility of concomitant KD was considered. Serial 2D-echocardiography showed improvement in cardiac contractility without any coronary artery abnormalities.


  OPC0122: NA Top


John Mathew, Rohit N Benjamin; Christian Medical College, Vellore, Tamil Nadu, India

Systemic lupus erythematosus (SLE) can present with a myriad of neurological manifestations. Neuromyelitis optica spectrum disorders (NMOSDs) is a relatively new diagnosis attributed to patients with SLE and demyelinating presentations. It presents as a demyelinating disease and is characterized by the presence of aquaporin antibody. Here, we present two cases of patients with aquaporin antibody-positive and antibody-negative neuromyelitis optica (NMO) who fulfill the diagnostic criteria.

The first patient was a 26-year-old female with SLE and polyarthritis on follow-up who developed two transient episodes of left hemiparesis over the past month which had partially resolved and she presented to us with abnormal spasm-like movements of the right upper limb. The magnetic resonance imaging done showed features of vasculitis with infarcts and long-segment hyperintensity from the craniovertebral junction to the lumbar cord – suggestive of longitudinally extensive transverse myelitis. Her visual evoked potential was suggestive of bilateral optic nerve dysfunction and the antiaquaporin 4 antibody was positive. Hence, a diagnosis with SLE, central nervous system vasculitis with APLA syndrome, and NMO was made. The second patient was a 19-year-old female who presented with a chronic fever, arthritis, alopecia, recent-onset drowsiness, and persistent vomiting. Under evaluation, she developed a paraparesis of the lower limb with a left lower motor neuron facial nerve and lateral rectus palsy, suggestive of a Foville syndrome. The imaging showed long tact hyperintensities along the floor of the fourth ventricle and long-segment cord hyperintensity extending from thoracic level to conus. Aquaporin was negative, but she fulfilled both clinical criteria and radiological criteria. Both these patients were treated with aggressive immunosuppression and their overall condition has improved significantly. These cases give us an insight into the presentations and management of SLE with NMOSD.


  OPC0123: Follicular B-cell lymphoma presenting with polymyositis Top


S Suvarna Shilpa, V Sarath Chandra Mouli, Abitha Aliyar, Srisaila Datta; Department of Rheumatology, Krishna Institute of Medical Sciences Hospital, Hyderabad, Telangana, India

Case Report: A 48-year-old man presented with complaints of significant loss of weight (8 kg) over the last 2 months, progressive proximal muscle weakness of all limbs for 1 month, and diffuse myalgias, hoarseness of voice, difficulty in swallowing for 15 days. On examination, there were nontender, firm enlarged lymph nodes in the left axilla and bilateral inguinal regions; there was moderate nontender splenomegaly. Proximal muscle power was 3/5 in all limbs. Laboratory evaluation revealed marked lymphocytic leukocytosis (total white blood cells count - 49,500/mm3, DC-L - 74%, N - 22%, M - 4%, E - 0%) and elevated muscle enzymes (creatine phosphokinase - 1570 IU/L, aspartate aminotransferase - 100 IU/L, alanine aminotransferase - 135 IU/L). Hemoglobin and platelet counts were normal. Peripheral smear showed many mature lymphocytes and few smudge cells. Electromyography showed myopathic potentials. Bone marrow biopsy showed features suggestive of chronic myeloid leukemia. Antinuclear antibody immunofluorescence was negative, myositis antibody panel (Mi 2, ku, OM-Scl, JO-1, SRP, PL-7, PL-12, EJ, OJ, RO-52) was negative, and tumor markers (carcinoembryonic antigen, prostate-specific antigen, alpha-fetoprotein) were normal. Fluorodeoxyglucose positron emission tomography showed metabolically active bilateral axillary, pelvic lymphadenopathy, and large lobulated mesenteric, periportal, and retroperitoneal lymph nodal mass. Computed tomography-guided biopsy from retroperitoneal lymph nodal mass done, histopathology and immunohistochemistry, and flow cytometry confirmed the diagnosis of low-grade follicular type of B-cell lymphoma. He was treated with rituximab, cyclophosphamide, adriamycin, vincristine, and prednisolone chemotherapy. He improved with the therapy.

Discussion: This is a rare case of follicular B-cell lymphoma with myositis as its presenting manifestation. Usual myositis-specific and myositis-associated antibodies were negative in his serum. Treatment of underlying malignancy completely resolved his symptoms.

Conclusion: This is a rare case of follicular B-cell lymphoma presenting with polymyositis. Appropriate screening for malignancy is recommended in all cases of idiopathic inflammatory myopathies.

References

  1. De Weirdt J, et al. Cancer Associated Myositis. Faculty of Medicine and Health Sciences; 2012.
  2. Marie I, Guillevin L, Menard JF, Hatron PY, Cherin P, Amoura Z, et al. Hematological malignancy associated with polymyositis and dermatomyositis. Autoimmun Rev 2012;11:615-20.



  PC0042: A report on inflammatory muscle diseases seen at a private clinic setup Top


Alpana Parmar; Surat

Background: Data on inflammatory muscle disease are scarce from Gujarat.

Objectives: The objective was to study the demographic, clinical, laboratory profile, treatment given and outcomes of inflammatory muscle diseases.

Methods: This was a retrospective analysis from clinic database.

Results: Among 25 patients, 21 were females and 4 male (female:male ratio, 5:1). The age of presentation ranged from 3 to 85 years (mean 50.86 years). The duration of illness ranged from 1 to 24 months (mean 7.6 months). Polymyositis was diagnosed in 14 (56%) and dermatomyositis in 11 (44%) (2 juvenile) patients. In dermatomyositis, rash preceded muscle weakness by a mean 13.8 months in 5 (45.5%) patients. Constitutional symptoms, hair fall, and proximal muscle weakness of upper and lower limb were noted in all. In addition, 13 (52%) had truncal weakness, 6 (24%) had bulbar weakness, and 3 (12%) had respiratory weakness also. Cough and dyspnea were seen in 12 (48%). Inflammatory arthritis at a later phase was reported in three patients of dermatomyositis. Interstitial lung disease (ILD) was seen in 13 (52%) and pulmonary hypertension in three (all had mixed connective tissue disease) patients. Mean hemoglobin was 8.56 g%; erythrocyte sedimentation rate was 62.8/1st h by Westergren. Serum creatine phosphokinase was normal in 9 (36%) patients. Magnetic resonance imaging screening of pelvis and thigh muscles was done in 11 (44%), electromyography in 14 (56%), and muscle biopsy in 3 (12%). Antibody positivity seen in these patients were anti-Ro52 (10; 40%), anti-U1RNP (6; 24%), anti-MI2 (3, 12%), and pmscl100 and scl70 each (2, 8%). For induction, methotrexate was used in 15 (60%), azathioprine was used in 2 (8%), cyclophosphamide was used in 3 (12%, lung dominant disease), and intravenous immunoglobulin was additionally used in 3 (12%). Rituximab was used in 3 (12%) nonresponders of polymyositis. For maintenance, methotrexate was used in 16 (64%), azathioprine was used in 5 (20%) with low-dose steroids. 3 (12%) patients died.

Conclusions: Constitutional symptoms were common in inflammatory muscle disease. ILD was seen in 52%. Respiratory muscle weakness was used in 12%. Death occurred in 12%. Mean follow-up was 11.3 months.


  PC0043: Erdheim Chester Disease Top


Vikas Sharma, Sakshi Mittal, Shankar Naidu, Ritambhra Nada, Manphool Singhal, Varun Dhir, Rajinder, Sanjay Jain, Aman Sharma; PGIMER, Chandigarh, India

Background: Erdheim–Chester disease (ECD) is a rare form of non-Langerhans' cell histiocytosis originally described as “lipid granulomatosis” in 1930 by Jakob Erdheim and William Chester. It is a rare disease with nearly 600 cases described in literature. Its presentation may vary from an indolent disease to a life-threatening illness. In view of rarity, diagnosis requires high level of suspicion.

Case Report: A 46-year-old female presented to us with generalized fatigue for 6 years and osmotic symptoms for the last 4 years. She also had high-grade fever and exertional dyspnea for 3 years. She had pericardial effusion for which workup including fluid cytology, cultures, tuberculosis-polymerase chain reaction, and pericardial biopsy was inconclusive. She was empirically given antituberculous treatment without any relief. Pericardial window was created outside but without much improvement, and she developed features of congestive cardiac failure at the time of presentation to us. Her computed tomography (CT) scan showed thickened pericardium, mass encasing aorta, and right atrial mass besides pericardial effusion. Hence, a possibility of ECD was considered and review of the pericardial biopsy was done which confirmed the diagnosis based upon CD68(+), CD1a(−) histiocytes. Bone scan showed increased tracer uptake in periarticular region of the femurs and tibiae. Fluorodeoxyglucose avid pericardial thickening encasing heart and great vessels was evident on positron emission tomography (PET). She was initially treated with PEG-interferon α. However, she developed hypersensitivity reaction during the first dose. She was switched to “vemurafenib-the BAFF inhibitor,” only FDA-approved therapeutic agent. She became afebrile; osmotic symptoms and dyspnea got relieved. Repeat CT and magnetic resonance imaging showed marked regression of pericardial mass.

Discussion: V600E BRAF mutation has been described in more than half of patients. Interferon-α is most extensively studied agent in treatment and serves as the first line of treatment. Cladribine (2CDA), anakinra, and vemurafenib are currently advocated as the promising second-line treatments.


  OPC0207: Long-term outcome of juvenile idiopathic arthritis from a tertiary care center in South India Top


Sreedevi S, Balameena S, Ramesh R, Mythili S, Karthikeyan, Sujatha N; Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Juvenile idiopathic arthritis (JIA) is the most common chronic arthritis in children worldwide. It is a heterogeneous inflammatory disease and defined as arthritis persisting 6 weeks or longer with onset before the age of 16 years with no identifiable etiology.

Objectives: Our aim was to assess the outcome of various subtypes of JIA using juvenile arthritis disease activity scoring (JADAS) index as well as to weigh the morbidity as assessed by JADI caused due to the disease per se and treatment related.

Methods: Sixty-four children who satisfied the ILAR classification were studied during the period of August 2011 to August 2018. A retrospective analysis was done with their clinical distribution, JADAS, juvenile arthritis damage index-articular and extra-articular scores (JADI A and E).

Results: Sixty-four children (mean age at onset 9.7 ± 2.5 years) with JIA were studied during this period with a median duration of disease of 6.19 ± 2.1 years. 64% were males. The most common subgroup was polyarticular JIA (34%) followed by enthesitis-related arthritis (31.3%), systemic JIA (sJIA, 23.2.2%), and oligoarthritis (12.5%). One patient in JIA/early rheumatoid arthritis evolved into psoriatic arthritis with skin lesions. Weights and heights were less than the fifth centile in 20% and 17%, respectively, being most affected in sJIA. Mean JADAS was highest in sJIA (8.33) in spite of 95% children being on biologics. Mean JADI A (3) and JADI E (1.07) was highest in sJIA. Macrophage activation syndrome was diagnosed in 1.5 % of sJIA. Positive correlation was found between disease duration, steroid use, and JADAS and JADI - A and E scores in all subtypes. Rheumatoid factor positivity correlated well with JADAS scores in polyarticular JIA. Thirty-three percent were in clinical remission at the end of 2 years on antirheumatic medications.

Conclusion: sJIA had the most morbidity among the subtypes of JIA. JADAS and JADI were most severely affected in sJIA.


  OPC0208: Tofacitinib, an oral Janus kinase inhibitor, in the treatment of Indian patients with rheumatoid arthritis: Pooled efficacy and safety data from Phase 3 and long-term extension studies over 7 years Top


Chopra A, Shobha V1, Chandrashekara S2, Veeravalli SCM3, Sharma R4, Rao UR5, Pandya S6, Wagh S7, Kadel JK8, Thorat AV9, Raut S9, Santos Estrella P10, Kwok K11, Wouters A11; Arthritis Research and Care Foundation, Centre for Rheumatic Diseases, Pune, 1St. Johns Medical College Hospital, 2Chanre Rheumatology & Immunology Centre& Research, Bangalore, 3Krishna Institute of Medical Sciences, Hyderabad, 4Swastik Rheumatology Clinic, Ahmedabad, 5Sri Deepti Rheumatology Centre, Hyderabad, 6Rheumatic Diseases Clinic, Vedant Institute of Medical Science, Ahmedabad, 7Jehangir Clinical Development Centre, 8Mahavir Hospital and Research Centre, 9Pfizer Inc, Mumbai, India, 10PfizerInc, Makati City, Philippines, 11Pfizer Inc, New York, NY, USA

Background: Tofacitinib is an oral Janus kinase inhibitor for the treatment of rheumatoid arthritis.

Objectives: Post hoc analysis characterizing tofacitinib efficacy/safety in Indian patients versus rest of the world (ROW) was carried out.

Methods: Efficacy data were pooled from disease-modifying antirheumatic drug-inadequate response patients: Indian data from Phase (P) 3 studies ORAL Solo and ORAL Scan; ROW (excluding India) included aforementioned studies, P3 studies ORAL Step, ORAL Sync and ORAL Standard. Safety also included ORAL Start (methotrexate naïve) and the long-term extension (LTE) studies ORAL Sequel (data cutoff March 2017) and A3921041 (Japanese study). Efficacy of tofacitinib 5 and 10 mg twice daily (BID) versus placebo was assessed by ACR20/50/70 response, disease activity score 28-4 (erythrocyte sedimentation rate) ≤2.6 (remission) and ≤3.2 (low disease activity) rates, and Health Assessment Questionnaire Disability Index change from baseline. Safety included adverse events (AEs), serious AEs (SAEs), laboratory abnormalities, mortality, and incidence rates (IRs; patients with events/100 patient-years) for AEs of special interest.

Results: Analyses included 197 and 3879 patients from India and ROW, respectively. Generally, efficacy numerically improved with tofacitinib versus placebo in both populations [Table 1]. More ROW than Indian patients experienced AEs/SAEs, discontinuation rates were similar. Although safety profiles in Indian/ROW populations were generally similar, tuberculosis rates were higher in Indian patients (IR: 1.21; P3, n = 3, LTE, n = 4; patients received tofacitinib 10 mg BID at onset) versus ROW patients [IR: 0.17; [Table 1]] and an IR of 0.75 (95% confidence interval 0.49–1.15) in tofacitinib-treated patients from high-risk countries (per the WHO) including India was reported (Winthrop et al. Ann Rheum Dis. 2016;75:1133-1138); background tuberculosis IR in India of 0.2 (Global tuberculosis report 2017, http://www.who.int/tb/publications/global_report/en).



Conclusions: Efficacy of tofacitinib was generally similar, and no new safety risks were identified in Indian versus ROW patients. Results should be interpreted with caution due to limited sample size and patient exposure in the Indian population.


  OPC0127: Occult hepatitis B in rheumatoid arthritis patients undergoing immunosuppressive therapy Top


Sweety Kakoti, Safeer Moideen, A. K. Das, Sanjeeb Kakati; Department of Medicine, Assam Medical College and Hospital, Dibrugarh, Assam, India

Background: Occult hepatitis B infection (OBI) is defined as the existence of low-level hepatitis B virus (HBV) DNA in the serum (<200 IU/mL), lymphatic system, or hepatic tissue in patients with serological markers of previous infection (anti-HBc and/or anti-HBs positive) and absence of serum hepatitis B surface antigen. It is an emerging concern in patients of rheumatoid arthritis when treated with disease-modifying antirheumatic drugs, biological agents, or monoclonal antibodies. Antiviral prophylaxis is necessary in these cases.

Objective: The objective was to find out the proportion of OBI in patients with rheumatoid arthritis undergoing immunosuppressive therapy.

Methods: A hospital-based observational study was carried out in the Department of Medicine, Assam Medical College and Hospital, Dibrugarh, from July 1, 2016, to June 30, 2017. A total of 107 rheumatoid arthritis patients were taken.

Results: Out of 107 patients, 32.71% belonged to age group 40–49 years, 0.93% in >70 group. Male:female ratio was 1:3.28. 57.94% patients had a disease duration of 1–5 years, 20.56% had >5–10 years, 12.14% <1 year, and 9.34% of >10 years. A total od 102 patients (95.33%) presented with symmetrical and 5 (4.67%) with asymmetrical joint involvement. 60.74% patients had 4–10, 28.03% had >10, 15.88% had 1–3 small joint involvement while 5.60% had 2–10 and 0.93% had one large joint involvement. 55.14% had moderate disease activity, 29.90% high disease activity, 12.14% low disease activity, and 2.80% in remission. There was history of hepatitis in 7.47%, past surgical intervention in 7.47%, and blood transfusion in 8.41%, and 2% were vaccinated against HBV. 55% patients received methotrexate; 39.2% received methotrexate plus steroids; and 0.98% received rituximab. Reactivation of occult hepatitis B was not found in any of the patients.

Conclusion: The study did not find any patient with reactivation of occult hepatitis B.


  OPC0218: Does Anti-CCP antibody influence the long term outcome of Rheumatoid Arthritis (>10 years)? Top


Wasim Kazi, Dhaval Tanna, Dhiren Raval, Durgarao Yedavalli, Gayatri Ekbote, Shruti Bajad, Rajiva Gupta; Medanta - The Medicity, Gurgaon, Haryana, India

Background: Cyclic citrullinated peptide (CCP) has been considered as a bad prognostic marker in rheumatoid arthritis (RA). Literature shows that patients having CCP-positive RA develop erosions, deformities, and extra-articular complications earlier than those having CCP-negative RA. We hypothesized that long-term outcome of RA is not dependent on CCP positivity.

Methods: We included all 94 patients of RA, who presented consecutively to outpatient and inpatient department of Medanta Hospital, from August 2017 to April 2018. The disease characteristics including rheumatoid factor and CCP positivity, comorbidities, and articular and extra-articular complications diagnosed on the basis of clinical or laboratory data was noted. CCP status was available for 94 patients. Only patients with disease duration of more than 10 years who were on disease-modifying antirheumatic drugs were selected. The study was approved by the Institutional Research Board. Ethics Committee clearance was also taken (Reference No. MICR-789/2017).

Results: Out of the 94 patients, CCP was positive in 69 patients (73.4%) and it was negative in 25 patients (26.6%). Mean disease duration of the study cohort was 16.6 years. The disease characteristics and outcome in relation to the CCP positivity versus CCP negativity were as follows as shown in the following tables.

Conclusion: There was no difference in CCP-positive and CCP-negative patients with regard to the development of articular or extra-articular complications in RA patients with a disease duration >10 years. We thus conclude that although CCP positivity is associated with earlier development of articular and extra-articular complications, it is not a predictor of severity in established cases of RA.


  PC0040: CIMT as a marker of subclinical atherosclerosis in Ankylosing spondylitis patients – A Prospective study Top


Rajavel Mohan, Balameena Selvakumar, Ramesh, Anuja, Anusha, Raghavendra; Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Ankylosing spondylitis (AS) is a chronic inflammatory disease, which attributes to accelerated atherosclerosis leading to an increased risk of myocardial infarction and stroke. In this study, carotid intima-media thickness (CIMT) is used as a marker to assess subclinical atherosclerosis in AS patients.

Objectives: The objective was to assess whether subclinical atherosclerosis is increased in primary AS patients when compared to healthy controls by measuring CIMT and its correlation with inflammatory markers and disease activity indices.

Methods: It is a prospective case–control study, conducted at a tertiary care center over a period of 6 months from February 2018 to July 2018. A total of 46 male patients fulfilling 2009 ASAS criteria for axial spondyloarthritis, who were admitted in the rheumatology ward of RGGGH were included as cases in this study. The controls were same number of healthy individuals matched for age and sex was recruited from the staff of hospital. Exclusion criteria were diabetes mellitus, chronic kidney disease, coronary artery disease (CAD), hypertension, family history of premature CAD, tobacco chewing, and smoking. Detailed history and examination, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), thyroid function test, lipid profile, Bath AS Activity Disease Activity Index (BASDAI), Bath AS Functional Index (BASFI), Bath AS Metrology Index (BASMI), and carotid intima-media thickness (CIMT) measurements using B-mode ultrasound were recorded. Data were analyzed using SPSS version 19.

Results: Mean age of the study groups was 32.43 ± 8.00 years. Mean CIMT (mm) in cases was 0.64 ± 0.16 which was significantly increased when compared to control groups (0.52 ± 0.08; P < 0.001). CIMT in the case groups positively correlated with duration of disease (r = +0.588; P < 0.01) and BASMI (r = +0.364; P < 0.05); no significant correlation was observed between CIMT and age at onset of disease, BASDAI, BASFI, ESR, CRP, triglycerides, and total cholesterol levels (P > 0.05).

Conclusions: AS patients had a significantly increased CIMT than control groups and it is positively correlated with chronicity of disease, which can be used as a marker of subclinical atherosclerosis to predict future cardiovascular events.


  OPC0129: Arterial Behcet's disease: an experience from a tertiary care center of North India Top


Arghya Chattopadhyay, C Bharat1, Aadhaar Dhooria, Nupoor Acharya, Saket Jha, Sakshi Mittal, Shankar Naidu, M Valliappan1, Manphool Singhal2, Ajay Savlania3, Rajesh Vijayvergia4, Sanjay Jain, Aman Sharma; Departments of Internal Medicine, Clinical Immunology and Rheumatology Services, 1Pulmonology and Critical Care, 2Radio Diagnosis, 3General Surgery and 4Cardiology, PGIMER, Chandigarh, India

We report seven patients with Bechet's disease who were diagnosed to have arterial involvement (2013–2018). The diagnosis of Behcet's is based on revised international criteria for Behcet's disease (2008). Mean age of the patients at the time of presentation was 32 ± 8 years, with a male:female ratio of 6:1. The arterial involvement was in the form of a pulmonary aneurysm in three patients; an abdominal aortic aneurysm in two; and thoracic aortic aneurysm, left internal iliac artery aneurysm, and right dorsalis pedis artery aneurysm in one patient each. One patient had both pulmonary and thoracic aortic arterial aneurysms. Mean disease duration before the development of an aneurysm was 38 ± 25 months. The oral and genital ulcer was present in all, and three of them had superficial thrombophlebitis and ocular involvement. Pathergy test and human leukocyte antigen B51 were positive in two patients. The presenting features were abdominal pain in four, hemoptysis in three, and pain in the foot due to aneurysm in the left dorsalis pedis artery in one patient. Six patients received immunosuppression (steroids and cyclophosphamide), while one died immediately after coming to the emergency department due to rupture of iliac aneurysm. Surgical repair of abdominal aneurysm was done in two patients following immunosuppressive therapy. One patient who did not respond to cyclophosphamide responded to infliximab. Three patients expired: two died due to massive hemoptysis and the third was the one described above.

Discussion: Arterial involvement in Behcet's disease is rare and almost exclusively found in young males. Although pulmonary artery aneurysm is classical of Behcet's disease, abdominal aortic aneurysm is also common. Arterial involvement in Bechet's syndrome is a treatable rare but potentially fatal complication. For aortic and peripheral artery involvement, treatment with corticosteroid and cyclophosphamide is necessary before surgical intervention.

Conclusion: Arterial Behcet's disease is associated with very high complications and mortality. Early detection and aggressive treatment are essential for good outcomes.


  OPC0131: Systemic sclerosis with antineutrophil cytoplasmic antibody-associated crescentic glomerulonephritis: a rare association Top


Arghya Chattopadhyay, Shankar Naidu, Varun Dhir, Shefali K Sharma, Ritambara Nada1, Sanjay Jain, Aman Sharma; Departments of Internal Medicine, Clinical Immunology and Rheumatology services and 1Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Case Report: A 53-year-old woman was diagnosed to have diffuse systemic sclerosis (SSc) with interstitial lung disease for 15 years. She developed acute limb ischemia involving the left hand and foot and progressive normotensive renal failure. On evaluation, there was anemia, neutrophilic leukocytosis, thrombocytosis along with erythrocyte sedimentation rate of 65 mm, and C-reactive protein 15.5 mg/L (<6 mg/L) with normal serum procalcitonin. Creatinine increased from 0.6 to 2.4 mg/dl during the hospital stay with proteinuria of 1.46 g/day. Serum anti-myeloperoxidase (MPO) was 47.2 U/ml (normal <20 U/ml). Renal biopsy evaluated on light microscopy and immunofluorescence showed the presence of p-antineutrophil cytoplasmic antibody (p-ANCA)-positive pauci-immune crescentic glomerulonephritis (Berden classification-mixed type) with evidence of occasional small interstitial epithelioid cell granuloma [Figure 1]. There was no evidence of vasculitis. Electron microscopy confirmed the absence of the immune complex type of deposits. She was started on oral prednisolone (1 mg/kg) and injection rituximab 1 g on days 0 and 15. After 12 weeks of follow-up, her creatinine reduced to 1.1 mg/dl and other disease manifestations were stable.



Discussion: Prevalence of ANCA positivity in SSc is usually between 0% and 6%. Opinion regarding outcome varies from no difference to organ or life-threatening. Both typical and atypical ANCA are reported in association with SSc, of which MPO is the most frequent. ANCA positivity in SSc should be considered as ominous “red flag sign” rather than just coincidence. Reported treatment for SSc-(ANCA)-associated vasculitis overlap is a high dose of corticosteroid and immunosuppressive such as cyclophosphamide or rituximab.

Conclusion: ANCA-associated vasculitis may be a rare cause of renal failure in SSc which should be considered in patients with normotensive renal failure and increased markers of inflammation along with ANCA positivity.


  OPC0212: Profile of adverse events in patients receiving rituximab: A prospective cohort study at a tertiary care center Top


Ramakant Singh, Vivek Vasdev, Arun Hegde, Ashwani Kumar, K Kishore, S Bhatt; Army Hospital Research and Referral, New Delhi, India

Background: Rituximab, like other biological drugs, increases the risk of infections and other adverse events in patients. The risk of infections is rather high in India due to the high incidence and prevalence of infective diseases. The data depicting the adverse effect profile in Indian population are sparse.

Objectives: The objective was to study the profile of adverse effects in patients receiving rituximab for rheumatological diseases.

Methods: Patients who received rituximab for various rheumatological illnesses at a tertiary care center in the year 2017 were recruited after consent and observed during the administration of the drug and afterward for a minimum of 6 months for appearance of long-term adverse events. All patients were screened for tuberculosis and other viral infections as per the existing guidelines.

Results: A total of 37 patients were observed for a mean period of 10.1 months. Four patients had a past history of tuberculosis. Of the total 37 patients observed, three developed infusion reactions, ten developed self-limiting upper respiratory tract infections, three developed infections requiring antibiotics as outpatients (one urinary tract infection and two furunculosis), two patients were treated as inpatients for lower respiratory tract infections, two were detected to be hepatitis B surface antigen positive, three developed herpes zoster, and one had reactivation of tuberculosis. No incidences of malignancy or John Cunningham virus infection were noted. No fatalities occurred.

Conclusions: Rituximab appears to be a safe biological. The complications are few and treatable. There were no fatalities.


  OC0034: Still too hot!!! Are clinical indices sufficient to assess remission in rheumatoid arthritis? Top


Anuja Rajan, Selvakumar Balameena, R. Ramesh, S. Mythili, M. Rajavel, Sreedevi S.; Institute of Rheumatology, Madras Medical College and RGGGH, Chennai, Tamil Nadu, India

Background: Remission is the primary therapeutic goal in patients with rheumatoid arthritis (RA). Though remission can be established using instruments such as Disease Activity Score (DAS) 28 and Simplified Disease Activity Index , clinical assessment alone may underestimate the presence of persistent synovitis. Over the past decade, ultrasound has shown value in the evaluation of patients with RA.

Objective: The objective was to investigate the presence of synovitis using B-mode ultrasound in patients with RA in remission using gray-scale (GS) abnormalities signifying synovial hypercellularity and power Doppler (PD) which assesses vascularity.

Methods: A prospective study was carried out between March 2018 and August 2018 among patients attending the review clinic at the Institute of Rheumatology (IOR), Madras Medical College, Chennai, Tamil Nadu, India. Forty-four consecutive cases of RA in remission were selected. Remission was defined as DAS 28 value <2.6. The minimum period of remission for inclusion was 3 months. The B-mode ultrasound scan was done at IOR using Mylab 6 Esaote machine with a multifrequency linear array transducer. The joints scanned were wrist, 2–5 metacarpophalangeal joints, ankle, and 2–5 metatarsophalangeal joints on both sides. Scoring for GS and PD was done semi-quantitatively using European League Against Rheumatism OMERACT ultrasound taskforce 2017 scoring system. The severity of synovitis was assessed using combined score defined in the same.

Results: The mean age of patients in this study group was 49.1 years; 59% of patients were in remission for >6 months. Synovial hypertrophy was detected in 63% of patients in remission. Synovial PD signal was noted in 45% of patients with remission. Among patients with synovitis, 50% had Grade 1 synovitis and 42% had Grade 2 synovitis. No correlation was found between seropositivity or duration of disease with the presence of synovitis.

Conclusion: Ultrasound assessment of joints is superior to clinical assessment in detecting patients with persistent synovitis and should be included in the regular assessment of patients with RA.


  OPC0132: To assess the ultrasonography abnormalities in inflammatory myositis Top


Nayan Patel, Narayanan R, Phani Kumar Devarasetty, Sravan Kumar Appani, Rajasekhar L; Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background: Imaging of muscles is useful in detecting myositis. Magnetic resonance imaging of muscle is not always feasible due to cost and availability. Ultrasound may be useful in identifying inflamed muscles (IMs).

Objectives: The objectives were (1) To assess ultrasonography (USG) findings in patients with IM and (2) to correlate USG findings with clinical muscle weakness and muscle enzymes.

Methods: Consecutive patients with connective-tissue disease, significant symmetric proximal muscle weakness, and raised creatine phosphokinase (CPK) (except in dermatomyositis [DM]) satisfying the classification criteria of DM, lupus, scleroderma, or mixed connective-tissue disease, attending rheumatology services between August 2017 and June 2018, were included. Patients with hypothyroidism, recent thigh trauma, or who underwent electromyography and/or muscle biopsy were excluded. Demographic data, disease duration, muscle power (Manual Muscle Testing-8 [MMT8]), CPK, lactate dehydrogenase (LDH), aspartate transaminase (SGOT), and alanine transaminase (SGPT) were noted. USG of middle of the bilateral vastus lateralis was done using 5–12 MHz linear array transducer by single musculoskeletal sonologist. Increasing muscle echogenicity was graded as 1–4 (gray scale [GS]) and vascularity 0–4 (power Doppler [PD]). USG parameters (Grade 2–4 GS and Grade 1–4 PD) were correlated with MMT8 score and muscle enzymes. The study was approved by the institute's Ethics Committee.

Results: Thirty-one cases (28 females) were recruited. Patient clinical characteristics are mentioned in [Table 1]. GS was normal in six patients and abnormal in one or both sides in the rest. PD was normal in one and the rest had increased vascularity on one or both sides. Unilateral increased echogenicity was seen in one and vascularity in four patients. LDH showed a modest correlation with the presence of PD abnormalities (rs = 0.5, P = 0.01).



Conclusions: Clinical muscle weakness and muscle enzymes did not show correlation with USG findings. Unilateral abnormalities on USG were seen in patients with symmetrical weakness. USG may not be sensitive for evaluating muscular abnormalities in IM.


  OPC0210: Non-Hodgkin's lymphoma: The great masquerader for systemic lupus erythematosus Top


Prashant Bafna, Urmila Dhakad, Siddharth Kumar Das, Akhil Pawan Goel; Department of Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India

A 36-year-old male presented with fever for 3 months and bilateral 6th and 7th cranial nerve palsy for a month. Examination revealed nonscarring alopecia, generalized lymphadenopathy, and hepatomegaly. Complete blood count showed hemoglobin of 11 g/dl, total leukocyte count of 4600, and platelet count of 45,000. Erythrocyte sedimentation rate was 44 mm/h and was negative for HIV. Peripheral smear did not show any abnormal cells and lactate dehydrogenase was 1160 U/L. Lymph node and bone marrow biopsy was normal. Workup for tuberculosis, sarcoidosis, brucellosis, Borrelia, and syphilis was negative. Cerebrospinal fluid study and magnetic resonance imaging brain were noncontributory. Given the negative workup, a probability of autoimmune process was kept. Antinuclear antibody was 3+ fine speckled (1:100 dilution) and extractable nuclear antigen positive for nucleosome and dsDNA (negative by enzyme-linked immunosorbent assay) with very low C3 and C4. His immunoglobulin M anti-cardiolipin antibody at admission and after 3 months was >100 IU/ml. He was diagnosed as systemic lupus erythematosus (SLE) with secondary antiphospholipid antibody (APLA) and started on hydroxychloroquine, 1 mg/kg of prednisolone, and monthly intravenous cyclophosphamide with anticoagulation. The patient showed significant neurological improvement. Subsequently, he had recurrent viral infections. Later, he presented with features suggestive of macrophage activation syndrome, started on dexamethasone and cyclosporine. However, the patient's condition was deteriorating, developed worsening pancytopenia and multiorgan dysfunction syndrome, and was succumbed to death. Repeat bone marrow biopsy reported as a case of peripheral T-cell lymphoma.

Discussion: A study by Bairey et al. showed APLA positivity in 41% of non-Hodgkin's lymphoma (NHL) at diagnosis and correlated with shortened survival. The probable mechanism is that autoantibodies are produced in response to release of tumor-associated antigens or as monoclonal immunoglobulins that possess APLA activity. We describe a patient who was diagnosed as a case of SLE with secondary APLA who showed neurological improvement which was actually due to the chemotherapeutic effect of cyclophosphamide and steroids, and hence NHL was masquerading for SLE.

Conclusion: APLA is frequently increased in NHL at diagnosis and is associated with poor overall survival in aggressive lymphoma.


  OPC0169: Correlating DAPSA and BASDAI in axial Psoriatic Arthritis Top


Nibha Jain, Dhaiwat Shukla, Puja Srivastava, Sapan Pandya; Sheth V S hospital and Smt NHL Municipal Medical College, Ahmedabadth V S hospital and Smt NHL Municipal Medical College, Ahmedabad, Gujarat, India

Introduction:

  • Disease Activity in Psoriatic Arthritis (DAPSA) is a widely used outcome measure in psoriatic arthritis; however, it does not take into account the axial pain and enthesitis. Bath Ankylosing Spondylitis Activity Disease Activity Index (BASDAI) is a patient-reported outcome measure for axial spondyloarthritis (SpA) and does not use erythrocyte sedimentation rate or C-reactive protein (CRP).
  • Our objective is to correlate DAPSA and BASDAI in axial psoriatic arthritis (AxPsA).


Methods: This is a prospective longitudinal study of 8 months' duration (January 2018–August 2018) done at the outpatient department of a government medical college. All patients of AxPsA (CASPAR criteria) were enrolled after informed consent, and baseline and follow-up demographic data were collected along with the calculation of BASDAI and DAPSA. All data analyses were done using SPSS software.

Results:

  • Total number of PsA patients: 54
  • Number of AxPsA patients: 24
  • Mean duration of AxPsA: 4.6 (standard deviation [SD] 4) years (median = 3 years)
  • Mean age: 40 (SD 12) years (median 42 years)
  • Mean CRP (mg/dl): 12.6 (SD 5)
  • Mean DAPSA: 24 (SD 16)
  • Mean BASDAI: 4.6 (SD 1.6)
  • BASDAI versus DAPSA correlation coefficient (r) = 0.88.


Conclusion: Our data show a good correlation between DAPSA and BASDAI in AxPsA. While BASDAI is a simple tool that has been validated well, it just classifies patients (cutoff 4). DAPSA stages the patients according to disease activity and can be used for follow-up and management of disease even in AxSpA.


  OC0047: Biosimilars in rheumatology: Real-world experience over 3 years Top


Subramanian N Subramanian N; Velammal Medical College Hospital and Research Institute, Madurai, Tamil Nadu, India

Objectives: The objective was to evaluate the effects of biosimilar drugs in common rheumatic diseases in South India.

Methods: All patients who had biosimilars for rheumatoid diseases, psoriatic arthritis, ankylosing spondylitis, or lupus erythematosus (SLE) under rheumatology department were reviewed. All patients should satisfy the American College of Rheumatology criteria for diagnosis; should have failed classic disease-modifying antirheumatic drugs (DMARDs); and had 3-, 6-, and 12-month follow-up and under review.

Results: A total of 91 patients are in the biologic database and 34 were excluded. The mean duration of follow-up was 24 months. Fifty-seven patients were studied, including rheumatoid arthritis (RA – 30), spondyloarthritis (SpA – 17), psoriatic arthritis (PsA) – 5, pulmonary manifestation – 1, and SLE – 4. The details of RA group are as follows: 26 females and 4 males, with mean age of 48.8 years. Mean Disease Activity Score (DAS) 28 was 5.35 and after 3 months of biosimilar, it was 3.1. Twelve patients had Intascept, 7 had exemptia, 7 had Ritux, and 4 had others. Patients are in sustained remission with DAS 28 score of 2.74 (August 2018). The details of SpA group are as follows: 13 males and 4 females, mean age 30 years. Human leukocyte antigen B27 was positive in 6/11 patients. Mean BASDAI score was 5.6 and BASFI score was 5.61. Eleven patients had exemptia and six had others. Three months after biosimilar, BASDAI score was 2.1 and BASFI score was 2.8 and still in remission. Mean duration of follow-up was 21 months. The details of PsA group are as follows: -five patients had adalimumab biosimilar for peripheral arthritis and now in remission, three are on-demand biosimilar, and two on methotrexate. The details of SLE group are as follows: four patients had rituximab biosimilar. All had nephritis too. Three patients had avascular necrosis hips and had failed cyclophosphamide. All are on mycophenolate mofetil and hydroxychloroquines. Systemic Lupus Erythematosus Disease Activity Index score before biosimilar was 31 and 6 months after treatment, it was 8 and now it is 5. All patients came off steroids before biosimilar therapy. Adverse effects: two patients on Intascept had cutaneous allergy, one developed lung tuberculosis (TB), and two patients with rituximab developed lung TB and recurrent impetigo.

Conclusion: Biosimilar DMARDs are safe and effective in achieving long-term remission in RA, SLE, PsA, spondyloarthritis, and myositis.


  OPC0213: Sjogren's syndrome is more common in younger female population in India Top


Sapan Pandya, Balkrishna, Chandrashekara S; Sjogren's interest group (SIG-IRA)

Aim: The aim was to study the demographic and clinical presentations of primary Sjogren's syndrome (SS) in Indian population.

Materials and Methods: This cross-sectional study, conducted at three tertiary care centers across India, included 362 patients with SS. The study excluded patients with overlap connective-tissue diseases. The baseline demographic, clinical, and laboratory parameters were obtained from all the participants and descriptive analysis was performed.

Results: Average age of the recruited patients was 46.74 ± 11.82 years and the male-to-female ratio noted was 1:11. The corresponding incidences of clinical features such as arthritis, myalgia, dry skin/mouth/eyes, Raynaud's phenomena, shortness of breath, chronic dry cough, and sleep disturbances noted were as follows: 179 (49.7%), 70 (19.7%), 48 (23.6%), 316 (88.76%), 321 (91.1%), 14 (6.79%), 10 (4.95%), 20 (9.9%), and 51 (24%), respectively. Out of the 362 patients, comorbidities such as hypertension, diabetes mellitus, and hypothyroidism were present only in 21 (12%), 10 (5.68%), and 17 (9.5%) patients, respectively. Laboratory parameters of SS such as anti-Ro, anti-La, and antinuclear antibody (IF) were positive in 269 (90.26%), 208 (75.91%), and 265 (96.36%), patients, respectively.

Conclusion: SS is not a rare disease. It has high female preponderance, especially in younger females, in contrast to the data from other populations.


  OPC0135: Non-digital ulcers in systemic sclerosis: Prevalence and clinical features Top


Benzeeta Pinto, Ramya Janardana, B Sheba Charles, Jasmine Mathew, John Michael Raj1, Vineeta Shobha; Departments of Clinical Immunology and Rheumatology and 1Biostatistics, St John's Medical College, Bengaluru, Karnataka, India

Background: Skin ulcers (SUs) are a common and difficult-to-manage problem in systemic sclerosis (SSc). There are no consensus on the classification or guidelines for the management of nondigital ulcers (non-DUs).

Objective: The objective was to describe SUs in SSc with a focus on non-DUs.

Methods: We conducted a retrospective study of all patients diagnosed to have SSc in a tertiary care center in the last 7 years. SUs were broadly classified into DUs and non-DUs. Non-DUs were further subclassified based on the proposal by Giuggioli et al.

Results: One hundred and eight patients with Ssc were included (87% females). Mean follow-up was 43.7 ± 39.5 months. The lifetime prevalence of DU was 61.1%. New-onset DUs during the follow-up were seen in 23 patients [Table 1]. Twenty-two patients developed non-DUs during follow-up, 7 within 2 years, 6 in 2–4 years, and 9 patients >5 years after disease onset. The most common site for non-DU was lower limbs. Nine patients with non-DUs had concomitant active DU. Most of the patients (16/22) were treated with steroids. Eighteen patients were on immunomodulatory therapy and nine patients were on colchicine. Immunomodulatory therapy was given for nonulcer indications in most patients. Outcome was available in 18 patients with non-DU (duration of ulcer <6 months in 4 patients). In 12 patients, ulcers healed by 6 months, in 4 patients, ulcers healed in 1 year, and in one patient, the ulcer healed after debridement and skin grafting. One patient expired. No differences were found in clinical features or autoantibody profiles in patients with and without SUs.



Conclusion: SUs were common in our patients with SSc. The etiology of non-DU is multifactorial. Vasculopathy may play an important role for the development of both DU and non-DUs.


  PC0041: Antiphospholipid antibody syndrome: Demography, Clinical and autoantibody profile from a tertiary care center in south India Top


Manoj Kumar, Chengappa KG, VS Negi; Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

Background: Antiphospholipid antibody syndrome (APS) is an uncommon disorder associated with thrombotic, noncriteria manifestations and pregnancy morbidity. However, data regarding various manifestations of disease are scarce from India.

Objectives: The objectives were to characterize the epidemiological, immunological, and clinical manifestations of APS and to compare them among various APS subgroups.

Methods: A retrospective medical record-based analysis of patients attending Clinical Immunology services at JIPMER, Puducherry, from November 2008 to June 2018, with primary APS and APS associated with systemic lupus erythematosus (SLE) was done. Patients with primary APS were stratified into the following three groups: those with isolated thrombotic, those with isolated obstetric, or those with both manifestations. Epidemiological data, immunological parameters, and clinical presentation including thrombotic, noncriteria, and obstetric manifestations were compared among these subgroups and also among primary and secondary APS.

Results: A total of 116 patients were diagnosed as primary APS, of them 74 fulfilled the classification criteria for APS. Lupus anticoagulant was the most commonly detected antiphospholipid antibody. Among these, 31, 22, and 21 had isolated thrombotic, isolated obstetric, or both manifestations, respectively. No difference was noted between antibody positivity and noncriteria manifestations among these groups. The manifestations included deep-venous thrombosis (22%), cerebral venous thrombosis (15%), stroke (11%), skin ulcers (11%), seizures (11%), and thrombocytopenia (8%) (venous thrombosis – 49% and arterial thrombosis – 30%). Of 930 patients with SLE, 73 fulfilled the laboratory criteria for APS and only 16 patients had thrombotic events. Antinuclear antibody (ANA) positivity, cerebrovascular accident (CVA), autoimmune hemolytic anemia (AIHA), leukopenia, and low complement levels were more frequent among patients of SLE with secondary APS as compared to patients with primary thrombotic APS [Figure 1]. Eighteen patients had recurrent thrombosis, with the median interval between the two events being 13.1 months.



Conclusion: Among the different subgroups of primary APS, demographical, immunological, and clinical manifestations were comparable. Antinuclear antibody positivity, CVA, AIHA, leukopenia, and low complement levels were more frequent in SLE with secondary APS as compared to primary APS.


  PC0044: Hyperferritenemia as an initial presentation of systemic lupus erythematosus (SLE) with sle disease activity index (SLEDAI)<10 and no clinically apparent antiphospholipid syndrome (APS) Top


Debasish Sinha, Syamalkundu; Bankura Sammilani Medical College and Hospital, Bankura, West Bengal, India

Case Report: We report the case of a 30-year-old female patient who presented with fever, oral ulcers, and heavy menstrual bleeding. On evaluation, the patient was found to have minimal ascites, small right pleural effusion, hemolytic anemia, and pancytopenia. Her serum ferritin level was too high (2000 ng/ml). On further investigations, antinuclear antibody was negative, but anti-dsDNA was positive. As the diagnostic criteria for systemic lupus erythematosus (SLE) were fulfilled, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was calculated as 7 (mucosal ulcers = 2; pleurisy = 2; and 1 each for fever, thrombocytopenia, and leukopenia). Further clinical and laboratory evaluation excluded the possibility of hematochromatosis (serum iron was normal, fasting blood sugar was normal, contrast-enhanced computed tomography abdomen revealed only mild hepatomegaly, and there were no cardiomegaly and no gonadal failure), adult-onset Still's disease (did not fulfill Yamaguchi criteria), and hemophagocytic lymphohistiocytosis (bone marrow aspiration cytology revealed mildly hypoplastic marrow). There was no clinical manifestation of antiphospholipid antibody syndrome (APS).

The patient was treated with supportive measures including blood and platelet transfusion with minimal benefit. Pulse methyl prednisolone was started after 7 days. The patient started showing improvement in another 6 days. She was treated further with prednisolone 60 mg OD + hydroxychloroquine 400 mg OD + chronic proton pump inhibitor + calcium. At discharge after 3 weeks, she had normal complete blood count and feeling of general well-being.

Discussion: In SLE – SLEDAI >10, secondary APS, thrombocytopenia, and serositis are correlated with hyperferritinemia. Hyperferritinemia is thus taken as an important marker of disease activity (SLEDAI >10) in SLE as well as a predictor of manifestation of secondary APS, but our patient had hyperferritinemia with SLEDAI <10.

Conclusion: Hyperferritinemia (serum ferritin = 2000 ng/ml) with SLEDAI <10 may be a rare initial presentation of SLE without APS, as seen in our case.


  PC0071: New kid on the block : OCT scanning in patients on HCQ Top


Dhaiwat Shukla, Nibha Jain, Puja Srivastava, Sapan Pandya; Smt. NHL MMC, Ahmedabad, Gujarat, India

Background: The newer American Ophthalmic Society recommendations suggest using optical coherence tomography(OCT) scans for hydroxychloroquine (HCQ) screening. There is scarce Indian data on the same. We therefore decided to study this.

Objective: The objective was to study if OCT scans pick up early HCQ toxicity in rheumatology patients.

Methods: Ours was a cross-sectional study. All patients attending rheumatology outpatient department at VSGH and on HCQ therapy for any indication, irrespective of the duration of the disease, were enrolled. Demographic data, clinical details (diagnosis, duration of illness, body mass index, etc.), and details of HCQ use including the current dose, previous doses, and average cumulative dose were recorded in pro formas. Patients were evaluated by an ophthalmologist (retina expert) for visual acuity, field of vision, fundoscopy, and all of them underwent an OCT scan.

Results:

  • Total patients: 143
  • Mean age (standard deviation [SD]): 43.03 (13.9747)
  • Mean HCQ cumulative dose (SD): 241.9 (283.606)
  • Mean HCQ dose (milligram per kilogram) (SD): 5.09 (1.50)
  • Mean duration of HCQ intake: 2.72 (2.57).


A total of 20/143 patients with HCQ intake >5 years did not show any clinical or OCT evidence of maculopathy. Only one patient, a 55-year-old woman with RA, had classical bull's eye maculopathy on fundoscopy as well as on OCT without the clinical features of maculopthy detected during the study.

Conclusion: The OCT scan did not pick up any HCQ ophthalmic toxicity with a mean 2.7 years of use and mean cumulative dose of 241.9 gm. These patients will need to be prospectively followed up with follow-up OCT scans and longer duration to validate their utility in these patients.


  OPC0138: A report on ANCA associated vasculitides from western part of india Top


Alpana Parmar, Shabbir Chikani, Sapan Pandya, Puja Srivastava, Vishnu Sharma, Namisha Patel, Pradip Prajapati; Rheumatology Association Gujarat

Background: Data on antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are scarce from this part of the subcontinent.

Objectives: The objectives were to study demographic, clinical, and laboratory features; treatment given; and outcome of patients with AAV at a private practice setup in Gujarat.

Methods: Data were collected from seven centers across Gujarat. International Chapel Hill criteria (2012 revised) were used to classify patients with AAV.

Results: Among 53 AAV patients, 35 (66%) had granulomatosis-with-polyangiitis (GPA), 9 (16.98%) had microscopic polyangiitis and 10 (18.9%) had eosinophilic GPA. Seventeen were males (male:female ratio – 1:2). Age of presentation ranged from 12 to 80 years, duration of illness ranged from 15 days to 3 years, and Birmingham Vasculitis Activity Score was 3–33 (mean 15). Fever and weight loss were present in all patients, scleritis in 18 (33%), epistaxis in 23 (43.4%), paranasal sinusitis in 17 (32%), subglottic diseases in 4 (7.5%), sensory deafness in 9 (16.9%), dyspnea in 24 (45.2%), hemoptysis in 10 (18.86%), and wheezing in 24 (45.2%) patients. Peripheral neuropathy was seen in 8 (15.09%) and mononeuritis multiplex in 4 (7.5%) patients.

Active urine sediment/proteinuria was noted in 18 (33.9%) and elevated serum creatinine in 15 (28.3%) patients. Imaging findings are as follows: bilateral infiltrates in 28 (52.83%), nodules/cavities in 11 (20.7%), diffuse alveolar hemorrhage in 3, and interstitial lung disease in 3 patients. ANCA positivity was seen in 52 (98.11%) patients. Enzyme-linked immunosorbent assay was done in 32 patients – 20 (62.5%) were anti-PR3 positive, while 12 (37.5%) were antimyeloperoxidase positive. For induction, cyclophosphamide was used in 37 (69%), rituximab in 9 (16.9%), methotrexate in 7 (13.2%) and azathioprine in 5 (9.43%) patients. For maintenance, azathioprine was used in 22 (41.5%) patients. Relapses were noted in 23 (52.2%) patients – lung (5; 21%), 2 organs (4; 17.39%), and renal (3; 13%). Relapses were treated with rituximab in 13 (56.5%) patients. Remission was achieved in all relapses. Sequelae were hearing loss, nasal/orbital wall damage, and chronic kidney disease. No deaths were reported.

Conclusions: GPA was more common of AAVs. Majority presented with constitutional and pulmonary symptoms. Most rheumatologists used cyclophosphamide for induction and rituximab for relapses. Outcome was good in majority of the patients.


  OC0042: To evaluate the association of anti-CCP antibody with echocardiographic findings in established cases of Rheumatoid Arthritis Top


Ravi Nishad; RIMS, Imphal, Manipur, India

Cardiovascular disease is the most common cause of death in rheumatoid arthritis (RA) patients. Anti-cyclic citrullinated peptide (CCP) appears to confer an increased risk. The study was to assess the association of anti-CCP antibody with echocardiographic findings in RA patients.

Aims and Objectives: (1) To perform transthoracic echocardiography in patients of RA and determine the association of anti-CCP antibody with echocardiographic findings and (2) To evaluate the contribution of anti-CCP as an independent risk factor for cardiovascular disease in RA patients.

Materials and Methods: This was a cross-sectional study done among 99 diagnosed patients of RA, admitted in medicine wards and those attending the rheumatology outpatient department from September 2015 to September 2017 at RIMS, Imphal, Manipur, India.

Results: Anti-CCP antibodies were detected in 63.6% of our study population. Anti-CCP-positive patients had higher disease duration (P = 0.291) as compared to the other group. Only six patients (6.06%) had valvular involvement on echocardiography. All of them were positive for anti-CCP antibodies (P = 0.3). Mild mitral regurgitation alone was observed in three patients, mild aortic regurgitation alone in one patient, and coexisting mitral and aortic regurgitation in one patient. Ejection fraction appeared to be diminished in the anti-CCP-positive group. Left ventricular systolic dysfunction was noted in eight anti-CCP-positive patients (8.1%) and none in the negative group (P = 0.091). Concentric left ventricular hypertrophy was another finding observed in six patients, all of them were positive for anti-CCP antibody (P = 0.208). The anti-CCP-positive group had slightly higher incidence of diastolic dysfunction as compared to the negative group (37.4% vs. 33.3% with P = 0.14). There was significant relation of anti-CCP with disease activity which was ascertained by calculating Disease Activity Score 28-erythrocyte sedimentation rate score (P ≤ 0.001).

Conclusion: There was no significant increase in echocardiographic abnormalities in clinically asymptomatic anti-CCP-positive RA patients when compared with anti-CCP-negative patients after excluding conventional cardiovascular risk factors.


  OPC0139: Sex hormones in SLE and their relation with disease activity Top


Mahesh K Katakbhavi, Daisy Doley, Rebecca Marak, Sanjeeb Kakati; Assam Medical College and Hospital, Dibrugarh, Assam, India

Background: Systemic lupus erythematosus (SLE) is an autoimmune, multisystemic disease known for its female predilection and peak incidence during the reproductive years. The increased female-to-male ratio of SLE patients suggests that sex hormones modulate disease proclivity and development.

Objectives: The objective was to assess the levels of various sex hormones in female SLE patients and its relation with disease activity.

Methods: Sera of 82 female SLE patients were tested for various sex hormones such as luteinizing hormone (LH), estradiol (E2), and progesterone (P). Blood samples for LH were collected in the follicular phase and those of E2 and P in the luteal phase. LH, E2, and P were measured by enzyme immunoassay and the disease activity was calculated using Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores.

Results: The mean age of SLE patients was 26.05 ± 8.34 years. The P level was lower in the age group of 15–20 years, 31–35 years, and >35 years with a mean of 1.29, 2.72, and 3.55, respectively, and the E2 levels were found to be high in the age group of 31–35 years and >35 years with a mean of 369.0 and 518, respectively The mean value of LH was significantly higher in the age group of >35 years with a mean of 50.81 (P = 0.000), and no association was found between SLEDAI score and levels of sex hormones.

Conclusion: The reported study suggests that the alterations of sex hormones might be a factor for the development of SLE, but it does not establish any causal relationship. Hence, for a better understanding of hormonal relationship in SLE, further studies need to be done.


  OPC0140: Study on co-morbidities and estimation of atherogenic index of plasma (AIP) in Systemic Lupus Erythematosus (SLE) patients attending a tertiary care centre Top


Sujatha N, Balameena S1, Ramesh R2, Mythili S2, Sowndhariya V A, Karthikeyan S, Aravindan A, Sreedevi S; Post graduate, Institute of Rheumatology, Madras Medical College, 1Senior Assistant Professor, Institute of Rheumatology, Madras Medical College, 2Assistant Professor, Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Systemic lupus erythematosus (SLE) patients have improved life expectancy. Hence, it is imperative to focus on the comorbidities which contribute to morbidity and mortality.

Objectives: The objectives were to evaluate the comorbidities in SLE, prevalence of metabolic syndrome (MetS), and its association with clinical phenotype and disease activity and to compare Atherogenic Index of Plasma (AIP) among SLE patients with and without MetS and with the general population.

Methods: This cross-sectional study included adult SLE patients diagnosed and followed up in our hospital. The demographic data and clinical and laboratory parameters were assessed for all patients. Disease activity was measured using Systemic Lupus Erythematosus Disease Activity Index-2k. MetS was diagnosed according to the National Cholesterol Education Program Adult Treatment Panel III. AIP, calculated as log (triglycerides/high-density lipoprotein-C [in mmol/l]), was compared between SLE patients with and without MetS and with the general population.

Results: A total of 72 SLE patients were included with a mean age of 32.4 ± 8.8 years and median disease duration of 24 months The prevalence of MetS was not significantly increased in patients with neuropsychiatric systemic lupus erythematosus or lupus nephritis (LN). AIP was significantly increased among SLE patients with MetS when compared with those without MetS (P < 0.01), and there was no significant difference in AIP values between patients with active SLE and those in remission and between patients with and without LN. AIP was very significantly elevated among SLE patients when compared with normal population (P < 0.0001).

Conclusion: This study showed that AIP, a marker of increased cardiovascular risk, is significantly increased among SLE patients when compared with the normal population and is further increased by the co-existence of MetS emphasizing the importance of effective management of the comorbidities.


  OPC0141: Acute Pancreatitis in Systemic Lupus Erythematosus: Multicentric retrospective analysis Top


Hafis Muhammed, Mohannad Irfaan Korvi1, Sheba Charles2, Preksha Dwivedi3, Pallavi Pimpale Chavan4, Amit Sharma5, Avinash Jain, Liza Rajasekhar1, Vineeta Shobha2, Varun Dhir3, Aman Sharma3, Ramnath Misra, Amita Aggarwal; Sanjay Gandhi Postgraduate Institute, Lucknow, 1Nizam Institute of Medical Sciences, Hyderabad, Telangana, 2Saint John's medical college, Bengaluru, Karnataka, 3Postgraduate Institute of Medical education and research, Chandigarh, 4Jaslok Hospital and Research Center, Mumbai, Maharashtra, 5Fortis Escorts Hospital, Jaipur, Rajasthan, India

Introduction: Acute pancreatitis (AP) is a rare (0.1%) manifestation of systemic lupus erythematosus (SLE) and has significant mortality. Thus, we studied its presentation and outcome in patients with SLE seen in centers across India.

Methods: On behalf of SLE-SIG group, clinicians were invited to send data of patients with SLE who had AP in the past. Diagnosis of AP required presence of two of the following three criteria: acute-onset epigastric pain, elevation in serum lipase or amylase (≥3 times normal), and characteristic findings of AP on imaging.

Results: There were 43 episodes of AP in 41 patients (36 females, mean age: 26.2 ± 9.3 years). Nearly 97.3% had active lupus (Systemic Lupus Erythematosus Disease Activity Index [SLEDAI] >4), with one-third having nephritis and one-fourth having central nervous system disease. Around 53.5% developed AP within 1 year of diagnosis of SLE. Mean SLEDAI score was 16.7 ± 7.4, and 28 patients were on corticosteroids, 4 on cyclophosphamide, 3 on azathioprine, and 2 were getting mycophenolate mofetil. Among known factors, methotrexate, furosemide, gall stone, and alcohol were implicated in one patient each.

All had abdominal pain, while 53.5% had abdominal distension and 9.3% had jaundice. Thirty-three patients had elevated enzymes and 36 had evidence of AP on imaging. Twenty-one patients had mild, 6 had moderate, and 16 had severe AP (Atlanta classification). There was no difference in lupus features or activity between the three groups.

All received immunosuppression (intravenous methylprednisolone or dexamethasone) (67.4%), cyclophosphamide (30.2%), or oral prednisolone (93.0%). Two patients had necrotizing pancreatitis; 14 had abdominal collections; 11 each had ascites, pleural effusion, and antirheumatic drugs; and 8 had sepsis (3 culture positive) as complications. Eight patients with severe AP died of multiorgan failure. On follow-up, 2 patients developed diabetes, while 1 had malabsorption.

Conclusions: Pancreatitis is an early manifestation of SLE and is associated with active disease. High mortality rate is seen, particularly with severe pancreatitis.


  OPC0144: Osteoporosis in scleroderma: Prevalence, clinical characteristics and correlation with disease phenotype, organ involvement, and vasculopathy Top


Dhaval Tanna, Gayatri Ekbote, Dhiren Raval, Natasha Negalur, Lucky Sharma, Shruti Bajad, Rajiva Gupta; ???, Medanta-The Medicity, Gurgaon, Haryana, India

Background: Inflammation is considered to be the causal factor for osteoporosis in rheumatic diseases. However, apart from inflammation, scleroderma is uniquely characterized by vasculopathy. Clinical features such as digital gangrene and pulmonary arterial hypertension are considered to be manifestations of the underlying vasculopathy. Studies have shown that in scleroderma patients, digits having severe digital gangrenes have higher prevalence of bone loss in the form of acro-osteolysis. From this finding, we extrapolate that scleroderma may have higher prevalence of osteoporosis because of systemic vasculopathy apart from inflammation.

Methods: All patients classified as having scleroderma as per the American College of Rheumatology/European League Against Rheumatism 2013 criteria were included. After written informed consent, demographic profile, clinical features, laboratory and radiology parameters, and treatment details were recorded. Presence of osteoporosis was assessed by two-site dual-energy X-ray absorptiometry (DEXA) scan (hip and lumbar spine) and was correlated with disease phenotype, organ involvement, and vasculopathy. The study was approved by the Ethics Committee (MICR-771/2017).

Results: From June 2017 to August 2018, ninety patients were diagnosed to be having scleroderma. Seventy patients underwent DEXA scan and were included in the present analysis. Demographic and clinical characteristics of the patients are shown in [Table 1]. Out of the 70 patients, 20 (28.5%) had osteoporosis, while 32 (45.7%) had osteopenia as per the WHO definition [Table 2]. More patients in the osteoporotic group were postmenopausal (P 0.01). No association was found between osteoporosis and extent of skin involvement, duration of disease, and type of serology. Presence of interstitial lung disease, pulmonary arterial hypertension, digital ulcers, and acro-osteolysis was found to be significantly associated with osteoporosis (P < 0.05). No association was found between glucocorticoid intake and prevalence of osteoporosis [Table 3].
Table 1: Demographic and clinical characteristics

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Table 1: Bone health in patients undergoing DEXA scan

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Table 2: Comparison of clinical and serological characteristics in patients with and without osteoporosis

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Conclusion: Scleroderma is associated with an increased prevalence of osteoporosis. Vasculopathy may be an important pathophysiologic mechanism causing bone loss as shown by higher prevalence of digital ulcers, pulmonary arterial hypertension, and acro-osteolysis in patients with osteoporosis.


  PC0047: NA Top


Sandesh Guleria, Ankur Kumar Jindal, Rakesh Kumar Pilania, Vignesh Pandiarajan, Deepti Suri, Amit Rawat, Surjit Singh; Post Graduate Institute of Medical Education and Research, Chandigarh, India

Background: Kawasaki disease (KD) is a multisystem medium-vessel vasculitis of unknown etiology affecting predominantly younger children. Arthritis (7.5%–31%) is the commonest musculoskeletal manifestation in KD and resolves in most cases without any long-term sequelae.

Objective: The objective was to study the clinicolaboratory profile of children with KD and arthritis.

Methods: Case records of 680 children of KD, diagnosed at the Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India, during 1994–2017 were analyzed. Of these, 35 children were found to have arthritis during the course of the disease.

Results and Discussion: Occurrence of arthritis was 5.2% (35/680) with significant male predominance (65.71%). Mean age at diagnosis was 5.9 years (range, 1–12 years) and 54.3% children were >5 years of age. Twenty-five (71.4%) children developed arthritis in acute phase, 7 (20%) in subacute phase, and 3 (8.6%) in convalescent phase of KD. Oligoarticular involvement was observed in 28 (80%) children. Knee joint was involved in 74.3%, ankle in 40%, and hip in 28.6% children. Prominent laboratory findings included anemia (82.8%), thrombocytosis (68.6%), elevated erythrocyte sedimentation rate (80%; mean, 51.3 mm in 1st h), and elevated C-reactive protein (92.6%; mean, 100.23 mg/L). Seven (20%) children did not require any specific therapy for arthritis. Twenty-eight children (80%) were treated with nonsteroidal anti-inflammatory drugs (naproxen in 25 and ibuprofen in 3). Median duration of arthritis was 10 days with uneventful recovery in all cases.

Conclusion: While arthritis in children with KD is not an uncommon manifestation, it can result in diagnostic confusion. It is predominantly asymmetric and additive and involves the large joints with a predilection for knees. Arthritis in KD is usually self-limiting and responds well to a short course of nonsteroidal anti-inflammatory drugs.


  OC0049: Masqueraders of vasculitis Top


Prashanth Poulose, N V Jayachandran, N K Thulaseedharan, Jayesh kumar, Sajeeth Kumar; Department of Internal Medicine, Government Medical College, Kozhikode, Kerala, India

Case Report: Vasculitic mimics are more common than primary vasculitis, which should be excluded in the evaluation as treatment varies dramatically. Case 1: Infective endocarditis – a 19-year-old male, with 2-week history of fever, polyarthritis, and altered sensorium for 2 days. Cultures revealed methicillin-resistant Staphylococcus aureus. Echocardiography showed anterior mitral leaflet vegetation. Case 2: Meningococcemia – a 42-year-old male with fever, rash, and drowsiness. Blood pressure was 90/60 mmHg. Cerebrospinal fluid showed Gram-negative diplococci. Antigen detection revealed  Neisseria More Details meningitidis. Case 3: Leptospirosis with secondary vasculitis – a 38-year-old male with fever and decreased urine output. Purpuric lesions were positive, icterus was positive, calf tenderness was positive, and lepto IgM was positive. Case 4: Erythema nodosum leprosum – a 42-year-old male with erythematous nodular lesions. Skin smear – Lepromatous leprosy. Case 5: Left cervical rib with thoracic outlet obstruction – a 26-year-old female with bluish discoloration of her left hand. ADSON was positive. X-ray was suggestive of bilateral cervical rib.

Case 6: Secondary syphilis – a 28-year-old male with oral and genital ulcers.  Treponema pallidum Scientific Name Search magglutination was positive. Case 7: Crohn's disease – a 38-year-old male with fever, polyarthritis, oral ulcers, and rash for 2 weeks. Abdominal pain persisted for 4 days. Hemoglobin was 10.6, SOB was positive. Contrast-enhanced computed tomography (CECT) and colonoscopy were suggestive of inflammatory bowel disease. Anti-Saccharomyces cerevisiae antibodies were positive. Case 8: Cholesterol atheroemboli – a 65-year-old male with coronary artery disease – anterior wall myocardial infarction. Postthrombolysis on day 2, rash developed over the lower limbs; serum creatinine was 2.6, P/s – Eosinophilia, absolute eosinophil count was 1120. Case 9: Ovarian adenocarcinoma with secondary vasculitis – a 56-year-old woman presented with vasculitic lesions for 1 month. Ascites was positive, PV nodules in POD. CECT abdomen – Ovarian malignancy. C/A 125–1445. Biopsy –adenocarcinoma. Case 10: Catastrophic antiphospholipid antibody (APLA) – a 34-year-old female with a history of recurrent abortions and pregnancy-induced hypertension. Later, she developed breathlessness, hemoptysis, gangrene at multiple sites, and acute kidney injury for 5 days. Antiphospholipid antibody Ab was repeatedly positive.

Conclusion: In suspected cases of vasculitis, exclude secondary causes such as drugs, infections, autoimmunity, and malignancy.


  PC0045: Marie–Bamberger syndrome Top


Vikas Sharma, Rajiv Ranjan Kumar, Saket Jha, Sanjay Jain, Aman Sharma; PGIMER, Chandigarh, India

Background: Hypertrophic osteoarthropathy consists of a triad of digital clubbing, periosteal reactions of long bones, and synovitis. Primary form is a rare hereditary disease with skin hypertrophy and hence named pachydermoperiostosis. Secondary form occurs in a majority of patients with etiologies ranging from lung cancers to benign conditions. If etiology is pulmonary in origin, it is referred to as hypertrophic pulmonary osteoarthropathy or Marie–Bamberger syndrome. Physicians not familiar with this entity can easily miss this gateway to diagnose more serious underlying disease.

Case Report: A 59-year-old gentleman, reformed smoker, presented to us with joint pains for 6 months. There was no history suggestive of inflammatory arthritis or history of any systemic involvement. On examination, he had mild pallor, pan digital clubbing, and nonpitting bilateral pedal edema with erythema. Systemic examination was normal. Both knee and ankle joints were tender and there was tenderness over bilateral shin. There were no swollen joints. A differential diagnosis of secondary osteoarthropathy versus paraneoplastic polyarthritis was considered. On evaluation, chest X-ray showed opacity in the right mid zone and X-ray of limbs had subperiosteal new bone formation in diaphysis of both radius and tibia. Positron emission computed tomography and immunohistochemistry were suggestive of non-small cell lung cancer. He was diagnosed as “Marie–Bamberg syndrome” secondary to lung cancer. He had marked resolution of symptoms following treatment with nonsteroidal anti-inflammatory drug, chemotherapy, and radiotherapy.

Discussion: Secondary form accounts for 95%–97% cases of osteoarthropathy. Thompson first described its association with bronchogenic carcinoma in 1904. Since then, its association with multiple malignancies has been reported. It is seen in 0.7%–17% of total lung cancers. The most sensitive test for diagnosis is bone scintigraphy. Apart from symptomatic relief, treatment of the underlying disease has shown to improve symptoms and cause radiological regression. Rheumatologists must be aware of this rare syndrome.


  OPC0145: DRESS; a great mimicker of rheumatic diseases Top


Rutviz Mistry, Avinash Jain, Shiva Prasad1, Abhra Chowdhury2, Durga Prasanna Misra, Narayan Prasad3, Samir Mohindra4, Mohan Gurjar5, Afzal Azim5, Vikas Agarwal; Departments of Clinical Immunology, 3Nephrology and Renal Transplantation, 4Medical Gastroenterology and 5Critical Care Medicine, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 1Department of Rheumatology, Apollo BGS Hospital, Mysuru, Karnataka, 2Department of Rheumatology, Institute of Neurosciences, India

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially life-threatening multisystem disorder with fever, skin eruptions, lymphadenopathy, eosinophilia, and systemic involvement, most commonly after a drug exposure. These cases may mimic various rheumatological conditions. We report a series of 16 cases of DRESS who presented to the rheumatology clinic as suspected connective-tissue disease or sepsis in North India with an aim to report the etiology, characteristics, treatment, and prognosis.

Objectives: The objective was to highlight DRESS as a common mimic of common rheumatologic conditions and sepsis and report its etiology, characteristics, treatment, and prognosis.

Methods: We manually searched the inpatient records of Immunology Department in SGPGI during 2015–2018 for the cases discharged with a diagnosis of possible/probable/definite DRESS. The discharge summaries and outpatient department records of the patients with probable and definite DRESS according to regiSCAR criteria were reviewed.

Results: All the 15 patients fulfilled clinical criteria for diagnosis (four probable and 11 definite series). Median age of the patients was 27 years (Impact Questionnaire Revised: 20–42 years). Majority of the patients were referred to us when their total leukocyte counts were rising in the setting of fever and skin rashes with a suspicion of rheumatic disease/sepsis. The clinical and laboratory features of these patients are summarized in Table.

Out of the 15, patients two patients were lost to follow-up after the 1st visit. Mean follow-up was 9.2 months in our series. All the patients were treated with oral prednisolone of 1 mg/kg/day. Steroids were tapered over 3–6 months in most of the patients. Two patients in our series had relapse of transaminitis on tapering steroids, requiring more than 6 months of steroid therapy.

Conclusions: Skin rash, arthritis, and multiorgan failure of DRESS closely mimic rheumatologic disorders or sepsis (especially with rising total lymphocyte count). Increased awareness and low threshold for suspicion will help in the early diagnosis and favorable outcome.


  PC0046: Buerger's disease: A therapeutic challenge Top


Vikas Sharma, Shankar Naidu, Ritambhra Nada, Manphool Singhal, Prakash, Varun Dhir, Sanjay Jain, Aman Sharma; PGIMER, Chandigarh, India

Background: Buerger's disease is a nonatherosclerotic segmental inflammatory disease that most commonly affects the small- and medium-sized vessels of distal extremities. Its diagnosis can be challenging as it requires exclusion of many other causes. Other than smoking cessation and intravenous (IV) iloprost, there is no standard-of-care treatment. We present a case of Buerger's disease treated successfully with tadalafil, a phosphodiesterase type 5 (PDE5) inhibitor.

Case Report: A 28-year-old gentleman from Gujarat presented with recurrent ulceration in both hands and feet for the last 12 years and was being treated as cutaneous polyarteritis nodosa. He had no relief in symptoms despite various immunosuppressive agents including steroids, azathioprine, mycophenolate mofetil, and cyclophosphamide. He denied constitutional symptoms and symptoms to suggest systemic nature of illness. He had intermittent claudication, Raynaud's phenomenon, and significant history of tobacco consumption. On examination, he had ulcers in all the four extremities with absent dorsalis pedis and anterior tibial pulses bilaterally. Computed tomography angiography revealed severe attenuation of bilateral anterior tibial and peroneal arteries with corkscrew collaterals. All the other investigations including inflammatory markers, antinuclear antibody, antineutrophil cytoplasmic antibody, procoagulant workup, and viral markers were negative. A diagnosis of Buerger's disease was made and he was started on ecosprin, statins, and tadalafil. He had a dramatic response at 3 months of follow-up.

Conclusion: PDE5 inhibitors cause vasodilation in pulmonary and peripheral circulation. Abeles et al. have successfully used tadalafil in Buerger's disease as maintenance agent after induction with IV iloprost. Ours is the first case where we have successfully used PDE5 inhibitor as induction agent as well. Prospective studies are the need of the hour.


  OPC0146: Prevalence of IgA Anticardiolipin antibody and its association with pregnancy morbidity in Asian Indian patients with Primary antiphospholipid antibody syndrome and Systemic lupus erythematosus Top


Harshini Shivakumar, Jayakanthan K, Mahasampath Gowri, Debashish Danda, John Mathew; Christian Medical College, Vellore, Tamil Nadu, India

Background: There are many clinical and laboratory parameters which have been proven to be associated with an increased risk of pregnancy morbidity in patients with systemic lupus erythematosus (SLE) and anti-phospholipid antibody syndrome (APS). This study was undertaken as there is a lacuna in explaining all the pregnancy-related morbidity in patients with SLE and APS.

Objectives: Primary objective – to assess whether the IgA anticardiolipin antibody correlates with the risk for pregnancy morbidity in patients with APS/SLE. Secondary objective – To find the association with the risk of thrombotic and embolic events.

Methods: Patients who have been diagnosed as SLE and/or primary APS who are married and conceived at least once were recruited and 3 ml blood sample was obtained from these patients for immunoglobulin A (IgA) anticardiolipin assay. They were categorized into two different groups depending on whether they had a pregnancy morbidity or not. Then, the significance of this antibody in both the groups was compared.

Results: A total of 186 patients were recruited with a mean age of 34.5 years and a mean duration of illness of 5.52 years with a diagnosis of 76.88% SLE, 7.53% primary APS, and 15.59% SLE with secondary APS. Nearly 36.56% of patients had pregnancy morbidity (majority being pregnancy-induced hypertension 14.52%, prematurity 11.29%, intrauterine fetal growth restriction 10.22%, preecclampsia 3.76%, and eclampsia 1.08%) and 19.35% of patients had a history of thromboembolic events. IgA anticardiolipin was found to be positive in 4.84% (n = 9), i.e., 10.29% in the group with pregnancy morbidity as against 1.69% without pregnancy morbidity. Out of the total positives, titer in 37.5% was indeterminate; 25% low positive and 37.5% high positive. All patients who had high titer positivity had pregnancy morbidity. The positivity in group with thromboembolic events was 5.56%.

Conclusions: Though the positivity for IgA anticardiolipin antibody was found to be more in patients with pregnancy morbidity in SLE and APS, statistical significance was not reached.


  OPC0215: A case report of giant cell arteritis with polymyalgia rheumatica in a 46-year-old female Top


M P Das, Rajshekhar Chakraborty, Subhajit Mitra, Ashish Agrawal; Gauhati Medical College and Hospital, Guwahati, Assam, India

Case Report: We report a case of a 46-year-old married female who presented with severe headache more on the left side, jaw claudication, fever, fatigue, and anorexia for the last 6 months. It was preceded by pain and stiffness of shoulder, hips, and thigh for the last 2 years. The nonspecific symptoms and age <50 years led her being treated as a case of fibromyalgia with amitriptyline. When she reported to us, her erythrocyte sedimentation rate was 140 mm at the end of the 1st h, and C-reactive protein was 221 mg/dl. The patient's superficial artery tenderness, jaw claudication, raised inflammatory markers, and history of polymyalgia rheumatica were highly suspicious of giant coronary aneurysm (GCA). As the consent for biopsy of the artery could not be obtained, Doppler study was done which showed irregular wall thickening and stenosis of bilateral superficial temporal artery more on the left side suggestive of GCA. A diagnosis of GCA was made as per the American College of Rheumatology guidelines and the patient was initiated on prednisolone 60 mg/day. There was significant improvement in the muscle-related complaints and alleviation of pain. There was also steady decline in inflammatory markers.

Conclusion: Giant cell arteritis (GCA) along with polymyalgia rheumatica is a large-vessel vasculitis affecting adults aged >50 years. A well-recognized and dreaded complication is ischemic optic neuropathy which may lead to sudden blindness. This complication can be prevented by early initiation of glucocorticoid therapy. Rarity of prevalence in our part of the country sometimes leads to misdiagnosis of the disease. Our report underscores the need for complete evaluation of middle-aged patients presenting with headache for GCA.


  OPC0148: Profile of ocular changes in patients taking hydroxychloroquine: An observational study at a tertiary care center Top


Vivek Vasdev, Anuradha Singh, Sagarika Patiyal, Ramakant Singh, Arun Hegde, Ashwani Kumar, K Kishore, S Bhatt; Army Hospital, Research and Referral, New Delhi, India

Background: Hydroxychloroquine (HCQ) is one of the most frequently prescribed medicines in rheumatological clinics. Ocular toxicity is one of the most feared side effects and requires frequent evaluation of patients taking long-term HCQ.

Methods: A total of 100 patients attending rheumatology outpatient department and receiving HCQ irrespective of the indications for at least 5 years were eligible for the study. Patients with preexisting ocular morbidities at the time of recruitment were excluded. The patients were also subjected to visual acuity testing with Snellen's chart and near vision including best-corrected visual acuity, Amsler Grid assessment, color vision assessment with Ishihara plates, slit-lamp examination, indirect ophthalmoscopy, 10-2 automated perimetry, spectral domain-optical coherence tomography (SD-OCT), color fundus photography, fundus fluorescein angiography, and fundus autofluorescence (FAF).

Results: The mean age was 52.9 ± 10.30 years. The mean daily intake of HCQ was 212 ± 47.73 mg over a mean duration of 8.25 ± 3.58 years. The cumulative dose intake was 621 ± 261.80 g. Majority of the patients were asymptomatic. SD-OCT, 10-2 automated perimetry, and FAF picked up retinal abnormality in 17%, 21%, and 10% cases, respectively. Amsler grid, color fundus photography, color vision, and fundus fluorescein angiography failed to detect abnormalities in these patients.

Conclusions: This study highlights the importance of ophthalmological assessment of patients on long-term HCQ treatment irrespective of symptoms.


  OPC0149: To assess the response to methotrexate in treatment naive Takayasu arteritis patients Top


Spoorthy Kothapalli, Phani kumar D, Ramakrishna1, Rajasekhar L; Departments of Clinical Immunology and Rheumatology and 1Radiology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Objective: The objective was to evaluate clinical outcome with methotrexate in patients with active Takayasu's arteritis (TA).

Methods: In this prospective study from May 2017 to April 2018, patients fulfilling the American College of Rheumatology criteria for TA and who had active disease (Indian Takayasu Clinical Activity Score [ITAS]-A >5) were included. Detailed clinical and erythrocyte sedimentation rate (ESR), two-dimensional echocardiography, and computed tomography/magnetic resonance angiographic details were noted. Recruited patients were followed up serially to assess the response to treatment based on ITAS-A. ITAS-A and Takayasu Damage Score were done every 3 monthly and 6 monthly, respectively. Descriptive statistics for the categorical variables were performed by computing frequencies in each category.

Results: Of thirty patients with TA, 17 patients had at least 6 months of follow-up. All were started on methotrexate (mean dose 14.4 ± 1.6 mg/week) and steroid (0.5–1 mg/kg in tapering dose). The mean age was 28.4 ± 9.7 years and the median time gap between symptom onset and diagnosis of TA was 6 months (4–36). The commonest symptom was limb claudication (9/17). Hypertension was seen in 8/17 patients, of them six patients had renal artery involvement. The most common angiographic type was Type V (7/17), with left subclavian artery predominantly involved. Vessel wall narrowing is the commonest angiographic abnormality. Symptoms improved in 15/17 patients at 6 months. Median ESR decreased from 35 (19–52) to 27 mm (17–35). Median ITAS-A at baseline was 13 (10–18), at 3rd month was 1 (0–3), and at 6th month was 1 (0–1). No changes in the peripheral pulses or difference in limb blood pressures were noted.

Conclusion: An immunosuppressive regimen of methotrexate and prednisolone is safe, well tolerated, and effective in ameliorating systemic symptoms and laboratory parameters of disease activity in TA.


  OPC0216: Adult-onset Still's disease: Diagnosis of exclusion “still” rare, a retrospective study on clinical profile and outcome at a tertiary care center Top


Gayatri Ekbote, Lucky Sharma, Dhaval Tanna, Dhiren Raval, Natasha Negalur, Wasim Kazi, Rajiva Gupta; Division of Rheumatology and Clinical Immunology, Medanta-The Medicity, Gurgaon, Haryana, India

Adult-onset Still's disease (AOSD) is a diagnosis of exclusion and area of confusion. It is often difficult to exclude other diseases namely infections, malignancies, and other connective-tissue diseases which mimic as AOSD. However, uniformly used Yamaguchi criteria have made it somewhat easier to classify this rare disease.

In this retrospective study, a total of 34 patients of diagnosed AOSD as per Yamaguchi criteria were included. The study period was September 2012 till July 2018. Past and present medical records were noted. Data during the initial presentation were recorded. Outcome was noted during the study period.

In this study, female-to-male ratio was 1:1.2 (16 and 18, respectively) and mean age (standard deviation) was 35.17 years (12.4). Median duration (range) from the first symptom onset to diagnosis was 3.5 months (0.5–5.5 months). Follow-up period was 3–30 months (median 15 months). Baseline clinical and laboratory features are summarized in [Table 1] and [Table 2].



Systemic steroids were given in all patients. Methotrexate was the commonest steroid-sparing agent used mostly for arthritis. Two patients were given tocilizumab because of persistent disease activity and recurrent flares.

Initial low albumin and low-to-normal total counts were predictors of macrophage activation syndrome (MAS).Three mortalities were seen during the study period.

Hence, AOSD is a multisystem disease with increased chances of MAS and still has high mortality rate.


  OPC0151: Lung Dominant Connective Tissue Disease : A Case Series Top


A. Raut, R. Samant, C. Balakrishnan, G. Kakade, A. Khune, R. Nanavati, S. Yadav; P.D. Hinduja National Hospital and MRC, Mumbai, Maharashtra, India

Introduction: Patients with interstitial lung disease (ILD) who have specific autoantibodies in the absence of extrathoracic features of definite connective-tissue disease (CTD) are termed as having LD-CTD. Here, we present eight cases of LD-CTD, their clinical features, management, and outcome.

Methods: We retrospectively collected data of eight patients attending collagen-lung clinic at our institute, who had ILD based on chest computed tomography (CT), pulmonary function test findings without clinical features of CTD, and tested positive more autoantibodies followed over 4 years.

Results: Out of a total of 8 patients, 7 were females and 1 was male. Median age at presentation was 49 years (range: 33–88 years). Seven out of 8 patients first presented to chest physician with cough and breathlessness. Three patients had nonspecific polyarthralgia without synovitis. Two patients had gastroesophageal reflux disease. On high-resolution computed tomography (HRCT), five patients had nonspecific interstitial pneumonia (NSIP) pattern, two had usual interstitial pneumonia, while one patient had no definite pattern. Two patients showed progression on follow-up CT. Median forced vital capacity % predicted was 56.5 (37–88), diffusing capacity of lung for carbon monoxide was 33% (11–91), 6 min walking distance was 319.5 m (315–567). Antinuclear antibody by immunofluorescence was positive in seven patients, anti-Ro/anti-Sjögren's syndrome in three patients, rheumatoid factor in four, anti-citrullinated peptide antibody in two, anti-ds-DNA in two, and anti Scl-70 in one patient. All patients were treated with corticosteroids. In addition, azathioprine was used in five patients, mycophenolate mofetil in three, methotrexate in two, hydroxychloroquine in three, and cyclophosphamide in one patient. One patient also had severe pulmonary arterial hypertension with right heart failure which was treated with tadalafil, ambrisentan, and home O2 with diuretics. Five patients improved on treatment, two worsened, and one lost to follow-up. One patient developed steroid-induced osteoporosis with vertebral fracture.

Conclusions: In our LD-CTD case series, there was female predominance with NSIP pattern on HRCT with all patients were positive for different autoantibodies. Hence, it is worthwhile to screen every ILD patient for autoantibodies, as they may respond better to immunosuppressants.


  OPC0217: Etiological study of anemia in rheumatoid arthritis Top


Irfanul Hoque Choudhury, Mrinal Gogoi, A. K. Sen; Assam Medical College, Dibrugarh, Assam, India

Background: With the fact that since very few studies have been done on anemia in rheumatoid arthritis from northeastern India, this study has been undertaken with the following aims and objectives.

Objectives: The objectives were to study the prevalence and cause of anemia in rheumatoid arthritis and relationship of anemia with the disease activity.

Methods: This hospital-based observational study was conducted for a duration of 1 year. A total of 137 (32 males and 105 females) patients of rheumatoid arthritis (RA) ≥13 years of age, diagnosed by the 2010 American College of Rheumatology/European League Against Rheumatism criteria for RA, meeting inclusion and exclusion criteria, were included in the study. After enrolling and through clinical examination, the following investigations were done in each patient: complete blood counts, peripheral blood smear, erythrocyte sedimentation rate, serum ferritin estimation, total iron-binding capacity, hemoglobin typing, serum B12 estimation (if mean corpuscular volume >100), stool for occult blood, and upper gastrointestinal (UGI) endoscopy (whenever indicated).

Results: Among the 137 patients, anemia was found in 91 (66.42% [anemia chronic disease in 42.86%, iron deficiency anemia in 36.26%, hemoglobinopathies in 17.58%, and megaloblastic anemia in 3.30%]). Stool for occult blood was positive in ten patients. UGI endoscopy showed antral erosions in four and chronic duodenal ulcer in six patients. Mean Disease Activity Score (mDAS)28 was found higher in anemic patients compared to nonanemic (P < 0.0001). In addition, a significant association was found between early-morning stiffness, mean tender joint count, and swollen joint count in anemic and nonanemic patients. DAS in anemic patients has an inverse correlation with hemoglobin (r = −0.4291, P < 0.0001). There is a positive correlation between tender joint count and disease activity in anemic patients (r = 0.8999, P < 0.0001).

Conclusions: Anemic patients with RA have higher disease activity compared to nonanemic patients. Cause of anemia in RA is multifactorial and in this part of country, it is affected by the higher prevalence of hemoglobinopathies.


  OPC0154: A case series of pediatric-onset Behçet's disease from a tertiary-care centre from North India Top


Anjani Gummadi,Pandiarajan Vignesh, Rakesh Kumar Pilania, Avinash Sharma, Deepti Suri,Anju Gupta, Biman Saikia, Ranjana W.Minz, Amit Rawat, Surjit Singh; Postgraduate Institute of Medical Education and Research, Chandigarh, India

Case Report: Case records of children with pediatric Behçet's disease (PED-BD) (1998–2017) were retrieved from the clinic files of the Pediatric Rheumatology Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India. A retrospective analysis was done, and parameters including age of onset, sex, clinical manifestations, inflammatory parameters, and treatment outcomes were analyzed. Presence of human leukocyte antigen (HLA)-B51 was assessed by polymerase chain reaction in all patients. The diagnosis of BD was based on the diagnostic criteria from the Behçet Syndrome Research Committee of Japan (1987 Revision). Thirteen children had suspected PED-BD based on clinical consensus, out of which 10 were classified as BD. Three children had only ocular manifestations (posterior uveitis – 2, panuveitis – 1, and retinal vasculitis – 3) and positive HLA-B51 by polymerase chain reaction. Mean age of onset of clinical manifestations and diagnosis of BD was 5.1 and 7.7 years, respectively. Male:female ratio was 4:1. Recurrent oral ulcers was the most common manifestation (9/10). Uveitis was the initial and presenting manifestation in one of the patients (10%). Genital ulcers were seen in four children and skin manifestations were noted in four children. Positive pathergy test was observed in four children (40%). Inflammatory colitis was seen in three children. Uveitis was noted in three children and all were boys. Ocular manifestations noted were retinal vasculitis, posterior uveitis, and panuveitis. Arthritis was noted in three children. HLA-B51 was positive in four children. One child died due to pulmonary hemorrhage. Thrombotic manifestations were not seen in our cohort.

Conclusion: We describe the first case series of PED-BD from India. A significantly higher proportion of male children (80%) was noted in our cohort. Arterial stenotic lesions mimicking Takayasu's arteritis, though well reported in adults, are described for the first time in a cohort of PED-BD.


  OC0032: Emergencies in lupus Top


Prashanth Poulose, Jayachandran NV, Jayesh Kumar, Sajeeth Kumar, Thulaseedharan, Chandni R; Department of Medicine ,Government Medical College ,Calicut, Kerala, India

Introduction: Systemic lupus erythematosus (SLE) patients are prone to develop serious life-threatening emergencies.

Case Series: Case 1 – Diffuse alveolar hemorrhage: a 29-year-old female patient with SLE with hemoptysis. Chest X-ray, computed tomography thorax, and bronchoalveolar lavage were suggestive of diffuse alveolar hemorrhage. Case 2 – Lupus nephritis (class-6); end-stage renal disease – a/c pulmonary edema: a 15-year-old patient with a history of polyarthalgia for 6 months now presented with acute breathlessness and decreased urine output. Clinical findings were as follows: urea – 140, serum creatinine – 9.8, serum C3 – 42, antinuclear antibody (ANA) by IF was 2+ positive, anti-dsDNA was positive. Case 3 – SLE vasculitis, critical limb ischemia: a 32-year-old k/c/o SLE, with a history of cardiovascular accident 1½ years back. Now presented with left foot gangrene. She also had left partial claw hand, bilateral foot drop (suggestive of mononeuritis multiplex). Case 4 – Evans syndrome: a 22-yearold female presented with a history of joint pain and recurrent oral ulcers presented with fatigue. Pallor was positive, splenomegaly was positive, hemoglobin – 4.2, platelet count – 23,000, erythrocyte sedimentation rate – 130 mm/h, reticulocyte – 5.2, direct Coomb's test (DCT) and immunochromatographic test (ICT) were positive, and ANA and anti-dsDNA were positive. Case 5 – A/c pulmonary thromboembolism, SLE with secondary antiphospholipid antibodies (APLA): a 15-year-old girl with a history of polyarthritis for 2 months now presented with acute breathlessness. Case 6 – Thrombotic thrombocytopenic purpura: a 17-year-old girl with SLE presented with altered sensorium and seizures. On deranged renal function test: P/s–MAHA, DCT, and ICT were negative. Case 7 – Avascular necrosis of bilateral femur: a 32-year-old female k/c/o SLE on steroids now c/o bilateral hip pain. Case 8 – SLE with secondary APLA: a 20-year-old girl with SLE presented with gangrene at multiple sites. Case 9 – Right synpneumonic effusion: a 26-year-old female with SLE presented with fever, cough, and breathlessness. X-Ray – right synpneumonic and pericardial effusion. Case 10 – A/c Budd–Chiari syndrome: A 23-yearold female with complaints of abdominal distension, pain, and jaundice.

Conclusion: Prompt recognition and aggressive management is required to prevent morbidity and mortality.


  OPC0156: An audit of biologic/biosimilar usage amongst rheumatologists across Gujarat Top


Sharma V, Solanki R, Sharma R, Jain N, Shukla D, Shah N, Srivastava P, Chikani S, Shah R, Patel N, Bavaliya M, Parmar A, Sanap A, Parikh T, Pandya S; (Rheumatology Association Gujarat) Vedanta institute of medical sciences, Ahmedabad, Gujarat, India

Background: With the availability of biosimilars, usage of biologics has increased throughout the country. We planned to do an audit of the use of same in the state of Gujarat.

Objective: The objective was to perform an audit of biologic/biosimilar usage for rheumatologic indications by specialists across the state of Gujarat.

Materials and Methods: Ours was a cross-sectional study. Data were filled in pro formas by practicing rheumatologists from 12 centers across the state of Gujarat. It included apart from demographic profile of patients, diagnoses of illnesses, indications, induction or maintenance, brand details, biosimilar or innovator, vaccination schedule, comorbidities, adverse effects related to biologic usage, and if patients had a reimbursement/insurance coverage. The study duration was from July 2015 to July 2018. Descriptive statistics was used to analyze the data.

Results: A total of 670 patients received biologics/biosimilars during the period. The diagnoses were as follows: 38% rheumatoid arthritis (RA), 30% spondyloarthritides (SpA), 7% vasculitides, 5.6% connective-tissue diseases, 5.5% systemic lupus erythematosus, 4.65% juvenile idiopathic arthritis, the rest were psoriatic arthritis, Sjogren's syndrome, immunoglobulin G4-related syndrome, and organ-threatening vasculitides. About 77.7% paid for the same from their pockets. Rituximab was the most frequently used biologic (44.7%) followed by etanercept (22.6%), adalimumab (15%), infliximab (9.8%), and then secukinumab, tocilizumab, golimumab, and abatecept. Of the biologics prescribed, 70.4% were biosimilars. A total of 15 deaths were documented, out of which nine were on rituximab (infections in five and disease related in two), three on inflximab (infections all), one on tocilizumab, one on secukinumab, and one on golimumab. Out of these, five died of tuberculosis. Infusion-related hypersensitivity reactions occurred in five patients, severe enough to stop the infusion.

Conclusion: Most patients received biosimilars. RA and SpA accounted for 2/3rd of indications for biologic usage. Infections were the leading cause in those who died, with tuberculosis in half of them.


  OPC0158: Gender differences in psoriatic arthritis and relevance of age of onset of psoriasis Top


Taral Parikh, Sapan Pandya, Rakesh Solanki; Vedanta Institute of Rheumatic diseases, Ahemdabad, Gujarat, India

Background: It is known that males with spondyloarthritis have more spinal disease, while in females, peripheral involvement is dominant. Gender differences have not been studied well in psoriatic arthritis (PsA).

Methods: A total of 163 patients, seen in the outpatient department from 2012 to 2018, fulfilling the CASPAR criteria, were analyzed. Disease variables were noted in a predefined pro forma. The population was stratified by age at the time of onset of psoriasis, using a cutoff point of 40 years.

Results: The patient population constituted 107 males and 56 females with a mean age of 43.32 (±11.02) years. Mean weight was 69.5 kg (±13.05). The mean duration of onset of psoriasis was 8.73 (±7.35) and mean duration of onset of PsA was 3.6 (±4.41). Mean Psoriasis Area and Severity Index was 3.08 (±5.45). Mean number of swollen joint count was 4.05 (±4.67) and tender joint count was 14.8 (±9.10). Family history of psoriasis was found in 34 patients (20.85%). Pattern of joint involvement: Overlap: Axial + oligoarthritis in 30 (18.5%), axial + polyarthritis in 27 (16.5%), and axial + Distal in 4 (2.5%). Isolated: polyarthritis was seen in 50 (30.65%), oligoarthritis in 37 (22.5%), axial in 10 (6.15%), and isolated desquamative interstitial pneumonia in 1 (0.6%). Clinical enthesitis was found in 36 (22.08%) and dactylitis in 59 (36%) patients. Significant differences in the clinical variables between males and females: Men were obese, had more family history of psoriasis, axial involvement, and oligoarthritis. Females had longer duration of psoriasis at presentation. Significant differences were found in clinical variables between males and females according to the age of psoriasis onset. Age of psoriasis onset >40 years (n = 98). Men had had more family of psoriasis history, axial involvement, and oligoarthritis. Females had longer duration of psoriasis at presentation. Age of psoriasis onset <40 years (n = 65). Men had had more axial involvement and oligoarthritis.

Conclusion: As shown, males had more axial and oligoarticular involvement. Females had longer duration of psoriasis at presentation, even when stratified by age of onset.


  OPC0221: A rare presentation of psoriatic arthritis with cutaneous polyarteritis nodosa Top


Akhil Pawan Goel, Anupam Wakhlu, Sourav Pradhan, Prashant Bafna; Department of Rheumatology, King George Medical University, Lucknow, Uttar Pradesh, India

Background: Cutaneous polyarteritis nodosa (CPAN) is a rare form of cutaneous vasculitis that involves small- and medium-sized arteries of dermis and subcutaneous tissue without systemic involvement. Cutaneous vasculitis in psoriatic arthritis is very rare. We report here a case of psoriatic arthritis with CPAN which has not been reported.

Case Report: A 26-year-old male presented with inflammatory polyarthritis involving small and large joints with knee contracture for 2 years; inflammatory back pain for 6 months; and diffuse plaque psoriasis involving scalp, upper limbs, trunk, and lower limbs with dystrophy of fingernails for 3 months. Diagnosis of psoriasis with psoriatic arthritis was made and was started on oral methotrexate, nonsteroidal anti-inflammatory drugs, steroid, salicylic acid ointment, and low-dose oral steroids. Three months later, he presented with multiple punched out skin ulcers involving groin, bilateral thighs, and legs for 1 month. These skin lesions initially appeared as nodules which ruptured to form nonhealing ulcers. Local examination revealed healed plaques and skin ulcers. Systemic examination was unremarkable. Investigations: Complete blood count, liver function test, kidney function test, and random blood sugar were normal; viral markers were negative; myeloperoxidase, PR3 (enzyme-linked immunosorbent assay), and antinuclear antibody (Hep2) were negative; urine examination was normal; nerve conduction studies were normal; X-ray pelvis showed bilateral Grade 4 sacroillitis with bilateral hip joint space narrowing, chest X-ray was normal; ultrasonography abdomen was normal; skin biopsy from the edge of ulcer was consistent with CPAN; skin biopsy of plaque was consistent with psoriasis. Steroids were increased to oral prednisolone equivalent of 1 mg/kg and methotrexate gradually increased to 25 mg/week. The vasculitic ulcers gradually healed. The patient is doing well on full dose of methotrexate and steroids which are gradually being tapered.

Discussion: Psoriatic arthritis can be associated with cutaneous vasculitis having p-ANCA positive, but association with CPAN is very rare. Such combination needs to be treated with increased immunosuppression.

Conclusion: Occurrence of psoriatic arthritis and CPAN appears to be coincidental. Early diagnosis and appropriate treatment is necessary.


  OC0048: Delay in diagnosis of Kawasaki disease is the commonest proximate reason for development of giant coronary artery aneurysms- our experience at Chandigarh, North India Top


Rakesh Kumar, Ankur Kumar Jindal, Avinash Sharma, Anju Gupta, Deepti Suri, Manphool Singhal, Surjit Singh; Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India

Background: Long-term effects of Kawasaki disease (KD) depend primarily on the development of coronary artery abnormalities. Giant coronary aneurysm (GCA) is one of the most severe sequelae in KD. Regression of giant aneurysm is rare. Herein, we review patients with KD who had GCA.

Aims: The aim was to describe the profile of patients with KD who developed GCA from a cohort of KD patients at Advanced Pediatric Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Patients and Methods: Records of all children diagnosed to have KD during 1994–2017 were analyzed. Out of the 680 patients with KD, clinical details of 17 (2.5%) children with GCA were retrieved.

Results and Discussion: Diagnosis of GCA was based on coronary artery diameter ≥8 mm or ≥+10 Z-score. Six of 17 children (boys 13; girls 4) with GCA had incomplete KD. Diagnosis of KD was made at a mean of 17.2 ± 12.2 days of fever. Eight (47%) children were <1 year old. Median age of diagnosis was 18 months (range: 1.5 months to 12 years). Left anterior descending (LAD) coronary artery was affected in 82% followed by right coronary artery in 59% of patients. Multiple GCA >1 were seen in 65% of patients. All patients had received first-line therapy as intravenous immunoglobulin (IVIg). Median day of IVIg administration was 15.5 days. Twelve had received additional therapy with infliximab. Thromboses developed in 4 (23.5%) and the most common coronary affected was LAD. All patients were started on anticoagulation therapy and there were no significant complications related to anticoagulation.

Conclusion: Results of this study suggest that GCA develops more commonly in infants and young children. Delay in diagnosis and consequent administration of IVIg appears to be the commonest proximate cause of the development of GCA.


  OPC0222: A Study of insulin resistance in rheumatoid arthritis Top


Debarun Choudhury, Prasanta Dihingia; Assam Medical College and Hospital, Dibrugarh, Assam, India

Background: Chronic activation of the immune system, as observed in the pathogenesis of rheumatoid arthritis, potentially leads to insulin resistance. Insulin resistance is a major component of several cardiometabolic abnormalities, including the metabolic syndrome, type 2 diabetes mellitus, and cardiovascular disease. The risk of myocardial infarction is more than twice in rheumatoid arthritis cases compared to the general population. Stratifying those at risk could direct therapies for the prevention of significant morbidity and mortality.

Objectives: (1) To study the proportion of insulin resistance in patients with rheumatoid arthritis using the Homeostatic model Assessment-Insulin Resistance model (HOMA-IR) and (2) To correlate the degree of insulin resistance with disease activity.

Methods: This was a case–control study consisting of 102 cases of rheumatoid arthritis diagnosed by the American College of Rheumatology/European League Against Rheumatism 2010 and similar number of age- and sex-matched healthy controls. Insulin resistance was calculated using HOMA-IR model based on fasting glucose and fasting insulin levels. Those having HOMA-IR values <2.5 were considered insulin resistant. HOMA-IR values were correlated to disease activity (using the disease activity score [DAS28]).

Results: Nearly 71.6% rheumatoid arthritis cases were insulin resistant, whereas the same in controls was 21.4%, although the mean body mass index was lower in the former (22.78 ± 2.71 vs. 23.21 ± 3.20). Insulin resistance was more prevalent (69.8%) in those with high disease activity (DAS28 >5.1).

Conclusion: This study showed that the majority of patients and 1/5th of the apparently healthy population had insulin resistance. There was significant correlation between disease activity and insulin resistance.


  OPC0223: B-cell therapy in cyclophosphamide-failed antineutrophil cytoplasmic antibody-associated vasculitis: A single-center prospective observational study Top


Durgarao, Gayatri Ekbote, Natasha Negalur, Dhiren Raval, Dhaval Tanna, Shruti Bajad, Rajiva Gupta; ???, Medanta-The Medicity, Gurgaon, Haryana, India

Aim: In antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), the disease activity correlates with increased circulating B-cells. Rituximab (Rtx) is shown to be useful in depleting these B-cells. According to the RAVE1 study, Rtx was shown to be superior to cyclophosphamide in refractory/relapsed AAV cases. Hence, we prospectively analyzed the effectiveness and safety of Rtx in relapsed/refractory disease in our cohort of AAV.

Methods: In this prospective study, there were 67 patients of AAV, diagnosed by clinical and serological criteria (by both line immunoassays and immunofluorescence assay). Follow-up was 2–60 months (median – 24 months) since Rtx was started. Patients were seen from August 2012 to July 2018. A total of 21 patients received Rtx for various reasons. This study was approved by the Institutional Review Board (MICR-546/2015).

Results and Conclusion: Twenty patients had anti-PR3 positivity, while one was antimyeloperoxidase positive. All had granulomatosis-with-polyangitis as per combined hepatocellular-cholangiocarcinoma. Mean age was 47.7 years. All patients had received cyclophosphamide and glucocorticoids. Most patients received Rtx for lung involvement, i.e., 52.38%, while the second commonest indication was ophthalmic involvement followed by renal (23.8 and 14.28%, respectively), and one patient each received Rtx for ear, nose and throat and nervous system involvement. Median dose of steroid (mg/day) (standard deviation) at the start of Rtx induction was 50 (11.54), while at first maintenance, it was 2.5 mg daily. Outcome with Rtx treatment as per European League Against Rheumatism stages is as follows: Hence, in our experience, Rtx is a good induction and maintenance strategy for a dreadful disease like AAV.


  OPC0224: Subclinical atherosclerosis in Indian patients with scleroderma and its clinical correlates Top


G Harikrishnan, Durga Prasanna Misra, Neeraj Jain1, Namita Mohindra1, Sudeep Kumar2, Narayan Prasad3, Mantabya Singh3, Harshit Singh, Saurabh Chaturvedi, Vikas Agarwal; Departments of Clinical Immunology, 1Radiodiagnosis, 2Cardiology and 3Nephrology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Cardiovascular disease (CVD) is a leading cause of mortality in scleroderma systemic sclerosis (SSc); however, Indian data are sparse. We studied subclinical atherosclerosis in Indian patients with scleroderma.

Methods: Patients with scleroderma (n = 61) attending wards/outpatient department at our hospital were recruited; carotid intima-medial thickness (CIMT) was performed by carotid ultrasonography, along with clinical assessment and traditional risk factor evaluation. Ten-year risk of CVD was calculated using the Joint British Societies 3 (JBS3) risk calculator. Controls with diabetes and hypertension (n = 62) were used for comparison. Subgroup analyses compared parameters between SSc with and without carotid plaque. Means were compared with unpaired t-test, whereas medians were compared using Mann–Whitney U-test. Ethical approval and written informed consent were obtained.

Results: Mean age of patients was 37.8 ± 11.9 years (female:male = 50:11; one each had hypertension and diabetes). Median (interquartile range) disease duration (from Raynaud's onset) was 6 (3–10) years, modified Rodnan Skin Score was 12 (5.5–18), body mass index (BMI) was 19.48 (16.63–23.26) kg/m2, and waist–hip ratio was 0.84 (0.79–0.89). Compared to a high-risk control group, SSc had higher mean CIMT (0.68 ± 0.10 vs. 0.54 ± 0.03 mm in controls, P < 0.05). JBS3 risk score could be calculated only in 22 patients (cannot be calculated with age <30 years or BMI < 20); JBS3 risk for CVD in the next 10 years was 1.65 % (0.69–2.85). About 13/61 (21.3%) patients had carotid plaques. Patients with and without plaques [Table 1] were similar for clinical parameters, except for gender distribution.



Conclusion: We noted an unusually high prevalence of plaques in about a fifth of patients with scleroderma, who also had higher subclinical atherosclerosis than a high-risk control population, despite lacking traditional risk factors. Endothelial dysfunction, a key initiating pathogenic event, might drive atherosclerosis in scleroderma.

Acknowledgments: This study was supported by Indian Rheumatology Association Research Grant to DP Misra.


  OC0033: Clinical profile and outcome of myositis: 5 yr experience from a tertiary care centre in south india Top


Anuja Rajan, Selvakumar Balameena, R. Ramesh, Mythili, M. Rajavel, Sreedevi S.; Institute of Rheumatology, Madras Medical College and RGGGH, Chennai, Tamil Nadu, India

Background: The idiopathic inflammatory myopathies are a group of systemic diseases which affect primarily the muscle and skin but may also affect other organs. Since it is an uncommon disease, studies regarding outcome of these patients are lacking in our population.

Objective: The objective of this study is to elucidate the clinical profile, response to therapy, and outcome of patients with idiopathic inflammatory myositis (IIM), admitted in our department.

Materials and Methods: We retrospectively reviewed the medical records of patients with IIM who were admitted at the Institute of Rheumatology, MMC, Chennai, Tamil Nadu, India, from 2013 to 2018. The study group included patients with polymyositis, dermatomyositis (DM), amyopathic DM, and juvenile DM (JDM). Patients with inclusion body myositis and overlap syndromes were excluded from the study. These patients were assessed again at the outpatient clinic. The disease activity and outcomes were assessed using the core set measures developed by the International Myositis Assessment and Clinical Studies Group. The response to therapy was assessed using 2016 American College of Rheumatology/European League Against Rheumatism Criteria for clinical response in adult DM/PM and JDM patients.

Results: A total of 41 patients with IIM were identified, among whom 32 patients were selected for detailed analysis. The mean age of adult patients was 35.8 years; among them, interstitial lung disease was the most frequent extramuscular manifestation, with usual interstitial pneumonia being the most common pattern of involvement. Nearly 56% of patients showed major response to treatment and 18% patients had moderate response to treatment. 9% of patients had refractory disease, 15% (5) of patients died due to complications from the disease, and 18% of adult IIM patients had complete clinical response. Clinical remission was attained by 2 of 5 patients with JDM. 45% of the adult patients who survived had no functional disability at follow-up.

Conclusion: The outcome of IIM patients in our cohort of patients is good. The patients who survived the illness has a high chance of reaching stable disease status.


  OPC0163: A case series of pediatric-onset Behçet's disease from a tertiary-care centre from North India Top


Anjani Gummadi,Pandiarajan Vignesh, Rakesh Kumar Pilania, Avinash Sharma, Deepti Suri,Anju Gupta, Biman Saikia, Ranjana W.Minz, Amit Rawat, Surjit Singh; Postgraduate Institute of Medical Education and Research, Chandigarh, India

Case Series and Discussion: Case records of children with Paediatric Behçet's Disease (PED-BD) (1998–2017) were retrieved from the clinic files of the Pediatric Rheumatology Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India. The retrospective analysis was done, and the parameters including age of onset, sex, clinical manifestations, inflammatory parameters, and treatment outcomes were analyzed. Presence of human leukocyte antigen (HLA)-B51 was assessed by polymerase chain reaction (PCR) in all patients. The diagnosis of BD was based on the diagnostic criteria from the Behçet Syndrome Research Committee of Japan (1987 Revision). Thirteen children had suspected PED-BD based on clinical consensus, out of which 10 were classified as BD. Three children had only ocular manifestations (posterior uveitis – 2, panuveitis – 1, and retinal vasculitis – 3) and positive HLA-B51 by PCR. Mean age of onset of clinical manifestations and diagnosis of BD was 5.1 and 7.7 years, respectively. Male:female ratio was 4:1. Recurrent oral ulcers was the commonest manifestation (9/10). Uveitis was the initial and presenting manifestation in one of the patients (10%). Genital ulcers were seen in four children and skin manifestations were noted in four children. Positive pathergy test was observed in four children (40%). Inflammatory colitis was seen in three children. Uveitis was noted in three children and all were boys. Ocular manifestations noted were retinal vasculitis, posterior uveitis, and panuveitis. Arthritis was noted in three children. HLA-B51 was positive in four children. One child died due to pulmonary hemorrhage. Thrombotic manifestations were not seen in our cohort.

Conclusion: We describe the first case series of PED-BD from India. A significantly higher proportion of male children (80%) was noted in our cohort. Arterial stenotic lesions mimicking Takayasu's arteritis, though well reported in adults, is described for the first time in a cohort of PED-BD.


  OPC0166: Gastrointestinal manifestations as the forerunner of Kawasaki disease Top


Anjani Gummadi,Ankita Singh, Ankur Kumar Jindal, Rakesh Kumar Pilania, Aman Gupta, Deepti Suri, Amit Rawat, Surjit Singh; Postgraduate Institute of Medical Education and Research, Chandigarh, India

Introduction: Kawasaki disease (KD) is the most common vasculitis in children. Gastrointestinal symptoms can occasionally be the forerunner of KD and may pose a diagnostic challenge to the treating physicians.

Aim: The aim was to report children diagnosed with KD who had predominant gastrointestinal manifestations.

Patients and Methods: In this retrospective review of all cases diagnosed with KD at a tertiary care center in Chandigarh, we identified cases who had predominant gastrointestinal manifestations.

Results: A total of 680 cases of KD were diagnosed during 1994–2017. Five among these had a predominant gastrointestinal presentation. All were boys with a mean age at diagnosis of 4.1 years. Case 1 was a 3-year-old boy with fever and upper gastrointestinal bleed due to a duodenal ulcer. Case 2 was an 18-month-old boy who presented with fever and acute intestinal obstruction. He underwent surgery with a clinical suspicion of intussusception and biopsy of the excised specimen from colon suggested vasculitis. Case 3 was an 8-year-old boy who presented with fever, right iliac fossa mass, and intestinal obstruction. Case 4 was a 6-year-boy who presented with fever, acute gastroenteritis, and a palpable mass in the left hypochondrium. Case 5 was a 3-year-old boy who presented with fever, pain abdomen, diarrhea, and blood in stool. Colonoscopy revealed multiple ulcers over the colon. Intravenous immunoglobulin was given to all the five patients and this resulted in rapid improvement in fever and gastrointestinal symptoms. Only one patient (case 3) had intravenous immunoglobulin (IVIg) resistance and required a repeat dose of IVIg. No patient developed coronary artery abnormality. There was a mean delay of 15.6 days in the diagnosis of KD.

Conclusion: KD may occasionally present with variable and predominant gastrointestinal manifestations and this may lead to avoidable delays in diagnosis. A careful assessment for other signs of KD is crucial for early diagnosis.


  OPC0165: Classifying ANCA associated vasculitis & correlating outcome based on anti-PR3/MPO serology: a prospective study from a tertiary care centre Top


Gayatri Ekbote, Natasha Negalur, Dhaval Tanna, Durga Rao, Rajiva Gupta; Medanta-The Medicity Hospital, Gurgaon, Haryana, India

Introduction: Diagnosis and management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a challenge for the internists, pulmonologists, nephrologists, and rheumatologists alike. Clinical overlap with infections and other rheumatic disorders further complicates the picture. Clinical phenotype may not always fit in with combined hepatocellular-cholangiocarcinoma designated disease subtypes, and discordance between autoantibody profile based on munofluorescence assay (IFA) and enzyme-linked immunosorbent assay (ELISA) adds to the confusion. European genome-wide association studies have firmly established the strongest association of various genetic polymorphisms to be related with ANCA specificity rather than the clinically defined syndromes. We aimed to classify clinical features and outcome of proven AAV according to their serology namely anti-PR3/myeloperoxidase(MPO) by ELISA. We hypothesize that this classification is better and easier.

Materials and Methods: This prospective observational study which was approved by the Institutional Review Board and Ethics committee included all consequent (old and new) AAV patients visiting a tertiary care center in northern part of India from August 2012 to June 2018. Data on demographics, clinical features, and laboratory variables were collected. Patients were followed up for a minimum of 6 months. ANCA was done by both IFA and ELISA. Patients with ELISA positivity were included in the study.

Results: Out of 66 patients included, 61 were followed up for at least 6 months [Table 1]. PR3 serology cohort had more relapses, while percentage mortality was significantly higher in MPO cohort. While nodular and cavitating lesions were more often found in PR3-AAV, diffuse alveolar hemorrhage was seen in both groups and it was statistically insignificant (PR3 = 20.75% vs. MPO = 30.76%, P = 0.44). Chronic kidney disease was similar in both groups (PR3 = 7.54% vs. MPO = 7.69%). Acute kidney injury/glomerulonephritis were statistically insignificant (PR3 = 47.16% vs. MPO = 30.76%, P = 0.288). Ear, nose, and throat involvement was significantly higher in PR3 group. Kidney biopsy findings were similar in both groups [Table 1].



Conclusion: Autoantibody-based classification is complementary/superior to clinical segregation of AAV phenotypes and better reflective of short-term outcomes.


  OPC0167: Prevalence and Association of Renal Impairment, Renal Cysts and Urinary Stones in Gout Top


Urmila Dhakad, Danveer Bhadu, Bhupendra Pal Singh, Siddharth Kumar Das; King George's Medical University, Lucknow, Uttar Pradesh, India

Background: Renal impairment, renal cysts, and urinary stones have been reported in gout patients with higher prevalence as compared to normal population. However, data on their complex relationship in gout patients are sparse in the published literature.

Objective: This study aims to elucidate the prevalence and relationship among renal impairment, renal cysts, and urinary stones in gout patients.

Methods: In this prospective study on 107 patients with gout, clinical assessment, body mass index (BMI), comorbidities, dietary habits, urine microscopy, serum uric acid, and renal functions were assessed. Ultrasonography for urinary stones, renal cysts, and urinary system was done. Data were analyzed using unpaired t-test and Chi-square test. P < 0.05 was considered statistically significant.

Results: Mean age, BMI, and male/female ratio were 49.2 ± 5.4, 26 ± 5.4, and 97/10 (90.7%/9.3%), respectively. Nearly 57% patients had comorbidities (dyslipidemia/hypertension/dermatomyositis/coronary artery disease). Urinary stones, renal cysts, and renal impairment (estimated glomerular filtration rate <60 ml/min/1.73 m2) were seen in 24.2% (26/107), 19.6% (21/107), and 28% (30/107) of patients, respectively. Stones alone, cysts alone, and both cyst and stones were seen in 18/107 (16.8%), 13/107 (12.1%), and 8/107 (7.4%) cases, respectively. Presence of renal cysts was not associated with the presence of stones (P = 0.153). Serum uric acid level was significantly higher in patients with renal impairment as compared to those without impaired renal function (10.78 ± 1.67 vs. 9.3 ± 1.65, P < 0.0001). Occurrence of stones was related to serum uric acid levels and duration of gout (10.38 ± 0.72 vs. 9.56 ± 1.94, P = 0.038; and 17.6 ± 10.76 vs. 7.0 ± 7.01, P < 0.0001). Prevalence of renal stones and duration of gout were significantly higher in patients with renal impairment as compared to those without renal impairment (50% vs. 12.5%, P = 0.0011 and 14.3 ± 6.05 vs. 7.68 ± 9.44, P = 0.0006, respectively).

Conclusions: Renal impairment and urinary stones are significant health problems in gout patients. Urinary stones and longer duration of gout are associated with renal impairment in these patients.


  OPC0168: Reproductive health in systemic lupus erythematosus Top


Nibha Jain, Dhaiwat Shukla, Sapan Pandya, Puja Srivastava; Sheth V S Hospital, Smt. NHL Municipal Medical College, Ahmedabad, Gujarat, India

Introduction: Our aim is to study menstrual irregularities in lupus patients and factors associated with sustained/secondary amenorrhea.

Methods: This was a prospective observational case–control study. Study period was 10 months (November 2017–August 2018). All patients attending the outpatient department of a government hospital were enrolled after informed consent. Baseline data were collected along with rheumatoid arthritis patients as disease controls. For healthy controls, the nursing staff was interviewed. Hormonal level including follicle-stimulating hormone/luteinizing hormone (LH/FSH) estimation was done for sustained amenorrhea (>12 months).

Results: A total of 82 patients of systemic lupus erythematosus (SLE) with disease controls rheumatoid arthritis (RA) = 87 and healthy controls (HCs) = 41. Lupus patients were younger as compared to RA (mean age: 32 [standard deviation (SD) 11] vs. 41 [SD 9] P = 0.001). Lupus patients had more oligomenorrhea or temporary amenorrhea as compared to RA patients (35% vs. 16% [P = 0.004]) and HCs (35% vs. 0 [P = 0.001]) and more secondary infertility [Table 1]. Both lupus and RA patients had more sustained amenorrhea (>12 months), which was significant when compared to HC (P = 0.002). On comparing patients of lupus and RA with sustained amenorrhea, although onset in lupus patients was earlier (35 [SD 5] years vs. 42 [SD 6] years, P = 0.007), none had FSH >40 [Table 2]. Factors associated with sustained amenorrhea in lupus included early onset of SLE (19.4 [SD5.2] years vs. 24.4 [SD6.2] years, P = 0.006) with higher Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score (12.5 [SD 4] vs. 5.6 [SD 5.4], P < 0.001) [Table 3].



Conclusion: More of lupus patients had oligomenorrhea or temporary amenorrhea and secondary infertility as compared to RA patients and HCs. SLE patients with sustained amenorrhea (>12 months) did not have hormonal ovarian failure as compared to RA patients who had true menopause. Early onset of lupus with high SLEDAI score is associated with sustained amenorrhea.


  OPC0225: Skeletal fluorosis mimicking seronegative spondyloarthritis Top


Manaswi Chaubey, Sharad Dev; Department of Medicine, IMS, BHU, Varanasi, Uttar Pradesh, India

Introduction: Skeletal fluorosis is endemic in many districts of India including Jharkhand, Chhattisgarh, few areas of Madhya Pradesh, Uttar Pradesh, Bihar, and southern districts of Rajasthan. The early symptoms of skeletal fluorosis are arthralgia and backache due to bone pain.  Achilles tendinitis More Details, early-morning stiffness, and mild increase in acute-phase reactants have also been reported. These findings can mislead to a diagnosis of inflammatory arthritis. However, definite signs of synovitis and synovial hypertrophy are not seen in these patients. It mimics seronegative spondyloarthritis but can also manifest as seronegative symmetric polyarthralgia, thus mimicking rheumatoid arthritis.

Case Report: A 44-year-old female with joint pains was referred from Chatra district of Chhattisgarh. Her complaints were pain in shoulders, knees, and heel region for 6 months. On examination, she had tender shoulders, peripatellar enthesitis, and Achilles tendinitis without any joint swelling. Investigations showed raised acute-phase reactants (erythrocyte sedimentation rate and C-reactive protein). Serum rheumatoid arthritis factor and anti-cyclic citrullinated peptide2 tests were negative. Based on these findings, a provisional diagnosis of seronegative spondyloarthritis was thought. Careful revised examination showed certain changes in the enamel with attrition of the teeth. X-ray images of the forearm, lumbosacral spine, and pelvis showed interosseous membrane calcification, and osteosclerosis of cortical bones, with prominent granular trabeculae and normal sacroiliac joints. Based on these findings, the diagnosis of skeletal fluorosis that mimicked seronegative spondyloarthritis was concluded. The level of fluoride in ground water in these districts has been reported to be high, 1–4 ppm. The patient lived in the region from birth and used well water for drinking. Thus, the source of excess fluoride intake was ground water.

Conclusion: Awareness of the above clinical, radiographic, and dental findings can help physicians and rheumatologists from the endemic areas to make early and accurate diagnosis of fluorosis, thus preventing unnecessary workup and treatment for inflammatory polyarthritis in these patients.


  OPC0226: Kawasaki disease in children more than 10 years: A single-center experience from Chandigarh, North India Top


Rankesh Kumar Pilania, Ankur Kumar Jindal, Sandesh Guleria, Deepti Suri, Anju Gupta, Amit Rawat, Manphool Singhal, Surjit Singh; PGIMER, Chandigarh, India

Background: Kawasaki disease (KD) is arguably the commonest childhood vasculitis. It usually affects children aged below 5. Diagnosis in older children and adolescents is difficult because KD is rarely considered in the differential diagnosis of fever in this age group. As the diagnosis of KD usually gets delayed in these children, there is higher risk of development of coronary artery abnormalities (CAAs).

Aims: The aim was to describe the profile of KD in children aged >10 years from a cohort of patients at Pediatric Rheumatology Clinic, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Patients and Methods: This was a review of records of patients with KD registered during 1994–2017. Case files of children with KD and age >10 years were retrieved and clinical details were analyzed.

Results: During 1994–2017, 680 children were diagnosed to have KD. Thirty-seven (5.4%) children were aged above 10 years (M:F 23;14) (median age: 11 years; range: 10–30 years). All patients had fever as the presenting manifestation and median duration of fever was 11 days. KD diagnosis was made at a mean of 13.4 ± 7.6 days after the onset of fever. Seven (19%) children presented with hypotensive shock. First-line treatment in all patients was intravenous immunoglobulin (IVIg) therapy. Six patients did not receive treatment because they presented late in convalescent phase when the fever had subsided and acute-phase parameters had normalized. IVIg-resistant KD was seen in four patients. Second-line therapy was given as follows: three infliximab and one glucocorticoids. CAAs were seen in three (8.1%) children, one of these being a giant aneurysm. Two patients had severe myocardial dysfunction secondary to myocarditis.

Discussion and Conclusion: Diagnosis of KD in older children is often missed initially and as a result, treatment may be delayed. It is, therefore, not uncommon to see the development of CAA in this group.


  OC0036: Bleeding manifestations in children with lupus: report of two cases with lupus anticoagulant-hypoprothrombinemia syndrome and review of literature Top


Rakesh Kumar, Ankur Kumar Jindal, Avinash Sharma, Deepti Suri, Amit Rawat, Anju Gupta, Jasmina Ahluwalia, Surjit Singh; PGIMER, Chandigarh, India

Objectives: We report the case of two children with systemic lupus erythematosus (SLE) having severe bleeding manifestations and lupus anticoagulant-hypoprothrombinaemia syndrome (LAHPS) along with a review of published cases of childhood SLE and LAHPS.

Methods: We report the clinical and laboratory profile of two children diagnosed with childhood SLE and LAHPS.

Results: An 8-year-old girl presented with fever, arthralgia, alopecia, anasarca, and bleeding from multiple sites. She was diagnosed to have SLE based on laboratory investigations which showed anemia, thrombocytopenia, low complements, and positive anti-nuclear antibody. She was also found to have prolonged prothrombin time (PT) and activated partial thromboplastin time (aPTT), positive lupus anticoagulant, and low factor II levels. She was diagnosed to have SLE with LAHPS and treated with intravenous methylprednisolone, intravenous immunoglobulin, and cyclophosphamide with good outcome. Patient 2 was a 7-year-old boy who was diagnosed to have SLE when he presented with fever, anasarca, malar rash, arthritis, and bleeding from skin and mucosa. Laboratory investigations revealed anemia, proteinuria, low complements, and positive antinuclear antibody and dsDNA titers. Coagulation studies showed deranged PT and aPTT, positive lupus anticoagulant (LA), and low factor II levels. He was diagnosed to have SLE with LAHPS syndrome and was treated with intravenous methylprednisolone and oral mycophenolate mofetil. Review of literature of cases with childhood SLE and LAHPS showed that 32 cases have been reported till date which have been summarized.

Conclusion: LAHPS is an uncommonly identified cause of bleeding in patients with SLE and must be suspected while evaluating these children.


  PC0051: Pattern and profile of psoriatic arthritis in a community-based referral practice over a decade (2007–2017) Top


Jain Amit, Sonees A, Manchanda B, Ghorpade R, Nahar N, Venugopalan A, Chopra A; Center for Rheumatic Diseases, Pune (www.rheumatologyindia.org)

Introduction: Data on psoriatic arthritis (PsA) in the Indian community are sparse. Very few population surveys reported PsA (Chopra A. Ind J Rheum 2015). We decided to study the clinical phenotype of PsA in patients examined in CRD which is a community-based popular rheumatology center.

Methods: This was a cross-sectional retrospective design study of patient records from January 01, 2007, to December 31, 2017. Data were extracted from a comprehensive referral database in CRD, maintained since 1996. Data on first examination were considered.

Results: PsA was classified in 0.9% (male:female ratio = 12:1) of the total patient records (n = 58439). The mean age was 45.1 years (range: 14–97) and the mean duration of disease was 6 years (range: 15 days–45 years). Nearly 14.8% of patients used tobacco. The phenotypic pattern seen were as follows: 20% oligoarticular, 58% polyarticular, 7.2% axial only, and 14.7% axial plus peripheral arthritis. Substantial desquamative interstitial pneumonia arthritis was evident in 18% of patients. The following comorbidities were seen: hypertension in 59.7%, diabetes in 32.3%, hypothyroidism in 12.2%, ischemic heart disease in 6.4%, and dyslipidemia in 15.82. Over 80% had plaque skin psoriasis (diffuse lesion in <30%). Psoriasis of scalp and other sanctuary sites were often missed by referring doctors. Comorbidities were mostly associated with peripheral arthritis; only 10.1% patients with spondyloarthritis (axial) showed comorbidities. Further data on nail disease/DIP disease and ischemic heart disease. and hyperlipidemia, serum (rheumatoid factor and anti-cyclic citrullinated peptide), and other laboratory parameters (erythrocyte sedimentation rate and serum uric acid) were presented.

Conclusion: There is a substantial burden of PsA in routine rheumatology practice and patients show a wide phenotype spectrum. Psoriatic patients need to screened for cardiovascular morbidity and metabolic disorders.


  OPC0227: Recurrent Kawasaki disease: Experience from a tertiary care center at Chandigarh, North India Top


Nameirakpam Johnson, Sandesh Guleria, Rakesh Kumar Pilania, Ankur Kumar Jindal, Aman Gupta, Dharmagat Bhattarai, Vignesh Pandiarajan, Deepti Suri, Amit Rawat, Surjit Singh; Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: Kawasaki disease (KD) is emerging as the most common medium-vessel vasculitis in children. Recurrence of KD (0.8%–3%) is unusual and not commonly reported.

Objective: To study the clinicolaboratory profile of children with recurrent KD.

Methods: Case records of 680 children of KD, registered from 1994 to 2017 in the Pediatric Rheumatology Clinic at the Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India, were reviewed, and data were analyzed in children who had recurrent KD.

Results and Discussion: Six (0.88%) children (4 boys; 2 girls) had recurrent KD. Mean age at diagnosis was 4.7 years (range, 2–9 years). Five (83.3%) had recurrence of KD within 1.5 years and among these, 4 (66.7%) had recurrence within 10 months of the first episode (range, 0.17–4.5 years). During the first episode of KD, fever (100%), oral mucosal involvement (100%), desquamation (83.3%), and rash (66.6%) were the common clinical features. While, during recurrence, fever (100%), desquamation (66.7%), oral mucosal involvement (66.6%), and rash (33.3%) were prominent clinical features and four children had desquamation in the 1st week of illness. Incomplete KD was more common during recurrence (83.3%) as compared to the first episode (33.3%). Laboratory investigations did not show any significant difference during both episodes of KD. A total of 5/6 children in the first episode received treatment with intravenous immunoglobulin (IVIg). One child had reported late and inflammation had subsided and he was not given IVIg. A total of 4/6 children received IVIg during recurrence. In two children, fever and inflammation had subsided when they presented. One child had left main coronary artery ectasia during the first episode of KD as well as during recurrence and it normalized after treatment with IVIg on both occasions.

Conclusion: Recurrences in KD are unusual. Recurrent episode is more often an incomplete form of KD with few or mild symptoms and early desquamation.


  OPC0312: Foot arthritis as an indicator of smoldering disease activity in rheumatoid arthritis Top


Sandeep Kansurkar, Vivek Kumar Saini, Namita Mohindra, Neeraj Jain, Durga Prasanna Misra, Amitabh Arya, Vikas Agarwal; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Introduction: In rheumatoid arthritis (RA), most widely used disease activity scores such as Disease Activity Score (DAS28) and Clinical Disease Activity Index do not include foot. We studied foot arthritis by clinical examination, ultrasonography, and scintigraphy to compare with disease activity scores.

Methods: Clinical examination including foot was performed in 57 cases of RA. Disease activity was measured using DAS28 and 44 and 66 joint scores. Remission was defined as DAS28 <2.6. Foot Joint Score was calculated by adding the number of involved joints of feet (ankle, subtalar, midfoot, metatarsaophalangeal joints [MTPs], and proximal interphalangeal joints) and compared with disease activity markers. Skeletal scintigraphy with 1-methyl-4-(4-diethylaminophenylazo) pyridinium iodide (MDP) was performed in thirty cases. Scintigraphy total joint score and foot scores were calculated as per the number of joints showing the uptake of MDP dye. These scores were compared with disease activity markers. Ultrasonography of foot was performed in thirty cases and score was calculated by OMERACT ultrasound scoring of synovitis in MTP joints of feet. This score was correlated with disease activity markers and scintigraphy total and foot joint scores.

Results: In 56 cases, 47 had active and 9 were in remission. In active group 35 (74%) and remission group 3 (33%) had involvement of foot joints. There was significant correlation between DAS28, 44 joint score, and 66 joint score with Foot Joint Score (P < 0.005, r = 0.68, 0.44, and 0.47, respectively). Out of disease activity markers, only 66 joint score had significant correlation (P < 0.05, r = 0.7) with scintigraphy total joint score. Foot Ultrasonography Score had good correlation with all disease activity markers. Among cases in remission, all had ultrasonographic evidence of synovitis in foot. There was poor correlation between scintigraphy and ultrasonography.

Conclusion: Foot arthritis although not included in DAS28 is an independent marker of disease activity. A third of patients in remission can have smoldering foot arthritis which can be easily detected by ultrasonography.


  OPC0172: Shock in a febrile child: Beware of Kawasaki disease Top


Nameirakpam Johnson, Rakesh Kumar, Ankur Kumar Jindal, Vamshi Srikrishna, Sandesh Guleria, Manphool Singhal1, Surjit Singh; Department of Paediatrics, Advanced Paediatrics Centre, Allergy Immunology Unit, Postgraduate Institute of Medical Education and Research, 1Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: Kawasaki disease (KD) is an acute febrile illness of young children. Shock at presentation is unusual and results in diagnostic confusion.

Case Reports: Case 1 – An 8-year-boy presented with fever of 5 days, headache, maculopapaular rash, conjunctival injection, red oral mucosa, and bleeding from the lips. He had shock at admission. Investigations revealed elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Initial clinical possibility was toxic shock syndrome (TSS) for which he received intravenous antimicrobials. He developed edema over the dorsum of hands/feet and periungual peeling. Blood culture was sterile. N-terminal-pro-B-type natriuretic peptide (NT-pro-BNP) was 499.6 pg/ml. Based on these clinical findings, a diagnosis of KD shock syndrome was considered. He received 2 g/kg intravenous immunoglobulin (IVIg). Two-dimensional echocardiography (2DE) showed dilatation of the left main coronary artery (3.9 mm; +2.9 Z), which was confirmed on computed tomography coronary angiography. In view of the severity of disease with ectatic coronary artery, infliximab (5 mg/kg) was also administered. Follow-up echocardiography at 6 weeks was normal. Case 2 – A 11-year-boy presented with fever of 6 days, headache, maculopapular rash, and conjunctival injection. At admission, he had hypotensive shock requiring vasopressor support. Systemic examination was unremarkable. Clinical possibilities included TSS and KD. He was initiated on antimicrobials. Investigations revealed anemia, neutrophilic leukocytosis, thrombocytosis, elevated CRP, and ESR. Blood cultures were sterile. NT-pro-BNP was 9435 pg/ml. 2DE revealed normal coronaries with ejection fraction of 30%. He received IVIg (2 g/kg) and showed significant hemodynamic improvement. Repeat echocardiography showed normalization of ejection fraction.

Discussion: Clinical presentation of both patients was similar to bacterial sepsis. Both patients had anemia, neutrophilic leukocytosis, elevated CRP, thrombocytopenia, and low albumin.

Conclusion: All children presenting with fever and shock must be carefully evaluated for KD and require echocardiography examination.


  PC0054: A case of polyarthritis with deformity- Is it always a CTD? Top


Saurabh Tyagi; Dr. Ram Manohar Lohia Hospital, New Delhi, India

Case Report: A 53-year-old male presented with complaints of polyarthritis with deformities involving small joints of both hand and feet associated with numbness for 5 years with no morning stiffness. He also complained of multiple nonhealing ulcers in the right lower limb for 6 months. On examination, flexion deformities at proximal interphalangeal joint, desquamative interstitial pneumonia, and intertarsal joints were present in the upper and lower limbs, respectively. In lower limbs, deformities were associated with multiple discharging sinus and ulcerations.

Rheumatoid factor, anti-cyclic citrullinated peptide, antinuclear antibody, dsDNA, Venereal Disease Research Laboratory, and treponema pallidum hemagglutination were negative, and complete blood count, liver function test, and kidney function test were normal, while erythrocyte sedimentation rate was 28 and C-reactive protein was 10.5. Ulcer swab was negative for cultures. Venous and arterial Doppler was normal. Radiograph of the right foot showed osteopenia with resorption of metatarsals. Hence, a provisional diagnosis of vasculitis or arthritis mutilans was made. He was started on antibiotics and antifungal, but showed no response. Meanwhile, nerve conduction velocity revealed sensory neuropathy in all the four limbs. Nerve biopsy was suggestive of leprosy. After starting him on anti-leprotic treatment, ulcers started to heal in 2 months. He has been kept in follow-up.

Discussion: Leprosy is a rare, chronic granulomatous infection of the skin and peripheral nerves caused by Mycobacterium leprae. Leprosy as arthritis can manifest both as a complication and a comorbid disorder and can be a challenging differential diagnosis in rheumatology practice due to several common features. Uncommonly, it may present as acute severe polyarthritis with skin lesions.

Conclusion: Presentation of Hansen disease (HD) as a chronic polyarthritis and deformity is unusual. This case shows that HD can present without skin manifestations but with severe disabling deforming arthritis (Charcot joints).


  OPC0174: Rare case of xanthoma disseminatum with inflammatory polyarthritis Top


Suvarna shilpa S, Sarath Chandra Mouli V, Abitha Aliyar, Anand Kumar V; Krishna Institute of Medical Sciences, Hyderabad, Telangana, India

Introduction: A 55-year-old man presented with a 2-year history of skin lesions in the form of asymptomatic small yellowish papules distributed predominantly over the trunk with few arranged like annular lesions. Nails and mucosa were normal. For the last 1 year, he developed pain and swelling of multiple joints involving bilateral proximal interphalangeal joints and metacarpophalangeal joints of hands, wrists, elbows, shoulders, and knees, with early-morning stiffness of about 10–15 min. On examination, there was symmetrical peripheral polyarthritis and the skin lesions as mentioned. Routine laboratory workup (hemogram and renal and liver functions), thyroid function tests, blood sugars, and fasting lipid profile were normal. Rheumatoid factor, anti-cyclic citrullinated peptide, and antinuclear antibody immunofluorescence were negative. Serum calcium and angiotensin-converting enzyme levels were normal. X-ray of the chest and the involved joints was normal. Skin biopsy showed loose collection of histiocytes with admixed lymphocytes, with occasional Tuton giant cells. Immunohistochemistry showed CD1a negativity and CD68-positive histiocytes with occasional histiocyte showing S-100 positivity; suggestive of xanthoma disseminatum (XD). He was treated with oral steroids (deflazacort 6 mg/day) and methotrexate (MTX) (15 mg/week). In 2 weeks, his skin lesions have completely regressed with resolution of joint pains. Deflazacort was withdrawn over the next month and he is maintained on MTX alone at low dose (15 mg/week).

Discussion: XD is a rare benign nonfamilial Langerhans cell histiocytosis characterized by disseminated xanthomatous lesions. So far, only one case of association of inflammatory arthritis with XD has been reported, in pediatric age group. Our case is an adult patient with XD and polyarthritis who responded to oral steroids and MTX.

Conclusion: It is a rare case of inflammatory arthritis associated with XD that responded to low-dose steroids and MTX.


  OPC0177: An unusual case of abdominal pain and weight loss Top


A Kapadia, A Jafaru, D Sahu, F Rees; Queens Medical Center, Nottingham University Hospital, Nottingham, England

Case Description: We present the case of a 77-year-old Caucasian woman who presented to her general practitioner with a 1-year history of weight loss, left iliac fossa pain, anemia, and raised inflammatory markers with change in bowel habits. She had no headaches or visual symptoms. She was investigated with a computed tomography scan which revealed an ovarian mass. She then went on to have a salpingo oophorectomy, omental biopsy, and appendectomy, which were done as part of a routine staging, for potential diagnosis of malignancy. The histology findings were surprising. The periappendiceal and peritubal tissue of both  Fallopian tube More Detailss showed numerous medium-sized blood vessels with chronic inflammatory cell infiltration with multinucleated giant cells consistent with GCA. This being an unusual site, she had further imaging with a positron emission tomography (PET) scan. This showed intense mural activity within the descending aorta; the brachiocephalic, subclavian, axillary, and proximal brachial arteries; as well as the common iliac arteries and common and superficial femoral arteries. These findings were consistent with an active large-vessel arteritis. She was commenced on high-dose prednisolone, and methotrexate was started as a steroid-sparing agent. Despite normalization of inflammatory markers, a repeat PET scan showed residual uptake. She has now been switched to mycophenolate.

Discussion: This case illustrates the unusual site for GCA. This would not be a routine diagnosis on the list of differentials for a patient with similar symptomatology. A literature search has revealed only one case of GCA in the gastrointestinal tract and four involving the female genital tract. In these cases, systemic involvement developed in the patient with gastrointestinal-tract GCA and in one of the patients with female genital tract involvement.


  OC0050: Autoantibody profile of children with juvenile dermatomyositis from a tertiary care center in North India Top


Dharmagat Bhattarai, Avinash Sharma, Anju Gupta, Sandesh Guleria, Amit Rawat, Deepti Suri, Surjit Singh; ???, Postgraduate Institute of Medical Education and Research, Chandigarh, India

To study the autoantibody profile and to look for phenotypical associations of autoantibodies in juvenile dermatomyositis (JDMS).

Methods: This was a cross-sectional retrospective study. All children diagnosed to have JDM, registered from 1995 to 2016 in Pediatric Rheumatology Clinic and who were tested for autoantibodies, were included in the study. Clinical findings, antinuclear antibodies (ANA), autoantibody for myositis specific autoantibodies, and myositis-associated autoantibodies were noted from case records. Immunoglobulin G antibodies against Jo1, threonyl-tRNA synthetase (PL7), alanyl-tRNA synthetase (PL12), glycyl-tRNA synthetase (EJ), signal recognition particle (SRP), Mi-2, MDA-5, transcriptional intermediary factor 1-γ, Ku, PMScl 100, Scl 70, and SSA/Ro 52 have been done by Immunodot. Evaluation for anti p-140 or nuclear matrix protein (NXP2) and anti-200/100 or 3-hydroxy-3-methyglutaryl-coenzyme (HMG CoA reductase) was done using enzyme-linked immunosorbent assay.

Results: Anti-ANA testing was done in 100 patients. Forty-eight (48%) tested positive. Anti-SRP antibodies were present in 4 (11.4%) children, anti-MDA5 in 3 (8.6%), anti-Mi2 in 1 (2.9%), and 1 patient tested positive for anti-SSA/Ro52 antibodies. All the four children with anti-SRP were girls, had polycyclic course, and two of them developed calcinosis. Patients with anti-MDA5 had predominant skin involvement, less severe muscle disease, and followed a monocyclic course. The only patient with anti-Mi2 had normal muscle strength/endurance at the time of follow-up. None of the patients had antisynthetase antibodies (anti-Jo1, anti-PL-7, anti-PL-12, and anti-EJ), anti-ku, or anti-Scl-70. None of the patients tested positive for anti-NXP2 or anti-HMG CoA.

Conclusion: Prevalence of autoantibodies in children with JDM in our study is similar to what has been described previously. Autoantibodies were tested in children while they were on treatment. This may have resulted in lower positivity. Evaluation of autoantibody profile at the time of diagnosis may assist in predicting the course of disease and response to treatment.


  OC0038: Pediatric systemic lupus erythematosus with Steven Johnson syndrome/toxic epidermal necrolysis: An unusual association Top


Dharmagat Bhattarai, Himanshi Chaudhary, Anju Gupta, Pandiarajan Vignesh, Niteesh Bhardwaj, Surjit Singh; Postgraduate Institute of Medical Education and Research, Chandigarh, India

An 11-year-old girl presented with a history of alopecia, intermittent fever, maculopapular rash progressing into patchy bullous and desquamating lesions, red scaly eyes, and two episodes of generalized tonic-clonic seizures. On examination, she had pallor, edema, oral ulcers, and alopecia. She had distinct atypical targetoid lesions and diffuse erythematous rashes with denuded skin all over the face, trunk, and extremities (>65% body surface area). There were bullae with positive Nikolsy sign. Deep tendon reflexes were brisk. Rest of the systemic examination was unremarkable.

Hemogram showed severe anemia, thrombocytopenia, lymphopenia, and high erythrocyte sedimentation rate. Liver and kidney function tests were normal. Urinalysis showed proteinuria. Antiphospholipid antibody workup was negative. Direct Coombs test was positive. Antinuclear antibody test by immunofluorescence was positive (4+ homogeneous with rim enhancement). Anti-dsDNA antibody test was positive by both enzyme-linked immunosorbent assay and immunofluorescence.

Skin biopsy had shown full-thickness epidermal necrosis. Magnetic resonance imaging brain showed the presence of cerebral atrophy with multiple small infarcts suggestive of vasculitic changes. On the basis of clinical and laboratory findings fitting into the criteria, a diagnosis of Stevens Johnson syndrome/toxic epidermal necrolysis (TEN) masquerading in a patient of systemic lupus erythematosus (SLE) was offered. She was treated with supportive care, intravenous immunoglobulin (IVIG), antimicrobials, anticonvulsants, and pulse methylprednisolone.

Discussion: Co-existence of TEN and SLE is a described phenomenon in literature. Presentation may be heterogeneous and diagnosis may be difficult. In our case, there were clinical features of both diseases which were confirmed through the laboratory investigations. Cause of coexistence of TEN in SLE patient is difficult to associate. IVIG and immunomodulatory agents are used for the treatment.

Conclusion: TEN may be the first presenting feature of in some of the children with SLE. In the presence of suspicious clinical database, no pediatrician should miss such noteworthy co-existence.


  OPC0229: A study of autoantibody profile in systemic lupus erythematosus Top


Alal Uddin, Naman Jain, S Kakati, L Saikia; Assam Medical College, Dibrugarh, Assam, India

Background: Once it has been established that antinuclear antibodies (ANAs) are present, it is important to determine which particular nuclear antigens may be the target of the autoantibodies because some of these antigen-specific responses provide great diagnostic specificity.

Aims and Objectives: To study autoantibody profile in systemic lupus erythematosus (SLE).

Methods: This is a hospital-based observational study carried out on 129 cases of SLE who fulfilled the American College of Rheumatology criteria (1997) attending rheumatology outpatient department, other outpatient departments, or admitted in various wards in the Department of Medicine, Assam Medical College, Dibrugarh, Assam, India, during the period of July 2016–June 2017 in order to know the prevalence of autoantibody profile. In our study, anti-ANA assay was done via immunofluorescent assays and various autoantibody relativities were studied by line immunoassay.

Results: All 129 SLE patients were ANA (immunoflorescent assay) positive (100%). The frequency of different autoantibodies studied by line immunoassay is as follows: anti-dsDNA (58.91%) and is the commonest followed by anti-Ro (60 kDa) (44.9%), anti-histone (38.76%), anti-Ro (52 kDa) (37.98%), anti-SmD1 (27.13%), anti-ribosomal Po (27.13%), antinucleosome antibody (23.26%), anti-U1snRNP (16.28%), anti-La/SS-B (13.95%), anti-proliferating cell nuclear antigen (5.43%), AMA M2 (3.1%), anti-Pm-Scl (3.1%), anti-Mi-2 (1.55%), anti-Scl – 70 (1.55%), anti-Ku (1.55%), and anti-centromere protein B (0.78%). Anti jo-1 was not found in any patient.

Conclusion: As there is a paucity of similar studies, especially in this part of the world, the present study is an attempt to find out the prevalence of various autoantibodies in SLE patients which may aid us to detect other associated connective-tissue disease disorder in SLE patients and prognosticate the disease.


  PC0094: Clinical profile of patients with digital gangrene admitted to Department of Rheumatology IPGMER & SSKM Hospital Kolkata Top


Basil Paul Kunnathu, Deepak Rath, Rudra Prasad Goswami, Geeta Bali Sircar, Parasar Ghosh, Alakendu Ghosh; Department of Rheumatology, IPGMER and SSKM Hospital, Kolkata, West Bengal, India

Background: Digital gangrene is a common problem in routine clinical practice. Rheumatological conditions presenting as gangrene are numerous, but generally underdiagnosed. We are presenting our cohort of patients admitted with digital gangrene between January 2016 and June 2018.

Objectives: To study the clinical profile of patients with digital gangrene admitted to Department of Rheumatology IPGMER&R and SSKM Hospital Kolkata, West Bengal, India.

Methods: Retrospective analysis of case records of patients with digital gangrene admitted to Rheumatology Ward between January 2016 and June 2018.

Results: 52 patients with digital gangrene admitted to our rheumatology ward between January 2016 and June 2018 were studied. There were 33 females and 19 males. The mean age at presentation was 39.94 ± 4.94 years. 10 patients had scleroderma (19.2%), 9 were diagnosed to have antiphospholipid syndrome (17.3%), 9 had SLE (17.3%), 5 were diagnosed to have atherosclerotic vascular disease (9.6%), 4 had Rheumatoid vasculitis (7.6%), 3 MCTD (5.7%), 3 Polyarteritis nodosa (5.7%), 3 had SLE /Systemic sclerosis overlap (5.7%), 2 myeloproliferative disorder (3.8%), 1 Sjogren vasculitis (1.9%) and 1 thromboangitis obliterans (1.9%). Out of the 9 patients with antiphospholipid syndrome, 5 had primary APS (55.5%), 3 had SLE (33.3%) and 1 SLE/systemic sclerosis overlap (11.1%). Out of the 9 patients with SLE, 6 were considered to have lupus vasculitis as the cause of gangrene (66.6%) and 3 (33.3%) were diagnosed to have antiphospholipid syndrome.

Conclusions: In our study, scleroderma has the highest prevalence among the rheumatological causes of digital gangrene, followed by antiphospholipid syndrome and SLE.


  OPC0181: Clinical features and outcomes in microscopic polyangiitis: A single-center experience from a tertiary care hospital in North India Top


Debashish Mishra, Sakshi Mittal, Saket Jha, Aadhaar Dhooria, Manphool Singhal1, Mahesh Prakash1, Ritambhara Nada2, Ranjana Minz3, Manish Rathi4, Kusum Sharma5, G S R S N K Naidu, Varun Dhir, Sanjay Jain, Aman Sharma; Department of Internal Medicine, Clinical Immunology and Rheumatology Services, PGIMER, Departments of 1Radiodiagnosis, 2Histopathology, 3Immunopathology, 4Nephrology and 5Microbiology, PGIMER, Chandigarh, India

Clinical

Background: Microscopic polyangiitis (MPA) is a distinct entity of small-vessel antineutrophil cytoplasmic antibody (ANCA)-related vasculitis. As per Western literature, relapses are more frequent than mortality in this entity; however, there is no Indian data on same. Here is a retrospective analysis of 44 patients with MPA over a period of 15 years.

Objectives: To study the clinical characteristics and outcomes of MPA patients in a tertiary care center in North India from 2003 to 2018.

Methods: This is a retrospective data analysis of all in/outpatients fulfilling American College of Rheumatology 1990 or Chapel Hill definitions of MPA. Patients' clinical features, ANCA status, treatment, and outcomes were recorded from hospital records.

Results: A total of 44 MPA patients (20 males and 24 females) were analyzed, with a mean age of which was 45.09 ± 15.75 years. Of the various systemic involvements, 81.8% had renal and 63.6% had pulmonary involvement. ANCA by indirect immunofluorescence was positive in 79.54%, 72.7% of which was P-ANCA. Of 24 tested samples, 79.17% were antimyeloperoxidase (MPO) positive. Mean BVAS score of the cohort was 18.29 ± 5.11. Nearly 47.73% of the cohort had presenting creatinine of more than 5.7 mg/dl or required hemodialysis, 53.85% had subnephrotic proteinuria, 40.91% received plasma exchange along with immunosuppression, 81.82% patients were induced with cyclophosphamide, and 11.36% were induced with rituximab. Azathioprine was used for remission maintenance in 84.09% of them. Five patients in the cohort died: three due to sepsis, one due to diffuse alveolar hemorrhage, and the cause was unknown in one.

Conclusions: Renal involvement was the most common initial clinical presentation in our cohort, and patients positive for anti-MPO/P-ANCA had severe renal involvement requiring hemodialysis. Early initiation of aggressive immunosuppression with/without plasma exchange had a favorable outcome in our cohort.


  PC0058: An unusual case of inflammatory bowel disease arthritis as a mimicker of adult-onset Still's disease: Diagnostic dilemma and therapeutic challenges Top


Debashish Mishra, Aadhaar Dhooria, Shefali Khanna Sharma, Aman Sharma, Sanjay Jain, Varun Dhir; Department of Internal Medicine, Clinical Immunology and Rheumatology Services, PGIMER, Chandigarh, India

An 18-year-old male was admitted with a history of fever and symmetrical polyarthritis for the last 3 months. He also had abdominal pain for 10 days without any other bowel complaints. On examination, he had lymphadenopathy and nontender hepatomegaly. On evaluation, he had anemia, neutrophilic leukocytosis, thromobocytosis, hypoalbuminemia, and transaminitis. After ruling out infection, occult malignancy and connective-tissue disease, he was diagnosed as adult-onset Still's disease (AOSD) as per Yamaguchi criteria and started on oral prednisolone 0.5 mg/kg with methotrexate 15 mg/week. On follow-up, his fever, lymphadenopathy, and laboratory abnormalities resolved; however, he continued to have synovitis of both knee joints. He was given methotrexate up to 25 mg/week and leflunomide 20 mg along with oral steroids. However, his joint symptoms persisted and progressed. Being nonaffordable for biologics, he was given a trial of thalidomide, to which he showed some improvement. On follow-up, after 4 months, he developed inflammatory low back pain and 4–5 episodes of loose stool per day without any pain or bleeding. On examination, right-sided FABER was positive with bilateral heel enthesistis. X-ray pelvis and both hips showed right-sided sacroilitis. His colonic biopsy showed the presence of cryptitis, crypt abscesses, with intact lining epithelium, suggestive of acute ulcerative colitis. He was continued on thalidomide and sulfasalazine was added. Currently, the patient is ambulatory, with no further synovitis, on 5-mg oral prednisolone, thalidomide, and sulfasalazine.

Discussion: This case highlights a rarity of AOSD preceding inflammatory bowel disease manifestations. Chronic gut inflammation related to axial inflammatory complaints and sacroiliac radiological abnormalities have been described in juvenile idiopathic arthritis. Thalidomide is a potent tumor necrosis factor-α inhibitor, which blocks signaling through nuclear factor kappa B and COX-2, and has T-cell co-stimulatory properties. In financially constrained settings, it can be a cheap alternative to biological disease-modifying antirheumatic drugs.

Conclusion: In cases of refractory AOSD or arthritis, gut inflammation should be looked for.


  OPC0182: Central nervous system vasculitis as a rare presentation of immune reconstitution inflammatory syndrome posttreatment of Cushing's syndrome Top


Debashish Mishra, Sakshi Mittal, Rama Walia1, Chirag Ahuja2, Ritambhara Nada3, Ramandeep Singh4, Anil Bhansali1, Sanjay Jain, Aman Sharma; Department of Internal Medicine, Clinical Immunology and Rheumatology Services, PGIMER, Departments of 1Endocrinology, 2Radio Diagnosis, 3Histopathology and 4Ophthalmology, PGIMER, Chandigarh, India

A 35-year-old male was admitted with bilateral proximal myopathy for 3 months and erectile dysfunction and hyperpigmentation of skin and oral mucosa for 2 months. On investigation, he had hypokalemia with metabolic alkalosis with very high serum cortisol (1750) and adrenocorticotropic hormone (ACTH) (1398) levels. On imaging, positron emission tomography-computed tomography showed mass in the pancreatic distal body and tail. Fine-needle aspiration cytology showed neuroendocrine tumor. Magnetic resonance imaging (MRI) brain showed pituitary microadenoma. He underwent surgical resection of tumor, which on histopathology revealed ACTH-secreting paraganglioma. Postsurgical resection, his ACTH (16) and cortisol (190) levels normalized. Fifteen days later, he got readmitted with a history of two episodes of right-sided transient ischemic attack and right-sided cerebellar symptoms. He also had left-sided painless loss of vision with redness. On evaluation, he was found to have left-eye panuveitis with retinal vasculitis. His routine investigations and cerebrospinal fluid study was all normal. MRI brain showed multiple-site T2 hyperintensities suggestive of acute infarcts, while angiogram revealed beaded appearance with vessel-wall imaging showing inflammation of the wall suggestive of active vasculitis. His workup for other systemic features of vasculitis and antibodies was negative. He was given injection methylprednisolone for 3 days followed by oral steroids and injection cyclophosphamide as steroid-sparing immunosuppressant. His vision which was initially only light perception on the left eye improved to 4/60 on discharge.



Discussion: Many case reports of immune reconstitution presenting as varied autoimmune diseases have been described in literature; however, most of them were associated with pituitary microadenoma. This is first of its kind report where central nervous system and retinal vasculitis with panuveitis has occurred in a patient of Cushing's syndrome on remission.

Conclusion: Aggressive immunosuppression is needed in such rare cases of immune reconstitution inflammatory syndrome to prevent further deterioration.


  OPC0183: Scleroderma-interstitial lung disease: Does maintenance therapy make a difference Top


Shefali K Sharma, Arghya Chattopadhyay, G S R S N K Naidu, Debashish Mishra, Varun Dhir, Sanjay Jain; Department of Internal Medicine, Clinical Immunology and Rheumatology Services, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Aims: Interstitial lung disease (ILD) is the commonest cause of death among scleroderma (Systemic sclerosis [SSc]) patients. Does maintenance therapy make a difference in outcome is not known. We retrospectively analyzed the effect of maintenance therapy following induction with monthly intravenous cyclophosphamide (IV CYC) in SSc-ILD.

Methods: This is a retrospective study. Patients were divided into the following two groups: those who received maintenance therapy with either mycophenolate (MMF) or azathioprine (AZA) and those without maintenance therapy. All were assessed at 3 years. Response to treatment was classified as improved, stable, and worsened based on the forced vital capacity change from baseline. Favorable outcome was defined as an increase in forced vital capacity (FVC) or a fall in FVC, <10% from baseline value and the unfavorable outcome was defined as a fall in FVC ≥10% from baseline value.

Results: Of the 43 patients, maintenance therapy was given in 29 (67.4%) patients; among them, favorable outcome was noted in 23 (79.3%) and the unfavorable outcome was noted in 6 (20.7%) patients. Fourteen (32.6%) patients did not receive maintenance therapy; among them, a favorable outcome was noted in 4 (28.6%) patients and an unfavorable outcome in 10 (71.4%) patients. This difference in the rates of favorable outcome between both the groups was statistically significant (P = 0.001).

Conclusions: Maintenance therapy for up to 3 years (either with AZA or MMF), following IV-CYC induction, resulted in a favorable outcome and helps in preserving the initial beneficial effects achieved with IV-CYC.


  OC0041: Delay at presentation to specialists result in poor functional outcome in Indian patients with Idiopathic Inflammatory myositis. Results from the MyoIn Registry (IRA Myositis Special Interest group) Top


Sravan kumar Appani, Latika Gupta1, Vineeta Shobha2, Sanjiv Amin3, Sangeetha KN2, Ramya Aithala2, Ramnath Misra1, Liza Rajasekhar; Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences (NIMS), Hyderabad, Telangana, 1Department of Clinical Immunology, Sanjay Gandhi Post Graduate Institute of Medical Sciences (SGPGIMS), Lucknow, Uttar Pradesh, 2Department of Clinical Immunology and Rheumatology, St John's Medical College Hospital, Bangalore, Karnataka, 3Consultant Rheumatologist, Mumbai, Maharashtra, India

Background: MYOIN Registry is a collaborative effort by Indian rheumatologists to study idiopathic inflammatory myositis (IIM) across the country.

Methods: Collaborators developed a pro forma, MS Access-based database, and obtained institutional ethical clearance for the registry at each center. Patients with an established physician diagnosis of IIM were recruited between January and July 2018 at four centers. Demographic, clinical, laboratory, and follow-up parameters were collected. Differences in various parameters were explored with respect to functional class at presentation and response at 6 months. Results were presented as median and interquartile range.

Results: A total of 166 patients (128 females) were enrolled. Dermatomyositis (n = 94) followed by overlap myositis (n = 35) was the most common diagnosis. Time to diagnosis from myositis symptom onset was 3 (1–6) months. The clinicolaboratory profile is delineated in Table 1. At presentation, 138 patients were in poor functional class with creatine phosphokinase values of 875.5 (232–3392) IU/ml. Duration of follow-up was 25 months (6.5–58). One mg/kg of steroids followed by methotrexate was the most commonly used treatment. Time to reach daily steroid dose <0.15 mg/kg/d in 68 patients was 12 months (6–22.5). Complete response at 6 months was seen in 38 of 98 patients with follow-up >6 months. Fifty-seven (34.3%) patients had monocyclic course with 126 relapses in 64 patients. At the last follow-up, 46 were in remission with 8 off-treatment. Twenty-two of 91 patients had at least one major infection. Time to diagnosis was significantly longer in functional class 3, 4 (3, 1–6.75) than in functional class 1 or 2 (1, 0–3) (P = 0.018). Occurrence of complete or partial response at 6 months was not related to time to diagnosis (P = 0.67).

Conclusion: Dermatomyositis and overlap myositis are the most frequent subgroups of IIM seen by rheumatologists. Delay in diagnosis results in poor functional class. Remission is frequently achieved. Two thirds relapse. Extent of response is not related with time to diagnosis.


  OPC0184: Interphalangeal joint of thumb involvement in psoriatic arthritis as a distinct entity: a low-field extremity magnetic resonance imaging study Top


Ashish J Mathew, Arvind Ganapati, Jyoti Panwar1, Anu Desai, Varghese Koshy, Debashish Danda; Departments of Clinical Immunology and Rheumatology and 1Radiology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Disproportionate swelling of interphalangeal joints of thumb in psoriatic arthritis (PsA) is described in 28.7% patients, with a positive predictive value of 84%. Our aim was to describe the extremity magnetic resonance imaging (eMRI) variables of inflammation at thumb joints in patients with inflammatory arthritis and to evaluate the association of MRI inflammation at thumb in different types of arthritis.

Methods: Age- and disease duration-matched patients of PsA (classified according to CASPAR), rheumatoid arthritis (RA – fulfilling 1987 American College of Rheumatology classification criteria) patients, and undifferentiated arthritis (undifferentiated arthritis [UA] – seronegative inflammatory arthritis) patients, attending general Rheumatology and Dermatology clinics at Christian Medical College, Vellore, India, over the past 3 years, and who have had imaging of hand using 0.2T Esaote C-scan (noncontrast), were included. Demographic and clinical data were noted from electronic medical records. eMRIs were reported by two rheumatologists trained in OMERACT scoring systems and one musculoskeletal radiologist. Inflammatory variables (synovitis, osteitis, and tenosynovitis) were scored as present or absent at the interphalengeal joint of thumb. Standardized definitions for noncontrast images were used for scoring.

Results: A total of 99 patients (42 PsA, 28 RA, and 29 UA) were included in the study. Table 1 describes the demographic and clinical and proportion of patients with thumb IP inflammation in each group. The reliability of readers, measured using single-measure, two-way random effects absolute agreement intra-class coefficient, was moderate to good. In regression analysis, PsA had 2.7 (95% confidence interval [CI]: 1.16–6.30) and 2.5 (95% CI: 1.15–5.44) times higher risk of developing thumb IP inflammation as compared to RA and UA patients, respectively.

Conclusion: Proportion of MRI inflammation at IP joint of thumb is higher in PsA patients as compared to RA and UA patients.


  PC0059: Bullous Systemic Lupus Erythematosis presenting with electrolyte abnormalities caused by suspected acquired Gitelman syndrome Top


Varun Pulugundla; Bhaskar Medical College and Hospital, R. R. District, Telangana, India

A 21-year-old woman presented to hospital with 1-month history of on-and-off fever and bullous rash for which she received short course of steroid treatment at local hospital, complaints of weakness of lower limbs with cramps, and carpopedal spasms for 3 days. Examination: Pallor was present and blood pressure was 90/60 mmHg. Multiple hyperpigmented plaques and papules with crusting and rims of scaling were present over the scalp, upper limbs, and also multiple depigmented patches over the anterior chest and back with hyperpigmented crusts. Multiple oral and palatal ulcers were present. Chvostek's and Trosseau's signs were positive. Power in both lower limbs was ⅗, DTRs were 2+, and plantars showed flexor response. Laboratory investigations: Complete blood picture showed anemia and thrombocytopenia. Erythrocyte sedimentation rate was 60. Serum electrolytes showed hypokalemia, hypocalcemia, and hypomagnesemia. Arterial blood gas analysis revealed metabolic alkalosis (pH 7.51, PCO2 39.9 mmHg, and bicarbonate 32.7 meq/L). Urine polymerase chain reaction was 0.97. Urine electrolytes were sent to evaluate electrolyte abnormalities – urinary k + was 17.7/mmol/day, transtubular potassium concentration gradient was >4, cl was 80 mmol/L, calcium was 9.38 mg/dl, and urinary creatinine was 77.54 mg/dl (urinary calcium/urinary creatinine 0.12). Laboratory investigations were conclusive of Gitelman's syndrome. Antinuclear antibody by immunofluorescence method showed 3+ homogenous pattern. Anti-dsDNA and anti-Smith were also positive. Treatment: The patient was treated with intravenous supplementation of potassium, calcium, and magnesium and also on pulse therapy of methyl prednisolone followed by oral glucocorticoids and hydroxychloroquine.

Discussion: Gitelman syndrome is a autosomal recessive disorder caused by mutations in SLC12A3 gene present on chromosome 16q13 encoding thiazide-sensitive sodium chloride cotransporter. It is characterized by hypokalemia, hypomagnesemia, hypocalciuria, and metabolic alkalosis.

Conclusion: Systemic lupus erythematosus (SLE) is an autoimmune-mediated connective tissue disorder with heterogeneous presentation and it has diversified systemic manifestations. Acquired Gitelman syndrome in SLE is an atypical presentation and might be attributed to the autoantibodies to NCCT.


  PC0061: Autoimmune hemolytic anemia secondary to systemic lupus erythematosus with inflammatory polymyositis Top


P Raghuramulu, B Anusha; Bhaskar Medical Collage, R. R. District, Telangana, India

A 22-year-old female presented with complaints of discoloration of the 2nd toe of both feet and weakness of limbs with myalgia for 1 year. She had a history of fever, dyspnea, and dysphagia to solids for 1 month. Her general examination revealed pallor, icterus, and maculopapular rash on the cheeks, skin, and over the palm, and dry and thick, palpable spleen and muscle tenderness. Systemic examination revealed proximal muscle weakness. Her complete blood picture revealed normocytic normochromic anemia with leukopenia, lymphocytosis, and thrombocytopenia. Hemoglobin – 2.5%, total leukocyte count – 2500 cu/mm, lymphocytes – 84%, platelet count – 1.2 lakhs/cumm, mean corpuscular volume – 119 fl, MCH – 38 pg, and lactate dehydrogenase – 740 IU/L. Ultrasonography abdomen revealed mild splenomegaly. Arterial Doppler of both lower limbs revealed peripheral small-vessel vasoconstriction. Antinuclear antibody and C-reactive protein were strongly positive. Anti-dsDNA was also positive indicating systemic lupus erythematosus (SLE). Direct Coombs test was positive confirming autoimmune hemolytic anemia. Serum creatine kinase levels were 300 U/L along with anticytoplasmic antibody testing for anti Jo-1 which was positive indicating polymyositis.

Conclusion: SLE is an autoimmune disease which is prevalent in young girls. Not only does it have a typical presentation and a single entity, but also can present associated with other inflammatory conditions as seen in this case where the girl presented as Raynaud's phenomenon and inflammatory conditions. Hence, careful examination and quick evaluation with early diagnosis will help in treating the symptoms and prevention of the progress and complications.


  OPC0186: Triple overlap between silicosis, dermatomyositis, and scleroderma Top


Siddharth Jain, Joydeep Samanta1, Uma Kumar1, Surabhi Vyas2, Vaishali Suri3; Department of Internal Medicine, Division of Clinical Immunology and Rheumatology, Postgraduate Institute of Medical Education and Research, Chandigarh, Departments of 1Rheumatology, 2Radiodiagnosis and 3Pathology, All India Institute of Medical Sciences, New Delhi, India

A 28-year-old male, resident of Allahabad, mason by occupation, presented with a 4-year history of Raynauds phenomenon, 3-year history of diffuse skin tightening, and 2-year history of dry cough and exertional shortness of breath (progressive from MMRC Grade 0–2) not associated with orthopnoea/PND. He was evaluated outside and diagnosed as diffuse cutaneous systemic sclerosis (anti-Scl-70 positive) with interstitial lung disease (ILD) and pulmonary hypertension (PH), for which he was started on steroids (15–5 mg/d), mycophenolate (defaulted), and tadalafil. However, his dyspnea progressed and he developed proximal muscle weakness (all the 4 limbs) with neck flexor and truncal weakness, for which he visited our center. On evaluation, his muscle enzymes were elevated; electromyography was suggestive of myopathic pattern; muscle biopsy was consistent with dermatomyositis. Repeat computed tomography chest showed upper lobe-predominant extensive fibrosis with mediastinal lymphadenopathy showing egg-shell calcification. Retrospectively, the patient was found to have significant occupational exposure to silica. He was thus diagnosed as a case of triple overlap between silicosis, dermatomyositis, and scleroderma with PH and started on oral steroids 1 mg/kg/d (gradually tapered) and methotrexate 25 mg/week, to which he responded with improvement in muscle power. His respiratory complaints persisted, for which vocational counseling was given.

Discussion: Association of silicosis with scleroderma (Erasmus syndrome) is well known; however, silicosis is only sporadically reported with dermatomyositis. Scleroderma-dermatomyositis overlap is also rare, and its differentiation from benign fibrosing myopathy of systemic sclerosis is critical therapeutically and prognostically. Correct recognition of this “triple overlap,” probably reported for the first time, has important implications in patient management.

Conclusion: Not all cases of respiratory decompensation in patients with connective tissue disease (CTD) are due to ILD or PH or respiratory muscle weakness. A meticulous search for significant occupational exposure is mandatory in the workup of CTDs. This would prevent inadvertent exposure to long-term immunosuppression for a mislabeled CTD-ILD.


  OPC0187: A study of cardiovascular involvement in cases of rheumatoid arthritis with high disease activity Top


Debleena Paul, Luhamdao Bathari, R K Kotokey, M S Chaliha1; Departments of Medicine and 1Cardiology, Assam Medical College and Hospital, Dibrugarh, Assam, India

Background: Cardiovascular involvement is an important extra-articular manifestation of rheumatoid arthritis (RA) and also one of the most common causes of death. The incidence of coronary artery disease, carotid atherosclerosis, congestive heart failure, pericarditis, cardio-myopathy, mitral regurgitation, etc., is higher in RA patients than in the general population.

Aims and Objectives: To study the cardiovascular involvement in patients of RA with high disease activity.

Materials and Methods: This was a hospital-based observational study conducted for 1 year from July 2014 to June 2015. Out of 168 patients of RA screened, 131 patients were undertaken for the study and classified according to disease activity.

Results and Observations: out of the 131 patients, 51 cases had high disease activity. Left ventricular (LV) diastolic dysfunction was the most common finding (19.61%) in them followed by pericardial effusion, mitral regurgitation, LV systolic dysfunction, and isolated pulmonary hypertension with tricuspid regurgitation and aortic regurgitation.

Conclusion: RA cases with high disease activity were having comparatively more cardiovascular involvement than the nonhigh disease activity group. Therefore, all cases of RA should be screened for cardiovascular involvement and treated so that they do not end up in high disease activity.


  PC0074: Metastatic breast cancer mimicking polymyalgia rheumatica Top


Yogesh Preet Singh, Vaibhavi G Velangi; Manipal Hospital, Bengalore

Introduction: Polymyalgia rheumatica (PMR) is commonly seen in people over 50 years of age. It is characterized by aching pain in proximal muscle groups such as both shoulders, pelvic girdle, neck, and associated morning stiffness of >45 min with elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). PMR can mimic various rheumatic and nonrheumatic diseases and hence it can pose a diagnostic challenge. Especially due to its occurrence in the elderly population, malignancy must be always excluded.

Clinical Case: A 58-year-old female patient presented with a 4-month history of pain in both shoulders, diffuse back pain, and loss of appetite. She had no history of any comorbidity. In view of elevated inflammatory markers (ESR and CRP) and negative rheumatoid factor and anti-cyclic citrullinated peptide, she was started on glucocorticoids. On subsequent follow-ups, the patient complained of increasing pain in shoulders and diffuse back pain. On detailed evaluation, due to persistent pain, she was found to have metastatic breast cancer.

Conclusion: By recognizing the similarities between clinical presentation of PMR and PMR-like neoplastic syndrome, high vigilance needs to be maintained to improve diagnostic accuracy.


  PC0075: Incidence of ocular manifestation in rheumatoid arthritis in corelation with Anti CCP and RA factor Top


Indira Pegu, B. S. Puzari, S. Kakati; Assam Medical College and Hospital, Dibrugarh, Assam, India

Aim: To study the incidence of ocular manifestation in rheumatoid arthritis (RA) in correlation with anticyclic citrullinated peptide (CCP)antibody and RA factor.

Methods: 114 RA cases attending RA and ophthalmology outpatient department of a tertiary care hospital were screened. Detailed history, systemic, and ocular examination with slit-lamp biomicroscopy, Goldman's applanation tonometry, dilated direct and indirect ophthalmoscopy for fundus examination, and special tests such as Schirmer's and tear breakup time tests were done.

Results and Observation: Nearly 42.10% of patients were found to have ocular manifestation and dry eye was the commonest (36.84%) followed by cataract (5.26%). Sight-threatening complications such as scleritis, peripheral ulcerative keratitis, Sjogren's syndrome, and sclerosing keratitis were also encountered in this study. A strong association was shown between the ocular manifestation and the duration of the disease, steroid use, and presence of RA factor, anti-CCP antibody by significant P value.

Conclusion: Hence, flaring up of ocular manifestation and the presence of RA factor and anti-CCP antibody in RA patients may signal impending deterioration or relapse of RA and help us to detect, treat it earlier, and prevent both articular and extraarticular complications.


  OPC0232: Isolated mitochondrial myopathy Top


Yogesh Preet Singh, Vaibhavi G Velangi; Manipal Hospital, Bengalore

Mitochondrial diseases are a group of rare disorders caused by pathologic dysfunction of the mitochondrial respiratory chain. A major clue to mitochondrial disease is a history of multisystem involvement, particularly in organs most prone to suffer from mitochondrial defects. These include the heart, brain, retina, and/or skeletal muscle.

Case Report: A 19-year-old female presented with total duration of illness of 18 months. The symptoms included progressive effort intolerance, fatigue, and myalgias. The symptoms had progressed over that past 6 months and at presentation walking for 10 min or climbing one flight of stairs was tiring. The past medical history and family history were unremarkable. Her physical examination results were normal including evaluation of muscle power.

Significant laboratory results included elevated creatinine kinase = 2467 U/L (normal range 21-215 U/L), normal complete blood count, liver function tests, creatinine, urine examination, and thyroid functions tests. Vitamin D levels were low = 21.6 ng/ml (30–80 ng/ml). Urine for myoglobin was negative. Antinuclear antibody (immunofluorescence) and extractable nuclear antigen were negative. The plasma lactate levels were elevated = 52 mg/dl (4.5–19.8 mg/dl). Electromyography was suggestive of myopic pattern. Magnetic resonance imaging (MRI) was normal and did not show any features of muscle inflammation. A muscle biopsy (vastus lateralis) was performed. The histopathology showed maintained fascicular architecture, with red ragged fibers. Biochemical evaluation revealed COX-deficient fibers. These findings were consistent with mitochondrial myopathy. Subsequently, MRI of the brain was done to look for associated central nervous system involvement and it was normal. A detailed eye and ear, nose, and throat (ENT) evaluation were also negative for any associated eye or ENT involvement. She was treated with co-enzyme Q. Good response to treatment-less myalgia and improvement in effort intolerance.

This case highlights the need for a high index of suspicion to diagnose these rare diseases, especially in the absence of multisystem involvement.


  OPC0233: Prevalence of musculoskeletal pain in an urban slum: Community-oriented program for control of rheumatic disorders study from Hyderabad Top


Meghna Gavali, Phani Kumar Devarasetti, Liza Rajasekhar; Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background: To understand the true burden of rheumatic diseases in India, there is need to extend Community-Oriented Program for Control of Rheumatic Disorders (COPCORD) to multiple sites.

Objectives: To assess the prevalence of musculoskeletal disorders in a selected urban area using specifically trained grassroot health-care workers (HCWs).

Methods: The study was conducted between September 2017 and September 2018 after Institutional Ethics Committee approval in an urban slum area of Hyderabad using COPCORD Core Questionnaires (CCQ) translated to Telugu and Hindi and validated. A team of rheumatologists, research coordinators, nursing students, and primary HCWs (doctors at primary health center, accrediated social health activists, and auxillary nurse midwifes) were trained in administering CCQ. Data were collected in three standard COPCORD phases by a door-to-door survey. Results were presented as median and interquartile range.

Results: A total of 647 participants from 189 households consented for survey. Demographic characteristics are listed in [Table 1]. Of 148 musculoskeletal pain (MSKP) ever, 139 consented for Phase 2. Median age was 45 (35–57) years, 59.7% were females. Frequent site of the current MSKP was knee, back, and soft tissue. Median duration of pain was 2 (1–5) years. Intensity of the current pain was very severe or severe in 45% participants leading to severe effect on work ability, sleep, mood, and occupation (in 14.5%, 8.1%, 24.6%, and 13%, respectively). Median HAQ score was 0.375 (0–0.88). Of 30% requiring help for daily activities, 22.5% needed for rising.



Fifty-one of 139 (36.7%) participants made themselves available for rheumatological evaluation. Knee osteoarthritis (n = 29; 56.9%), low back pain (n = 11; 21%), and soft-tissue rheumatism (n = 7; 13.7%) were most frequent. Inflammatory arthritis was detected in 6 (11.7%) with rheumatoid arthritis in 3 participants.

Conclusion: Prevalence of current MSK pain in an urban slum population was 17.3%, most often and severe in the knee, back, and soft tissue with significant functional and occupational disability and dependency. The most common diagnosis was osteoarthritis. Inflammatory arthritis diagnosed in 0.9%. There is significant hesitation in accepting medical help for MSKP.

Acknowledgment: This study was funded by IRA research grant (Epidemiology) for the year 2017–2018.


  OPC0239: NA Top


George Ipe, Sathish Kumar, Jeyakantan; Department of Pediatrics, Christian Medical College, Vellore, Tamil Nadu, India

Background: Anti-C1q has been associated with systemic lupus erythematosus (SLE) as well as in other connective-tissue diseases. They have been considered as a marker for disease activity and presence of nephritis in previous studies.

Objectives: The aim of this study was to determine the prevalence of anti-C1q antibodies in the pediatric SLE population and to determine clinical associations of elevated anti-C1q antibody levels, especially with lupus nephritis.

Methods: Sera of 150 pediatric SLE patients who fulfilled the American College of Rheumatology criteria for SLE were recruited. After obtaining informed consent, blood samples were tested for anti-C1q antibody by commercially available enzyme-linked immunosorbent assay kit. Prevalence of anti-C1q and its association with lupus nephritis were determined..

Results: Out of total 150 children with SLE, anti-C1q positivity was present in 95 children (64%), at a cutoff value of 20 U/ml. Children with proteinuria, low C3, low C4, and anti-dsDNA positivity were significantly more likely to have anti-C1q antibody positivity. Children with lupus nephritis were significantly more likely to have anti-C1q antibodies positivity than children without renal involvement (74% vs. 51%, P = 0.02). Among the children with lupus nephritis, children with active renal disease were more likely to have anti-C1q positivity than in children with quiescent disease (88% vs. 53%, P = 0.002). Anti-C1q antibodies had a sensitivity of 74% and specificity of 54% at a cutoff value of 22 U/L, for renal disease in pediatric SLE (pSLE).

Conclusion: Our study confirms previous findings of the association of anti-C1q antibodies with nephritis and disease activity in pSLE. Anti-C1q antibody titers were found to have a positive correlation with renal disease in children with pediatric SLE and could be used as an adjunctive biomarker in monitoring disease activity in children with lupus nephritis.


  OPC0234: Sleep disturbance and anxiety depression in rheumatoid arthritis and its correlation with disease activity Top


H Singh, A Kumar, J Singh, M Yadav, N Gupta, S Giri; Department of Medicine, Pt. B.D.S. PGIMS, Rohtak, Haryana, India

Background: Sleep and mood disturbance has been reported in rheumatoid arthritis (RA) with the prevalence of 75% and 30%, respectively, but no data are available in Indian population, especially regarding the impact of disease activity and its correlation with sleep and mood disturbances in RA.

Objectives: The objective was to study the correlation of sleep disturbances and anxiety depression with disease activity in RA.

Methods: A total of 100 patients of RA as per the American College of Rheumatology Criteria (1987) were enrolled in the study. All patients were evaluated for disease activity (using Disease Activity Score 28 [DAS28] and Clinical Disease Activity Index [CDAI]), sleep disturbance (using Pittsburgh sleep quality index [PSQI]), and mood disturbance (using Hospital Anxiety and Depression Score [HADS]) at baseline and 4 months.

Results: Mean age was 43.07 ± 12.41 years, with 79 females and 21 males. At baseline and 4 months, DAS28 score was 6.66 ± 0.86 and 3.50 ± 0.90; CDAI score was 38.20 + 11.43 and 8.53 + 5.86; PSQI was 17.55 ± 2.819 and 3.94 ± 2.498; and HADS was 27.99 ± 4.382 and 4.41 ± 6.11, respectively. The Pearson's coefficient of PSQI was found to be positively correlated with disease activity at baseline but negatively at 4 months, whereas Pearson's coefficient for HADS with disease activity was found to be positively correlated at baseline and 4 months.

Conclusion: Based on the above results, disease activity is associated with mood disturbances in RA, whereas sleep disturbances did not follow the improvement trend of disease activity. It is suggested that RA patients with high disease activity should be assessed with HADS to delineate associated mood disturbances.


  PC0077: The old man with myopathy: An uncommon case of lupus nephropathy in an elderly male Top


Mallik A, Pal P, Sinha S, Sarkar K, Pal S, Gonjhu D, Pramanick N; The School of Tropical Medicine, Kolkata, West Bengal, India

Objective: An elderly male presenting with lupus nephropathy is a rare finding in literature. We report a case of an elderly male systemic lupus erythematosus (SLE) patient presenting with myopathy and nephropathy, concluding that age and sex of a patient is no bar for lupus and its presentation is varied.

Methods: A 65-year-old, nondiabetic, hypertensive male presented with insidious-onset gradually progressive proximal muscle weakness of both upper and lower limbs for the last 1 month associated with weight loss and myalgia. A history of low-grade intermittent fever for the last 2 weeks subsided on medication. The patient is on antihypertensive for the last 1 year. Diminished power of proximal group of muscles of lower limbs was more than that of upper limbs. Blood examination revealed normochromic-normocytic anemia and erythrocyte sedimentation rate of 50 mm in the 1st h. Blood and urine culture and sensitivity both were negative. Liver function test showed hypoproteinaemia and hypoalbuminemia. 24-h collection of urine revealed 0.85 g protein. Hepatitis virus profile and HIV screening test were negative. Antinuclear antibody (ANA) was 1:160 titer, with perinuclear pattern, and anti-ds DNA was positive. The patient developed respiratory distress without expectoration. Chest X-ray and high-resolution computed tomography thorax showed bilateral pleural effusion. Pleural fluid cytology examination showed predominant lymphocytes, with no malignant cells. ANA of the pleural fluid was 1:320 titer, fine-speckled pattern. Renal biopsy f/b immunostaining confirmed SLE (Stage-IVa). Serum creatine kinase was normal. Electromyographic study of all the four limbs showed generalized myopathic pattern with left-sided peroneal neuropathy. Malignancy was ruled by upper gastrointestinal endoscopy, colonoscopy, and contrast-enhanced computed tomography abdomen, which were normal. The patient was then treated with pulse methylprednisolone 1 g per day for 3 days and f/b cyclophosphamide therapy. Repeat 24 h collection of urine revealed 0.32 g protein after 2 months.

Conclusion: Late-onset lupus nephropathy with myopathy and weight loss in an elderly male must be considered in the differential diagnosis of malignancy with unknown primary with paraneoplastic syndrome.


  OPC0235: A study of human leukocyte antigen phenotype prevalence among patients with spondyloarthritis presenting to a tertiary care hospital in South India Top


H Raghavendra, S Balameena, R Ramesh, S Mythili, R Anuja, M Rajavel, M Anusha; Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Spondyloarthritis (SpA) comprises a group of inflammatory diseases that share similar clinical presentations, radiological findings, human leukocyte antigen (HLA) association, and positive family history. HLA-B27 is the biggest risk factor for the development of SpA, and while it has a central role in its etiopathogenesis, the association is not the same across all ethnic groups. Various studies have shown that HLA-B27 is not the only HLA allele associated with SpA.

Aim and Objectives: The aim and objectives were to study the HLA phenotype prevalence in patients of SpA, to determine the HLA phenotype in patients of SpA, and to differentiate the HLA types associated with axial and peripheral SpA.

Methods: A total of eighty patients were included for the study. It consisted of ankylosing spondylitis, reactive arthritis, peripheral SpA, inflammatory bowel disease (IBD)-associated arthropathy, psoriatic arthritis (SpA form), and juvenile idiopathic arthritis (JIA)/early rheumatoid arthritis (ERA) patients who satisfied their respective classification criteria and without any contraindication for HLA typing. HLA Class 1 (A, B, C) phenotyping was done by microlymphocytotoxicity test using Terasaki HLA Class 1 Tissue Typing Trays coated with HLA Class 1-specific monoclonal antibodies procured from One Lambda, CA, USA.

Results: A total of eighty patients were included in the study. Nearly 37% of patients were ankylosing spondylitis, out of which 50% were HLA-B27 positive. A24 and Cw6 were other loci which were common in these patients. A2 and Cw6 phenotype were observed in peripheral SpA. A1, B35, and Cw6 were seen in patients of JIA/ERA. IBD-associated arthropathy was associated with HLA B51 and Cw6 positivity. Psoriatic SpA was associated with B35 and Cw6 positivity.

Conclusion: In our study, only 50% of patients with ankylosing spondylitis were positive for HLA-B27; hence, we conclude that various other HLA phenotypes are associated with SpA in the Indian subcontinent, and hence, they have to be considered during evaluation.


  OPC0236: Efficacy of tocilizumab in rheumatoid arthritis: A retrospective study from a tertiary care center in South India Top


Matam Sri Anusha, Ragavendra H, Balameena S1, Ramesh R1; Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Tocilizumab is a monoclonal antibody against the interleukin-6 receptor involved in pathogenesis of rheumatoid arthritis (RA). This is a retrospective study of RA patients and their clinical profiles during the first 6 months of tocilizumab treatment.

Aim: The aim was to study the efficacy of tocilizumab in RA patients from a tertiary care center.

Inclusion criteria: Patients fulfilling European League Against Rheumatism (EULAR) criteria and aged above 16 years were included.

Exclusion criteria: Patients aged <16 years, pregnant women, and patients with Any focus of infection, presence of deformities, and low disease activity were excluded.

Patients and Methods: The study cohort consisted of patients who fulfilled the EULAR classification criteria and who had undergone tocilizumab treatment between November 2017 and January 2018 at MMC, Chennai. All data were collected from medical charts and evaluated retrospectively. Baseline cardiac evaluation, chest X-ray, and viral markers were done before the initiation of tocilizumab therapy. Tocilizumab was infused every 4 weeks at a dose of 8 mg/kg to patients refractory to disease-modifying antirheumatic drugs (DMARDs). The disease activity indices and acute-phase reactants were evaluated at 12 and 24 weeks after the initial tocilizumab infusion.

Results: Disease activity was assessed by disease activity score (DAS) 28-erythrocyte sedimentation rate (ESR) and DAS28-C-reactive protein (CRP) calculated using standard formulas. The endpoint was decrease in DAS28-ESR and DAS28-CRP from baseline to week 24. The average DAS28-ESR and DAS28-CRP of all patients significantly decreased from 6.68 to 3.11 and 6.4 to 3.37, respectively, after 24 weeks of therapy. The average clinical disease activity index and simplified disease activity index reduced from 34.9 to 5.8 and 52.8 to 10.3, respectively. In this study, no major or minor adverse events were noted.

Conclusion: Tocilizumab is found to be a promising option in RA patients refractory to first-line conventional DMARDS consistent with reduction in acute-phase reactants and disease activity indices.


  OPC0237: Serum calprotectin levels as a marker of disease activity in children with juvenile idiopathic arthritis Top


Anish Sam George, T.Sathish Kumar; Christian Medical College, Vellore, Tamil Nadu, India

Background: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disorder of childhood and encompasses a complex group of disorders comprising several clinical entities with the common feature of arthritis. Calprotectin is a calcium- and zinc-binding protein which belongs to s100 family of proteins and is released during interaction of leukocytes with inflammatory activated endothelium at the sites of inflammation as occurs in JIA. We undertook this study to assess the usefulness of calprotectin as a marker of disease activity in Indian children with JIA.

Objectives: The objective of the study was to assess the usefulness of serum calprotectin levels as a marker of disease activity in children with JIA.

Materials and Methods: A total of 121 children who fulfilled the International League of Associations for Rheumatology criteria for JIA were recruited into the study. Baseline demographic details were collected, and blood counts, erythrocyte sedimentation rate, C-reactive protein, and calprotectin levels were analyzed in all children after obtaining consent. Children were then divided into two groups based on disease activity as per the Wallace criteria. Calprotectin levels were also analyzed in 10 normal healthy children. Calprotectin levels were measured.

Results: A total of 121 children with JIA were recruited into the study; 63 had active disease and 58 had inactive disease. Systemic-onset JIA constituted 42% of the study population and was the predominant disease subtype. Calprotectin levels were elevated in children with active disease compared to those with inactive disease. Mean calprotectin value in active disease (3954 ng/ml) was twofold higher than those with inactive disease (1899 ng/ml) (P < 0.001) and 16 times higher than children who were normal healthy controls (mean of 233 ng/ml).

Conclusion: Serum calprotectin levels were found to be a good marker of disease activity in children with JIA.


  OPC0238: Our experience with juvenile idiopathic arthritis Top


Anshu Kumar Jha, Rebecca R Marak, Sanjeeb Kakati; Department of Medicine, Assam Medical College, Dibrugarh, Assam, India

Case Series: Juvenile idiopathic arthritis (JIA) is the most common inflammatory arthritis of childhood. Various genetic factors have been implicated for JIA. One of the most commonly studied is the MHC locus on chromosome 6. Interleukins have a definite role in the clinical manifestation of the disease. Here, we present a case series of 14 patients suffering from JIA (7 polyarticular, 2 oligoarticular, 2 systemic, and 3 enthesitis related). All of them presented with the complaint of joint pain which was progressive. Four cases of polyarticular JIA who were rheumatoid arthritis factor positive did not respond to methotrexate and sulfasalazine had to be added to subside their symptoms. One patient of systemic JIA went into complication and ended up in macrophage activation syndrome. Another patient of enthesitis-related JIA was found to be human leukocyte antigen-B27 positive and was finally diagnosed with early rheumatoid arthritis with ankylosing spondylitis. His symptoms subsided only after receiving etanercept.

Discussion: Being the most common arthritis of childhood, early diagnosis and treatment are required to prevent long-term morbidity. “Step up” approach is recommended in all the cases. Methotrexate has been the most commonly used disease-modifying antirheumatic drugs, and use of biologic is recommended if symptoms do not subside or there are poor prognostic features.

Conclusion: Thus, this case series helps us to understand the common presenting features various complications and the treatment approach to a case of JIA.


  PC0078: The overlap of ra and spondyloarthritis is rarely recognized: Management may not differ but a research opportunity is lost Top


Kiran Adam, Anuradha Venugopalan, Arvind Chopra; Center for Rheumatic Diseases, Pune, Maharashtra, India

Conceptually, rheumatology has evolved around a distinct categorization of rheumatoid arthritis (RA) and spondyloarthritis (SpA). Intriguingly, there are few publications on the “overlap.” We describe some important features of this overlap in a retrospective design study. Data were mined from a comprehensive patient database maintained in CRD, Pune, since 1996. Records of 65,000 patients were accessed. In our community-based clinic, we use standard rheumatology case record forms. We found 98 patients with clinical diagnosis of an RA-SSA overlap. male:female ratio was 1.3:1; mean age at disease onset was 40 years; positive family history was noted in 37 patients. We present data of 61 patients who fulfilled the American College of Rheumatology/European League Against Rheumatism 2010 criteria for RA and/or Assessment of Spondyloarthritis International Society Criteria for SpA. Proportion of patients showing various features were as follows: 96% with symmetrical polyarthritis, 93% inflammatory axial disease, 58% sacroiliitis, 18% hip disease, 10% enthesitis, and 7% dactylitis. None had oculomucocutaneous or extramusculoskeletal feature. Typical X-ray hands/feet juxta-articular erosions were seen in 42% patients. Seropositive rheumatoid factor (RF)/anti-cyclic citrullinated peptide (CCP) (nephelometry/second-generation ELISA) was observed in 62% of patients and 52% tested positive for human leukocyte antigen-B27 (polymerase chain reaction - single-specific primer). Patients were treated with long-term sulfasalazine and methotrexate; weekly, methylprednisolone for 6 weeks was often used initially. Persistent back pain required considerable physiotherapy. Patients improved considerably by 1-year follow-up and often entered low disease activity (disease activity score-28). We believe that recognition of this overlap syndrome, albeit uncommon, is missed and/or neglected. Management seems similar to RA. Research may unravel individual contributions of B27 and RF/anti-CCP to improve our understanding of autoimmune arthritis.


  OPC0241: Prevalence and clinical associations of anti-C1q antibodies in pediatric systemic lupus erythematosus Top


George Ipe Vettiyil, Sathish Kumar T; Christian Medical College Hospital, Vellore, Tamil Nadu, India

Background: Anti-C1q has been associated with systemic lupus erythematosus (SLE) as well as in other connective tissue diseases. They have been considered as a marker for disease activity and presence of nephritis in previous studies.

Objectives: The objectives of this study were to determine the prevalence of anti-C1q antibodies in the pediatric SLE (pSLE) population and to determine clinical associations of elevated anti-C1q antibody levels, especially with lupus nephritis.

Methods: Sera of 150 pSLE patients who fulfilled the American College of Rheumatology criteria for SLE were recruited. After obtaining informed consent, blood samples were tested for anti-C1q antibody by commercially available ELISA kit. The prevalence of anti-C1q and its association with lupus nephritis were determined.

Results: Out of total 150 children with SLE, anti-C1q positivity was present in 95 children (64%), at a cutoff value of 20 U/ml. Children with proteinuria, low C3, low C4, and anti-dsDNA positivity were significantly more likely to have anti-C1q antibody positivity. Children with lupus nephritis were significantly more likely to have anti-C1q antibodies positive than children without renal involvement (74% vs. 51%, P = 0.02). Among the children with lupus nephritis, children with active renal disease were more likely to have anti-C1q positivity than in children with quiescent disease (88% vs. 53%, P = 0.002). Anti-C1q antibodies had a sensitivity of 74% and specificity of 54% at a cutoff value of 22 U/L, for renal disease in pSLE.

Conclusion: Our study confirms previous findings of the association of anti-C1q antibodies with nephritis and disease activity in pSLE. Anti-C1q antibody titers were found to have positive correlation with renal disease in children with pSLE and could be used as an adjunctive biomarker in monitoring disease activity in children with lupus nephritis.


  OPC0242: Renal manifestations in systemic sclerosis beyond 'Scleroderma Renal Crisis' Top


Shefali Khanna Sharma, Chitra Raj Sharma, Arghya Chattopadhyay, Manish Rathi, Anandita Sinha, Yashwant Kumar, Surjit Singh, Sanjay Jain; Post Graduate Institute of Medical Education and Research, Chandigarh, India

Background: Renal involvement is a major factor in determining prognosis in systemic sclerosis (SSc). Data on the spectrum of renal manifestations of scleroderma are sparse and focused mainly on scleroderma renal crisis (SRC).

Aims: The aim was to study the clinical and subclinical renal manifestations in patients of SSc.

Materials and Methods: Eighty-four patients (77 females and 7 males) fulfilling the 2013 American College of Rheumatology/European League Against Rheumatism Classification Criteria for SSc were included in this prospective, single-center study. Renal function tests, urine routine, 24-h urine protein, and urine creatinine were done; estimated glomerular filtration rate (eGFR) was calculated using Chronic Kidney Disease Epidemiology Collaboration formula. Renal resistive index (RRI) was calculated ultrasonographically.

Results: Seven (8.3%) patients were hypertensive, and 3 (3.5%) had SRC. Abnormal eGFR was seen in 7 (8.3%) patients. Three (3.5%) had GFR between 60 and 79 ml/min/1.73 m2; 4 had GFR <60 ml/min/1.73 m2. One patients with eGFR between 60 and 79 ml/min/1.73 m2 and 2 with GFR <60 ml/min/1.73 m2 had significant proteinuria. Overt proteinuria (24-h urine protein >300 mg/total volume) was present in 7 (8.3%) (4 patients had normal GFR) and microalbuminuria in 5 patients (3 patients had normal GFR). Mean RRI of the renal artery regarded solely as an indicator of renal vascular resistance measured 0.65 ± 0.08 on the right and 0.660 ± 07 on the left. Seven had increased RRI. One patient had significant proteinuria and one had GFR <60 ml/min/1.73 m2.

Conclusion: Of the spectrum of renal manifestations in SSc, impaired GFR and proteinuria are the most common these patients must be followed up to ensure that these do not become clinically overt.


  OPC0244: Adult-onset Still's disease with macrophage activation syndrome: A case report Top


Droupadi, S Rajeswari, Chethan, Mukarram, Saranya, Balaji, Shanmugesh, Pradeep, Deepak, Ramu; Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Case Report: A 20-year-old male presented with complaints of intermittent low-grade fever, sore throat, polyarthritis, and skin rash on and off for 1 month. Rashes found during fever spikes were palpable, blanching, nonitchy, spread over upper arm, both shoulders, and back. It exfoliated after 4 febrile days. On examination, he was febrile (102°F), was pale, had palmar erythema, icterus, supraclavicular and cervical lymphadenopathy, tender hepatomegaly, and severe arthritis over both elbows, wrists, and small joints of hands. Blood counts showed anemia and thrombocytopenia. He had elevated liver enzymes (OT/PT-642/1044), serum ferritin (12,436 pg/ml), lactate dehydrogenase, low triglycerides, and low serum albumin. Urine routine showed proteinuria. Other common causes of fever with arthritis were ruled out. He was hence diagnosed as a case of adult-onset Still's disease (AOSD), with a potentially fatal complication, namely macrophage activation syndrome (MAS). Serial liver function tests were done. He was treated with pulse steroids, liver protective, and other supportive medications. In view of severe disease activity, after getting consent, he was given monthly tocilizumab infusion (interleukin-6 receptor inhibitor) and weekly methotrexate. He started improving after the first infusion with marked reduction in disease activity. His serum ferritin, transaminases, and other inflammatory markers normalized.

Discussion: AOSD is an uncommon seronegative polyarthritis seen in young adults. It presents as arthritis, high spiking fever, and salmon-colored evanescent rash. MAS is an unusual presentation of AOSD. Our patient came with a history of intermittent low-grade fever, multiple joint pain, sore throat, and rashes and was diagnosed to have AOSD with MAS after thorough clinical examination and investigations. He responded to combination therapy with disease-modifying antirheumatic drugs and biologics.

Conclusion: This case is reported for its rarity as only 5%–10% of the AOSD cases were reported to have the complication of MAS.


  OPC0243: Infections are leading cause of in-hospital mortality in patients with inflammatory myositis Top


Hafis Muhammed, Latika Gupta, Abhishek Zanwar, Durga P Misra, Able Lawrence, Vikas Agarwal, Amita Aggarwal, Ramnath Misra; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Idiopathic inflammatory myositis (IIM) is debilitating and often leads to mortality. We explored causes of in-hospital mortality and predictors of early mortality at a tertiary care center in Northern India.

Methods: Records of adults and children with dermatomyositis (DM), polymyositis (PM), or anti-synthetase syndrome (ASS) who died between 2000 and 2018 were reviewed and cause of death was determined. Early mortality was defined as death with 6 months of diagnosis. For comparison, patients with IIM surviving more than 6 months after diagnosis and having registration number before and after the index case were included. Severe weakness was defined as MRC <3 in any group of muscles.

Results: Of the 38 (32 women) deaths, 20 were DM including 2 clinically amyopathic DM, 4 juvenile DM, 12 PM, and 2 ASS. Median age at death was 42.2 years. At time of death, 18 had active disease, while 14 and 6 had grumbling and inactive disease, respectively. Thirty-two (84.2%) had infection (11 bacteriologically proven), of which 19 died of septic shock. Other causes of death included myocarditis (n = 3), respiratory failure (n = 4), cerebral bleed (n = 1), and pulmonary embolism (n = 1). One patient of ASS succumbed to rapidly progressive interstitial lung disease, while another one died following rituximab-induced acute respiratory distress syndrome. Twenty-Five (65.7%) patients had early death. In comparison to controls (n = 50), they were older and had higher frequency of severe weakness, respiratory involvement, and thrombocytopenia (P < 0.005).

Conclusions: Infections are the most common cause of in-hospital mortality in IIM patients. Most deaths occur early in disease and older age; severe weakness and respiratory involvement are the predictors of early death.


  OPC0245: Do we need renal biopsy while planning management of lupus nephritis: An observational study from a tertiary care centre Top


Debaleena Mukherjee, Sanchita Saha, Alakendu Ghosh; IPGMER and SSKM Hospital, Kolkata, West Bengal, India

Introduction: Renal biopsy has remained the gold standard for diagnosis and therapy for suspected lupus nephritis patients. However, recently, experts have questioned the influence of renal biopsy in prediction of outcome, treatment, and prognosis in patients of systemic lupus erythematosus (SLE), suggesting that immunosuppressive protocol can probably be given in all patients of suspected lupus nephritis avoiding an invasive renal biopsy procedure.

Aim: We tried evaluating the outcome of planning of therapeutic strategy in lupus nephritis patients without doing renal biopsy.

Methods: An observational, prospective study was done including 50 newly diagnosed cases of SLE (as per the SLICC criteria, 2012), aged >18 years, with proteinuria ≥500 mg/24 h and active urinary sediments. Patients positive for antiphospholipid syndrome were excluded from the study. Patients were randomized into two groups – Group A underwent renal biopsy and Group B did not. Both groups were managed as per the NIH protocol and followed up at 3, 6, and 12 months. Primary endpoints included (1) proteinuria reduction: 50% reduction from baseline and/or ≤500 mg/24 h and/or ≤200 mg/24 h; (2) disappearance of active sediments from urine, and (3) return of creatinine to baseline or 25% increase in estimated glomerular filtration rate (eGFR).

Results: Both groups were compared and statistical analysis revealed that Group A had 92% remission while Group B had 96% remission with respect to proteinuria, disappearance of active urine sediments, and eGFR reduction. The study included six male patients. One patient required a repeat renal biopsy for induction failure. None of the patients from nonbiopsy group have required a biopsy yet.

Discussion: The study revealed no statistically significant difference between the groups, showing not doing renal biopsy in the presence of active urine sediments as noninferior to doing renal biopsy in planning management. However, at the moment, all the literature data do not allow us to state that omitting the test brings more advantages than threats.


  PC0081: Tenosynovitis: An unusual presentation of gout Top


Sandeep Nagar; Department of Rheumatology, AIIMS, New Delhi, India

Introduction: Given the prevalence of gout, tenosynovitis of the upper extremity is rarely reported. However, a few cases of tendon rupture secondary to tenosynovitis have been reported in literature, all in patients with a risk factor for or history of gout.

Case Report: A 40-year-old male patient without any history of comorbidity and addiction presented in our outpatient department with pain and swelling in the right forearm and hand, which was insidious in onset and slowly progressive. Pain was diffuse, was moderate in intensity, increased with movement, and relieved to some extent with nonsteroidal anti-inflammatory drugs (NSAIDs). On examination, he had diffuse nonpitting swelling of the right distal forearm and dorsal aspect of the hand; overlying skin was shiny with mild diffuse tenderness without any temperature difference, restriction of movement, and tendon rub. His serum uric acid was raised (10.1 mg/dl); ultrasound right hand showed tenosynovitis of extensor tendons. On follow-up, he revealed the history of two episodes of right ankle and first metatarsophalangeal (MTP) joint arthritis; 2 years back, each time resolved with NSAIDs. His ultrasound of the ankle and first MTP joint was negative for double contour sign and hyperechoic aggregates. In view of high suspicion of gout, dual-energy computed tomography was done which showed deposition of monosodium urate crystals at ankle and MTP joint. We started him on allopurinol and colchicine; after 9 months of treatment, his symptoms and ultrasound findings resolved completely.

Discussion: Gout results from accumulation of monosodium urate at level sufficient to drive the precipitation of crystals in the joints and soft tissue and initiation of inflammatory response by leukocytes.

Conclusion: In patients who are diagnosed with tenosynovitis, it is important to consider the possibility of gout-related pathological manifestation.


  OPC0254: Deficiency of adenosine deaminase 2: Report of three cases from single center in North India Top


G S R S N K Naidu, Nupoor Acharya, Saketh Jha, Arghya Chattopadhyay, Varun Dhir, Manoj Goyal1, Manish Modi1, Rithambra Nada2, Ranjana W Minz3, Sanjay Jain, Aman Sharma; Departments of Internal Medicine, 1Neurology, 2Histopathology and 3Immunopathology Postgraduate Institute of Medical Education and Research, Chandigarh, India

Introduction: Deficiency of adenosine deaminase 2 (DADA2) is a recently described hereditary autoinflammatory disease with limited published literature till date. We describe three cases of DADA2 diagnosed and treated at a tertiary care center in North India.

Case 1: A 14-year-old female child presented with recurrent ulcers over feet, recurrent right facial nerve palsy, peripheral neuropathy, Raynaud's phenomenon, and livedo reticularis. She had elevated inflammatory markers (erythrocyte sedimentation rate and C-reactive protein) and homozygous P.(Gly47Arg) missense mutation in CECR1 gene. She was treated with steroids and adalimumab with symptomatic improvement.

Case 2: A 35-year-old female presented with features mimicking antiphospholipid syndrome in the form of nonhealing ulcer, left hemiparesis, left ulnar artery occlusion, livedo reticularis, and preeclampsia with preterm deliveries. She had peripheral neuropathy; inflammatory markers were elevated, Antiphospholipid antibody was negative and plasma ADA2 activity was. She had two novel and yet unreported mutations (P[Leu188Val] and c.753 + 2T>A) in CECR1 gene. She was treated with adalimumab.

Case 3: A 37-year-old male presented with polyarthritis, mononeuritis multiplex, skin rash, Raynaud's phenomenon, and elevated inflammatory markers since 8 years of age, managed as polyarteritis nodosa with intravenous methylprednisolone (IVMP) and intravenous cyclophosphamide (IV CYC). In 2014, he developed gastrointestinal vasculitis, pseudoaneurysms in hepatic and renal arteries, and diffuse alveolar hemorrhage and received IVMP and IV CYC. A month later, he developed ileal perforation for which rituximab was initiated. In view of inadequate response to high-dose steroids and CYC, DADA2 was suspected and ADA2 activity was assessed which was low.

Discussion: DADA2 is characterized by recurrent fever, early-onset stroke, peripheral neuropathy, livedo reticularis, skin ulceration, hypogammaglobulinemia, and loss of function mutations in CECR1 gene. Treatment with anti-tumor necrosis factors was shown to be effective.

Conclusions: To the best of our knowledge, this is the first case series of DADA2 from India. Novel mutations of CECR1 gene, as seen in Case 2, can be associated with DADA2.


  PC0080: Study of 17 patients of anti-neutrophil cytoplasmic antibody-associated vasculitis presented to Department of Rheumatology, IPGMER and SSKM Hospital, Kolkata Top


Dipendra Nath Ghosh, Hiramanik Sit, Subhankar Halder, Pradyot Sinhamahapatra, Parasar Ghosh, Alakendu Ghosh; Department of Rheumatology, IPGMER, Kolkata, West Bengal, India

Background: Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is necrotizing vasculitis affecting small vessels and associated with antineutrophil cytoplasmic antibody. AAV is associated with significant mortality if it is not treated properly.

Objectives: The objectives were to study the clinical and laboratory parameters of AAV patients and to study the response to therapy.

Methods: This was a longitudinal observational study conducted in the Department of Rheumatology, IPGMER.

Results: We analyzed data of 17 patients of AAV with a median follow-up of 1 year 3 months. There were 12 females and seven males. The mean age at presentation was 46.24 ± 15.63 years. Eleven patients had granulomatosis with polyangiitis (GPA), four patients had eosinophilic GPA, and two patients had microscopic polyangiitis. Predominant presenting features were fever in 76%, cough in 76%, hemoptysis in 18%, oliguria in 6%, and neuropathy in 24%. Mean creatinine at presentation was 1.8 ± 1.6 mg/dl. Kidney biopsy was performed in seven patients and crescentic glomerulonephritis found in five patients. Mean Birmingham vasculitis activity score (BVAS) at presentation was 20.12 ± 6.96. Immunosuppression as induction was given as follows: 13 patients received cyclophosphamide, one patient received rituximab, and the rest received methotrexate or azathioprine. After induction, remission was achieved in 12 patients. Three patients failed to achieve remission with induction. Two patients died during induction. All three patients who failed to achieve remission were receiving cyclophosphamide as induction, and later, they received rituximab for repeat induction. Those who failed to achieve remission or who died despite induction therapy had renal and lung involvement and higher BVAS at presentation. Mean BVAS after induction was 5.75 ± 9.68. Azathioprine was used as maintenance in most patients.

Conclusions: Our patients had predominantly lung, renal, and peripheral nervous system involvement. Most patients received cyclophosphamide as induction therapy. With induction therapy, most patients achieved remission. Rituximab was used for repeat induction for those who failed to achieve remission.


  OPC0249: Prevalence of tuberculin positivity in patients of early rheumatoid arthritis Top


Shefali K Sharma, Kriti Kishor, Varun Dhir, Sanjay Jain; Postgraduate Institute of Medical Education and Research, Chandigarh, India

Objective: The objective of the study was to estimate the prevalence of tuberculin positivity in treatment-naïve and on treating rheumatoid arthritis (RA) patients.

Methods: Tuberculin testing was done using Mantoux method. Five tuberculin unit of purified protein derivative (PPD) (0.1 ml) was injected intradermally into the flexor aspect, left forearm. Results were read by measuring the horizontal induration at 72 h. Readings >10 mm were taken as positive and <5 mm as negative.

Results: Two hundred RA patients and 99 controls were included in the study. The study population had treatment-naïve patients (n = 115, 57.5%), patients on conventional disease-modifying antirheumatic drugs (methotrexate only) (n = 57, 28.5%), and patients on low-dose steroids (<7.5 mg/day) and methotrexate both (n = 28, 14%). Tuberculin positivity was lower in the treatment-naïve population (20%, n = 23) as compared to healthy controls (n = 20, 21.2%). 74.5% cases (n = 149) and 79.8% (n = 79) controls showed anergy to PPD. There was more no of positive tuberculin cases among the methotrexate group 38.6%, n = 22, though statistically not significant (P = 0.065). Tuberculin positivity was not influenced by disease activity, dosage of methotrexate, bacillus Calmette–Guérin vaccination, or presence of comorbid conditions not requiring immunosuppression. None of the patients or controls with positive tuberculin skin test (TST) showed the presence of active tuberculosis (TB) on chest X-ray.

Conclusion: The prevalence of tuberculin positivity was low in treatment-naïve RA patients. Patients on methotrexate alone had higher TST positivity as compared to steroids and methotrexate group. When compared to Indian data that show 34.2% TST positivity in the general population, both RA patients and controls had a lower positive rate. The mechanism by which RA as a disease affects TST needs further evaluation. Although cost-effective, variability of tuberculin positivity among RA patients warrants better tests than TST for the screening of latent TB.


  PC0082: Eosinophilic fasciitis: Role of MRI in diagnosis: Case report Top


Saumya Ranjan Tripathy, Manoj Kumar Parida, Bidyut Kumar Das; SCB Medical College, Cuttack, Odisha, India

Introduction: Eosinophilic fasciitis (EF) is a rare fibrosing disorder, first described by Shulman (1974). Histopathology is the gold standard for diagnosis. We report a case of EF, where magnetic resonance imaging (MRI) was the modality for diagnosis.

Case Report: A 30-year-old male presented 6 months back with polyarthralgia of 1.5 months involving small and large joints with early morning stiffness of 30 min. There was no history of fever, inflammatory back pain, skin or eye complaints, or preceding diarrhea or dysuria. Investigations revealed hemoglobin-14 gm/dl; total leukocyte count-10500/cumm (N-56%, L-19%, and E-18%); TPC-2.2 lakhs/cumm; erythrocyte sedimentation rate (Westergren)-30 mm 1st h, C-reactive protein-0.1 mg/dl. Absolute eosinophilic count was 2153/cumm. Liver function tests and kidney function tests were normal. Rheumatoid factor, antinuclear antibody, anti-cyclic citrullinated peptide antibody, and human leukocyte antigen-B27 were negative. Subsequently, he developed painless swelling of bilateral lower limbs, which progressed proximally to involve bilateral calves. Clinical signs of deep venous thrombosis were absent. Thyroid function tests and urinalysis were normal. Few days later, he developed tightness over bilateral calves and bilateral upper limbs, especially over the forearms. There was no history of Raynaud's phenomenon. Skin over digits and hands was spared. General and systemic examinations were normal. However, “groove sign,” the tell-tale sign of eosinophilic fasciitis (EF), was present over bilateral forearms [Figure 1]. The patient was reluctant for skin biopsy. MRI left forearm revealed diffusely thickened superficial and deep fascial planes with pronounced contrast enhancement, suggestive of EF. Oral prednisolone 40 mg/day and methotrexate 10 mg/week were started. Currently, after 3 months of diagnosis, he has received prednisolone 20 mg/day and methotrexate 20 mg/day and has partially improved.



Discussion: Deep skin biopsy including fascia is compulsory for diagnosing EF. False-negative results are common. MRI, a noninvasive tool to detect fascial inflammation (T1-hypointense with enhancement on contrast; T2-hyperintense), has shown good correlation with tissue biopsy. MRI can guide site of biopsy. It helps assess improvement with therapy on follow-up.

Conclusion: MRI is an alternative to tissue sampling for the diagnosis of eosinophilic fasciitis.


  OPC0250: Detection of gut mucosal inflammation in spondyloarthritis: role of stool calprotectin Top


Saumya Ranjan Tripathy, Ashirbad Behera, Manoj Kumar Parida, Aditya Panda, Bidyut Kumar Das; SCB Medical College, Cuttack, Odisha, India

Background: The link between gut inflammation and spondyloarthritis is being increasingly established. Colonoscopy followed by biopsy is the gold standard for diagnosis and subsequent follow-up of gut inflammation. However, the procedure is invasive and cumbersome. Repeated colonoscopy for follow-up is also distressing for the patient. Calprotectin is secreted by neutrophils and monocytes infiltrating the inflamed gut.

Objectives: The objective of the study was to assess the role of stool calprotectin in predicting gut inflammation.

Methods: The study was carried out in the Department of Clinical Immunology and Rheumatology, SCBMCH, Cuttack, over a period of 8 months. This was a cross-sectional, observational study. Inclusion criteria were spondyloarthritis patients presenting with flare of arthritis following diarrhea/dysentery and presence of pus cells/red blood cells/occult blood in stool. Exclusion criteria were patients with presence of fissure-in-ano and hemorrhoids and those who not consenting for colonoscopy. All eligible patients were evaluated for the presence of stool calprotectin by fluoroenzyme immunoassay method by KT-843 kit manufactured by epitope diagnostics. Patients with high stool calprotectin (normal <50 mg/kg) underwent colonoscopy and biopsy of suspicious lesions if detected. The data were processed by SPSS-21 software.

Results: Out of 90 eligible patients recruited, 72 patients (80%) had high stool calprotectin levels. Out of 52 patients who gave consent for colonoscopy, 48 patients (92.3%) had positive findings requiring biopsy. One patient had diverticulosis. Biopsy reports revealed inflammatory bowel disease (IBD) in 18 patients (37.5%) and nonspecific inflammation in 16 patients (33.3%); one patient had infective colitis (2%) and no abnormality was detected in 14 patients. The mean stool calprotectin level in IBD was 976 ± 808.7 mg/kg versus 431 ± 278.3 mg/kg in nonspecific gut inflammation (P < 0.01).

Conclusions: Gut inflammation is high in patients with spondyloarthritis. Stool calprotectin is an excellent alternative test to colonoscopy for detecting gut inflammation in spondyloarthritis.


  OPC0251: Interstitial lung disease in systemic sclerosis: Prevalence, clinical characteristics, and serological profile in Indian patients Top


Dhaval Tanna, Wasim Kazi, Gayatri Ekbote, Dhiren Raval, Natasha Negalur, Durgarao Yadavalli, Rohit Bajaj, Shruti Bajad, Rajiva Gupta; Medanta-The Medicity, Gurgaon, Haryana, India

Introduction: Systemic sclerosis (SSc) is an autoimmune disease of unknown etiology. Pathogenesis of SSc is characterized by a triad of autoimmunity, vasculopathy, and fibrosis. Interstitial lung disease (ILD) is common in SSc and despite advancement in management is a leading cause of morbidity and mortality. Hereby, we conduct this study to assess prevalence, clinical characteristics, and serological profile of patients with SSc.

Methods: All patients diagnosed as having SSc as per the American College of Rheumatology/European League Against Rheumatism 2013 criteria were included. Patients having overlap syndrome were excluded. ILD was diagnosed based on high-resolution computed tomography and/or pulmonary function testing. After written informed consent, demographic profile, clinical features, laboratory and radiology parameters, and treatment details of patients were recorded. The study was approved by the institutional ethics committee.

Results: A total of 90 patients were included from June 2017 to August 2018. The prevalence of ILD was 74.8% in these patients. Nonspecific interstitial pneumonia was predominant ILD pattern observed (86.5%). Median disease duration in patients with ILD was 7 years. More patients in ILD group had diffuse phenotype as compared to patients without ILD (59.7% vs. 34.7%, P = 0.03). Only one-fourth patients with ILD were found to be having pulmonary arterial hypertension denoting mild-to-moderate nature of ILD in majority of patients. Out of various demographic and clinical feature studies, the presence of acroosteolysis and osteoporosis was significantly more in patients with ILD. Out of various autoantibodies studied, Scl70 was found to be significantly associated with ILD (71.4% vs. 23.8%, P = 0.003). Anticentromere antibody was found to be negatively associated with ILD (1.7% vs. 52.3%, P < 0.0001). Significantly, more patients in ILD group were treated with glucocorticoids and immunosuppressants [Table 1].
Table 1. Demographic and clinical characteristics

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Table 2: Comparison of clinical and serological characteristics in patients with and without ILD

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Conclusion: Around three-fourths of Indian patients with scleroderma have ILD. Diffuse phenotype, acroosteolysis, osteoporosis, and anti-Scl70 antibody are associated with the presence of ILD. Anticentromere antibody shows negative association with the presence of ILD.


  PC0084: Lower limb entheseal ultrasonography in the diagnosis of nonradiographic spondyloarthritis Top


Anupama Mundu, Ganu Salil, John Mathew, Debashish Danda; Christian Medical College, Vellore, Tamil Nadu, India

Background: Enthesitis is a characteristic feature of spondyloarthritis (Spa), and it may be the initial site of joint inflammation in Spa. Peripheral enthesitis may produce pain or may be asymptomatic and correlate poorly with clinical examination. Magnetic resonance imaging (MRI) is an important diagnostic tool in patients without definitive sacroiliitis on X-ray, and MRI could show active sacroiliitis that precede radiographic change by 3–8 years. MRI and ultrasonography are important tools for evaluating entheseal site inflammation. Because of high cost and accessibility of MRI, its use is restricted. Ultrasound can be used for identifying enthesitis early and hence can be used for early diagnosis of Spa.

Objective: The objectives of the study were to identify the prevalence of peripheral enthesitis in patients with nonradiographic Spa and to compare this with patients with radiographic primary ankylosing spondylitis and normal age- and sex-matched controls.

Methods: Three groups of individuals were identified nonradiographic Spa, radiographic Spa, and age- and sex-matched healthy controls. Two independent investigators assessed the entheseal site inflammation clinically and by B-mode ultrasound independently. Five lower limb entheseal sites were examined, viz., (i) superior pole of the patella – quadriceps tendon insertion, (ii) inferior pole of the patella – proximal patellar ligament insertion, (iii) tibial tuberosity – distal patellar ligament, (iv) superior pole of the calcaneus – Achilles tendon insertion, and (v) inferior pole of the calcaneus – plantar aponeurosis. The scoring was done according to Glasgow ultrasound enthesitis scoring system. The data were analyzed and comparison of clinical and ultrasonographic findings was made between nonradiographic Spa, ankylosing spondylitis, and healthy controls. Intraclass correlation was calculated to assess the agreement between the two raters. The comparison of these scores was done across diseased and normals using independent t-test or Mann–Whitney U-test. Clinical examination assessment will be compared to scoring obtained from ultrasonogram using receiver operating characteristic curve.

Results: Forty patients with nonradiographic and 40 patients with radiographic Spa have been recruited. Age- and sex-matched healthy controls are to be recruited. Analysis was also made after completion of recruitment.


  OPC0252: Anemia as a hematological manifestation of systemic lupus erythematosus with special reference to disease activity Top


Hitesh Deka, Prasanta Dihingia, T Karthikeyan; Assam Medical College and Hospital, Dibrugarh, Assam, India

Background: Systemic lupus erythematosus (SLE) is a prototype autoimmune disease characterized by the presence of numerous autoantibodies and multisystem involvement. Hematological abnormalities are quite common in SLE, and among these, anemia can be used as an indicator of disease activity and severity.

Aim and Objectives: The aim was to study anemia as a hematological manifestation of SLE and its correlation with the disease severity.

Materials and Methods: This study was an observational cross-sectional study done in Assam Medical College for 1 year. All cases who fulfilled the American College of Rheumatology Criteria 1997 who came to rheumatology outpatient department and those who admitted in the medicine ward were taken up for the study.

Results: Totally, 106 patients were taken up for the study. The male-to-female ratio in this study was 1:105. Mean duration of illness was 2.92 years. The most common constitutional symptom was fatigue that present among 57 (53.7%). Raynaud's phenomenon was present among 15 (14.15%) patients. Anemia (hemoglobin <12 mg/dl) was seen in 99 patients (93.4%) of the patients. Anemia of chronic diseases was the most common type of anemia with 79.2% followed by iron deficiency anemia (14.1%). Autoimmune hemolytic anemia was absorbed in 6.6% of the patients. There was a statistically significant negative correlation seen between SLEDAI and the hemoglobin (r =−.330, P = 0.001). There was also a significant positive correlation seen between the SLEDAI and serum ferritin (r = 0.209 P = 0.032). There is also significant mean difference seen between the hemoglobin and dsDNA, antinucleosome, antihistone, anti-Pm Scl, anti-Ku, and other autoantibodies.

Conclusion: Anemia, particularly anemia of chronic diseases, is one of the most common manifestations of the SLE. Hemoglobin and serum ferritin can be used as a disease activity marker. Anemia in SLE patients requires early diagnosis, careful monitoring, and prompt therapeutic intervention.


  OC0052: Metabolic syndrome in rheumatoid arthritis and its association with disease parameters: a hospital-based study from Northeast India Top


Phibakordor L Nonglait, Bhupen Barman, P K Bhattacharya; North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, Meghalaya, India

Background: Metabolic syndrome (MetS), a cluster of cardiovascular risk factors, is highly prevalent in patients with rheumatoid arthritis (RA). The aim of the study was to assess the prevalence of MetS in RA patients from Northeast India and evaluate its relationship with disease activity of RA.

Methods: The study was conducted on 104 RA patients (15 males, 89 females; mean age 43.83 ± 13.32 years) according to the 2010 revised American College of Rheumatology Classification Criteria, and 104 age- and sex-matched healthy controls (16 males, 88 females; mean age 38.19 ± 12.12 years). The frequency of MetS was assessed using consensus statement for diagnosis of MetS for Asian Indians. Disease activity of RA was assessed using disease activity score (DAS)28, and its relationship with MetS was explored using suitable statistical techniques.

Results: MetS was found in a significantly higher (χ2 = 10.1, DF = 1, P = 0.002) proportion of individuals in the RA group (35.58%) as compared to control group (16.35%). Patients with RA were more prone to harbor MetS as opposed to normal individuals (odds ratio = 2.81, 95% confidence interval = 1.47–5.53). The RA group was more likely to have higher frequency of the individual MetS components, viz., low high-density lipoproteins (75%), high triglyceride (44.23%), elevated waist circumference (31.73%), high fasting blood sugar (17.30%), and elevated blood pressure (14.42%) levels. Average DAS28 score of RA patients was 4.48 ± 1.43. Subgroup analysis showed that DAS28 scores were higher in RA patients with (MetS) (5.33 ± 1.32) than those without MetS (4.05 ± 1.28).

Conclusions: The frequency of MetS in RA is significantly higher compared to controls (P < 0.05). The disease severity of RA seems to be modulated by MetS too. These findings suggest that physicians should screen for MetS in patients with RA to reduce the risk of cardiovascular disease in these patients.


  PC0085: Maternal and fetal outcomes of lupus pregnancies: Collective effort by Karnataka rheumatologists Top


Vineeta Shobha, Ramya Janardana, Vikram Haridas, Yogesh Singh, Vijay K Rao, Ramesh Jois, Chandrashekara Srikantiah; St John's Medical College and Hospital, Arthritis Speciality Centre, Hubli, Manipal Hospital,Bangalore, Vikram Hospital, Vasanthnagar, Chan Re Rheumatology and immunology Centre, Bangalore, Karnataka, India

Introduction: Identifying ways to predict which lupus women will have successful pregnancy course, and the outcome is a research priority.

Objectives: The aims and objectives were to study the maternal and fetal outcomes of systemic lupus erythematosus (SLE) patients who were pregnant and the factors associated with adverse pregnancy outcome and to study the effect of pregnancy on SLE disease activity of these patients.

Methodology: A structured pro forma was developed to record pre- and post-pregnancy status, in this am bidirectional study to record the progress of pregnancy and maternal and fetal outcomes of SLE patients fulfilling SLICC criteria. These records were retrieved and the clinical details of pregnancies that were temporally associated with lupus disease were recorded.

Results: A total of 108 pregnancies in 76 SLE patients were reviewed. The mean age of the patients was 26.8 (±4.2) years. In majority 87/108 (80%), the diagnosis was made before pregnancy with a mean disease duration of 4.2 (±3.86) years. Largely patients were in clinical remission 95/108 (88%) at the time of conception. Antiphospholipid antibody positivity was seen in 40/108 (37%) of patients. Lupus nephritis anytime during the disease was found in 25/108 (23%) patients. Flares/disease diagnosis concurrent with pregnancy and persistently active disease occurring during antepartum and intrapartum period was associated with adverse fetal. Univariate analysis of factors influencing fetal outcome revealed significant association with disease activity during pregnancy or within 6 months ante/postpartum period, shorter disease duration, and prednisone dosage ≥7.5 mg/day.

Conclusion: Our cohort of SLE patients had adverse fetal outcome in more than half of the pregnancies. Flares/high/persistent disease activity in the antepartum and intrapartum period, shorter disease duration at pregnancy onset, and steroid dosage are significantly associated with adverse fetal outcome.


  PC0086: Chasing acute ophthalmoplegia: An atypical tale Top


Debaditya Roy, Arka Bairagya, Ratul Ghosh, Debarup Das, Kaushik Basu; Department of General Medicine, Medical College and Hospital, Kolkata, West Bengal, India

Neuropsychiatric systemic lupus erythematosus (NPSLE) is characterized by a wide range of clinical features and occurs in 14%–75% of SLE patients. Focal neurological manifestations, including oculomotor palsy, have been reported in 10%–35% of patients with NPSLE.[2],[3],[4] To us, a 25-year female presented with fever and headache for 3 weeks and sudden-onset drooping of left-sided eyelid for 2 weeks. She had no history of altered sensorium, dimness of vision, or painful swelling around the eye. She also complained of walking instability 5 days before admission. On examination, we found complete third and fourth cranial nerve palsy in her left eye along with fully dilated pupil. She had impaired posterior column sensation but no sign of hemiparesis. Cerebellar signs were absent. Considering differentials as aneurysm of posterior communicating artery, postinfective demyelination, and cavernous sinus thrombosis, we performed complete blood count with erythrocyte sedimentation rate (ESR), cerebrospinal fluid (CSF) examination, and magnetic resonance imaging (MRI) of the brain with contrast and MR angiography. However, all the reports were normal except a raised ESR and high protein in CSF. Schirmer's test was negative in opposite eye which ruled out Sjogren's syndrome. Finally, secondary vasculitis was suspected considering her chronic joint pain for the last 18 months, and relevant investigations showed antinuclear antibodies (ANA) positivity in serum and CSF, positive direct Coombs test, and positive anti-sm and antiribosmal. She was given injection methyl prednisolone 1 g per day for 5 days followed by injection cyclophosphamide single dose which showed significant improvement of ataxia, joint pain, and ptosis. Eventually, she was diagnosed with secondary systemic vasculitis due to SLE. Hence, in patients presenting with sudden-onset ophthalmoplegia along with fever and joint pain, it is prudent to consider central nervous system vasculitis due to connective tissue disorders such as SLE in background so that early diagnosis and treatment can be done to prevent permanent neurodeficit.


  OPC0253: Predictors of endothelial dysfunction and atherosclerosis in rheumatoid arthritis in Indian population Top


Ashit Syngle, Inderjeet Verma1, Pawan Krishan1; Cardio Rheuma and Healing Touch City Clinic, Chandigarh and Rheumatologist Fortis Multi Specialty Hospital, Mohali, 1Department of Pharmaceutical Sciences and Drug Research, Punjabi University, Patiala, India

Background: Cardiovascular (CV) disease is the leading cause of mortality in rheumatoid arthritis (RA). Dysfunction of the vascular endothelium is a hallmark of most conditions that are associated with atherosclerosis and is therefore an early feature in atherogenesis. Biomarkers for rapid evolution of CV complications would be highly desirable for risk stratification. Finally, predictive biomarkers for CV risk would allow tailoring therapy to the individual.

Objective: We assessed endothelial function and atherosclerosis utilizing carotid intima-media thickness (CIMT) in RA in the context of clinical and laboratory markers in Indian RA population.

Methods: We performed a prospective study of 35 consecutive RA patients and 25 age- and sex-matched healthy controls. Patients with traditional CV risk factors were excluded. Flow-mediated dilatation (FMD) as measures of endothelial function and CIMT as measures of atherosclerosis were assessed. Disease-specific measures, inflammatory measures, serum cytokines, serum nitrite, lipids, and endothelial progenitor cells (EPCs) were estimated.

Results: FMD was significantly lower in RA (6.53% ± 1.81%) compared to controls (10.77% ± 0.53%; P < 0.001). CIMT (mm) was significantly increased in RA (0.62 ± 0.17) versus controls (0.043 ± 0.07; P = 0.003). In RA patients, FMD% inversely correlated with CIMT, C-reactive protein (CRP), disease activity score-28 (DAS-28), tumor necrosis factor (TNF)-α, and serum nitrite and positively correlated with EPC. CIMT correlated with age, DAS-28, interleukin 6 (IL-6), high-density lipoproteins (HDL), and low-density lipoprotein (LDL) and inversely correlated with EPC.

Conclusions: In the present study, FMD and CIMT were impaired in RA, indicating endothelial dysfunction and accelerated atherosclerosis, respectively. CRP, TNF-α, serum nitrite, DAS-28, and depleted EPC population predicted endothelial dysfunction. Age, IL-6, HDL, LDL, and depleted EPC population predicted accelerated atherosclerosis.


  PC0087: Diffuse Alveolar Haemorrhage as a presentation in an ANCA Negative systemic small vessel Vasculitis – A Case Report Top


Karthikeyan Thangaraju, Gopal Bohra, Bharat Kumar; AIIMS Jodhpur, Rajasthan, India

Systemic vasculitis is a group of disorders with multisystem involvement and varied clinical presentation. Although antineutrophil cytoplasmic antibody (ANCA)-associated small-vessel vasculitis is more common, we present a 28-year-old female of systemic vasculitis with renal and pulmonary involvement without serological ANCA positivity. She was a known young-onset hypertension and presented to us with hemoptysis. The patient was found to have chronic kidney disease with diffuse alveolar hemorrhage (DAH). Renal manifestation was reduced urine output with average creatinine values of 5 mg/dl and ultrasound abdomen suggestive of bilateral small kidneys with poor corticomedullary differentiation and raised cortical echotexture. The patient also developed erythematous lesion over the fingertips and toes with a good peripheral palpable pulse and a normal peripheral vessel Doppler study. Pulmonary involvement was breathlessness with hemoptysis. High-resolution computed tomography and bronchoalveolar lavage were suggestive of DAH based on diffuse areas of consolidation with ground glass opacity and hemosiderin-laden macrophages. The patient was on maintenance hemodialysis for chronic kidney disease, was started on pulse steroid of methyl prednisolone 1 g/day for 3 days, and was induced with mycophenolate mofetil 2 g/day in two divided doses. Hemoptysis improved, but she is on maintenance hemodialysis at present with average creatinine levels of 3 mg/dl. In conclusion, ANCA-negative systemic vasculitis may have varied clinical presentation with expected improvement for DAH with less improvement on renal parameters once the irreversible renal damage occurs.


  OC0053: Histological predictors of renal flare in patients with lupus nephritis: significance of tubulointerstitial lesions Top


Rudra Prosad Goswami, Geetabali Sircar, Hiramanik Sit, Alakendu Ghosh, Parasar Ghosh; Department of Rheumatology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

Background: Lupus nephritis (LN) affects nearly 60% of patients with systemic lupus erythematosus. Up to 30% of them experience a flare of renal disease activity within first 5 years.

Objective: The objective of the study was to identify the clinical and histopathological predictors of flares of LN.

Methods: Eighty-two patients with biopsy-proven LN who underwent continuous follow-up between 2014 and 2018 were included in this study. Clinical and histological variables were tested for their association with the end-point of renal flares either early flare (induction failure or [InF]) or late flare (maintenance failure or [MnF]). Baseline tubulointerstitial inflammation (TI) was categorized as < or ≥25%; presence or absence of interstitial fibrosis and tubular atrophy (IFTA) was also noted apart from International Society of Nephrology (ISN)/Renal Pathology Society (RPS) LN class.

Results: Of the 82 patients (median age 26 years, interquartile range [IQR]: 21–30; median duration of disease 48 months [IQR: 24–72]), 14.6% had class III, 34.1% had class IV, 14.6% had class V, 17.1% had class III + V, and 19.5% had class IV + V LN. Induction regimens used were high-dose cyclophosphamide (3.2%), low-dose cyclophosphamide (4.8%), and mycophenolate (22%). Maintenance regimens used were azathioprine (65.9%) and mycophenolate in (34.1%). During a median follow-up period of 23 months (IQR: 17-46 months), 19.5% (16/82) experienced InF and 31.7% (26/82) experienced MnF. None of the variables were associated with InF. In univariate logistic regression, MnF was associated with TI (>25%) (odds ratio [OR]: 5.33, 95% confidence interval (CI): 1.42–19.9, P = 0.013). In multivariate analysis after controlling for baseline prednisolone dose, maintenance regimen, ISN/RPS class of LN, IFTA, age, and duration of disease; TI ≥25% was associated with MnF (OR: 15.72, 95% CI: 2.06–120.22, P = 0.008).

Conclusions: TI involving >25% is a significant predictor of late or maintenance failure in LN and is independent of baseline LN class or maintenance immunosuppression used.


  OPC0255: Profile of overlap myositis across India: Results from myoin registry (IRA myositis special interest group) Top


Ramya Janardana, Sravan Kumar Appani1, Latika Gupta2, Liza Rajasekhar1, Ramnath Misra2, Vineeta Shobha1; Department of Clinical Immunology and Rheumatology, St John's Medical College Hospital, Bengaluru, Karnataka, 1Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, 2Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Aims: The aim of the study was to describe the clinical characteristics, autoantibody profile, and outcome of overlap myositis (OM) in a registry-based cohort of idiopathic inflammatory myositis (IIM).

Methods: Data of OM is extracted from the MyoIn registry database. The diagnosis of OM was as per Troyanov et al. Patients with antisynthetase syndrome were excluded.

Results: Of patients in the cohort, 68 patients were classified as OM. Of these, 49 (71.2%) patients satisfied the classification criteria for another connective tissue disease (CTD) and 19 (28.7%) patients did not independently satisfy classification criteria of another CTD.Demographic details are recorded in [Table 1]. In patients with at least 6 months of follow-up and data recorded on response (n = 34), 22 patients had complete response and five patients had partial response at the last follow-up. There were 25 relapses recorded in 50 individuals with three individuals developing more than on relapse. When compared to the cohort of 113 IIM other than OM from the same registry, there was no significant difference with respect to mean age at diagnosis, median duration between onset of myositis and diagnosis, mean creatine phosphokinase value at presentation, steroid dosage at initiation, first steroid taper, and median time to achieve 0.15 mg/kg steroids. However, more patients in OM were likely to remain unclassified as per the American College of Rheumatology (ACR)/European League Against Rheumatism criteria for myositis as compared to the cohort with no overlap features (P = 0.014).



Conclusion: Equal number of lupus, scleroderma, and undifferentiated CTD overlapping with myositis were found in our cohort. Ribonucleoprotein is the most common autoantibody in the nonlupus myositis overlaps. More patients in overlap as compared nonoverlap IIM remained unclassified when ACR Classification was applied. Two-thirds of the patients had good functional outcome at the last follow-up.


  OPC0256: A randomized controlled trial to compare the efficacy of oral mycophenolate mofetil with placebo in patients with systemic sclerosis-related early interstitial lung disease Top


G S R S N K Naidu, M B Adarsh, Shefali Sharma, Varun Dhir, Sahajal Dhooria1, Anindita Sinha2, Sanjay Jain; Departments of Internal Medicine, 1Pulmonary Medicine and 2Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: Early initiation of immunosuppression in systemic sclerosis (SSc) patients with interstitial lung disease (ILD) might help in halting the disease process and improve long-term morbidity and mortality.

Objectives: The objective of the study was to determine the efficacy and safety of oral mycophenolate mofetil (MMF) in the treatment of SSc-related early ILD (forced vital capacity [FVC] ≥70% of predicted). The primary outcome was comparison of change in FVC after 6 months of therapy with MMF or placebo. The secondary outcomes were change in SF-36 v2 scores, Mahler dyspnea index (MDI), and adverse events profile of MMF and placebo.

Methods: This was a double-blind, randomized, placebo-controlled trial conducted at single center in North India. SSc-ILD patients with FVC ≥70% were randomized to receive either MMF (up to 2 g/day) or placebo for 6 months. FVC, diffusing capacity for carbon monoxide (DLCO), MRSS, SF-36 v2 scores, MDI, and 6-min walking distance (6MWD) were noted at baseline and 6 months. The trial was approved by the institutional ethical committee and registered at ClinicalTrials.gov (NCT02896205).

Results: Forty-one patients were included (MMF: 20, placebo: 21) with a mean age of 40.29 ± 10.13 years. Mean FVC at baseline was 81.71% ± 9.35%. Mean change in FVC was −1.79% ± 7.32% in MMF arm and 1.34% ± 4.47% in placebo arm. Mean absolute difference in FVC change between two arms was 3.13% (95% confidence interval, −1.02–7.28; P = 0.131). SF-36 v2 scores showed significant improvement in both groups. No significant difference was noted in mean change of MDI (3.33 vs. 2.79; P = 0.638), DLCO (2.46% vs. 2.31%; P = 0.412), and 6MWD (2.8 m vs. 12.11 m; P = 0.522). Mean change in MRSS was −4.73 with MMF and −1.53 with placebo (P = 0.042). MMF was well tolerated and rates of diarrhea and infections were similar to that with placebo. Three and one patients discontinued MMF and placebo, respectively.

Conclusions: In this small study, six-month therapy of MMF was not effective in stabilizing the lung disease in SSc-related early and mild ILD but was effective in controlling skin disease in these patients. MMF was well tolerated.


  OPC0257: Clinical profile of patients with systemic sclerosis in a cohort from Eastern India Top


Snehalata Devi, Saumya Ranjan Tripathy, Rina Tripathy, Rasmi Ranjan Sahoo, Bidyut Kumar Das; SCB Medical College, Cuttack, Odisha, India

Background: Systemic sclerosis (SSc) is a connective tissue disorder with varied presentations, which manifests in patients in various combinations of symptoms and signs.

Aim: The aim of the study was to evaluate the clinical profile of SSc.

Methods: Seventy-five consecutive patients of SSc attending the Department of Rheumatology, SCBMCH, Cuttack, Odisha, diagnosed on the basis of revised American College of Rheumatology Criteria 2013 were recruited. Patients were examined in detail, with specific focus on skin, lungs, and cardiovascular, musculoskeletal, and gastrointestinal system.

Results: Out of 75 SSc patients, diffuse-cutaneous SSc (dc-SSc) was more common (82%) than limited-cutaneous SSc (lc-SSc) (18%). The female:male ratio (3:1) and mean age was similar in both lc-Ssc (37.2 ± 9.8 years) and dc-Ssc (37.6 ± 11.7 years). The mean duration of illness at presentation in dc-SSc (2.6 ± 2 years) was earlier than in lc-Ssc (6.4 ± 5.8 years). Raynaud's phenomenon was the most common clinical feature (96%). Digital ulcers and pits (85%) were seen more commonly in SSc-dc. Digital gangrene was present in three patients (all with SSc-dc). The average MRSS score was 21 in dc-SSc and 14.45 in lc-SSc. Proximal myopathy was present in 34.6% of patients of SSc (21.4% of SSC-lc and 37.7% of SSC-dc). Gastrointestinal complaints (all had gastroesophageal reflux disease [GERD]) were present in six patients of SSc-dc and none in SSc-lc. One patient of SSc-lc had chronic renal failure. CREST syndrome was present in one patient. Interstitial lung disease (ILD) was present in 45.9% of patients with dc-SSc (46.4% had nonspecific interstitial pneumonia [NSIP] and 53.6% had usual interstitial pneumonia) and 50% patients with lc-SSc (all patients had NSIP). Echocardiographic evidence of pulmonary arterial hypertension (PAH) was seen in 35% of SSc patients (29.5% of patients with dc-SSc and 7.14% patients with lc-SSc).

Conclusion: In this cohort from Eastern India, dc-SSc is more common and present earlier than lc-SSc. Unlike traditional concepts, ILD is found equally in both dc-SSc and lc-SSc, and PAH occurs more commonly with dc-SSc.


  OC0055: PET - CT and PET-MR in evaluation of Rheumatoid Arthritis, Polymyalgia Rheumatica and Relapsing Polychondritis Top


Mandakinee Phukan; Cancer Hospital attached to Gauhati Medical College, Guwahati, Assam, India

Case Report: Several clinical studies of rheumatoid arthritis (RA) have demonstrated that fluorodeoxyglucose (FDG) uptake in affected joints reflects the disease activity of rheumatoid arthritis with strong correlation with clinical parameters. FDG positron emission tomography-computed tomography (PET/CT) helps in early monitoring of the response to RA therapy. FDG-PET/magnetic resonance imaging can be used in imaging of small joints. In polymyalgia rheumatica (PMR), FDG PET/CT reveals characteristic uptake by bursitis in ischial tuberosity, greater trochanter, and spinous process and thus helps in differential diagnosis. FDG PET/CT can also be used for evaluating associated large-vessel vasculitis. In relapsing polychondritis, FDG PET/CT reveals unique findings for chondritis in the auricular, nasal, and bronchial tree and thus promotes early diagnosis. 68-Gallium-DOTA-Siglec-9 PET tracer is a novel imaging tool for the detection of synovial inflammation. It is a leukocyte ligand of vascular adhesion protein-1. Eichendorff et al. reported that 68 Ga-anti-CD163 antibodies can be used in imaging of macrophages in inflammatory conditions. Folate receptor PET imaging with 68Ga-deferoxamine-folate is promising as a diagnostic and therapeutic tool in RA. Folate receptors are often overexpressed on activated macrophages.

Discussion: FDG uptake in inflammatory tissues occurs because of enhanced rate of glucose utilization in macrophages. Beeker et al. reported that PET is a suitable and quantitative method for clinically evaluating patients with RA including extra-articular manifestations. The visual FDG uptake score may be useful for evaluating arthritis in large joints. Rehak et al. concluded that FDG-PET/CT is an advantageous one-step examination for detecting different variants of PMR, for assessing the extent, severity, and excluding occult malignancy. Gallium-68-based tracers are suited because of availability from generator, relative cost-effectiveness, and ease of labeling.

Conclusion: There is a great potential for an increased role for PET to serve as a primary or complementary modality in the management of rheumatologic disorders.


  PC0088: Hughes–Stovin Syndrome: An incomplete Behcet's? Top


Rishabh Nanavati, C Balakrishnan, Abha Dubey, Balakrishna Padate; P. D Hinduja Hospital, Mumbai, Maharashtra, India

Introduction: Hughes–Stovin syndrome (HSS) is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. Here, we present three patients with HSS seen by us in our clinic in the last 2 years.

Results: All three patients were male. Median age of the patients was 28 years and median duration of disease was 24 months. Duration of follow-up of three patients was 1 month, 18 months, and 24 months, respectively. Dry cough, hemoptysis with DOE, and fever were present in all three patients. Oral and genital ulcers were seen in two patients. Uveitis, EN lesion, and superficial thrombophlebitis were observed in one patient each, and one patient had unilateral lower limb swelling suggestive deep venous thrombosis. All three patients had pulmonary artery aneurysm with partial thrombosis. One patient had renal vein, inferior vena cava, and common iliac vein thrombosis with abdominal aortic aneurysm. All three patients had increased acute-phase reactants. Human leukocyte antigen-B51 and thrombophilia profile were negative in all three patients. One patient had pulmonary hypertension on two-dimensional echocardiography. Computed tomography pulmonary angiography in all three patients showed pulmonary artery aneurysm. Positron emission tomography scan was done in one patient was normal with no active uptake. All three patients were started on pulse steroid followed by oral prednisolone. All three received cyclophosphamide initially as a steroid sparer followed by mycophenolate mofetil in two and azathioprine in one patient. One patient needed coiling of aneurysm. One patient is well on maintenance steroid and mycophenolate, one patient has just started therapy, and one is lost to follow-up.

Conclusion: Patients with HSS present with hemoptysis. Imaging does show pulmonary artery aneurysms. Therapy is with immunosuppressant medications. They may need emergent vascular intervention to stop hemoptysis. Although the exact relationship is not known, patients with HSS do have some clinical features of Behcet's disease.


  PC0089: Leflunomide in Takayasu's arteritis Top


Yogesh Preet Singh, Vaibhavi Velangi; Manipal Hospital, Old Airport Road, Bengaluru, Karnataka, India

Introduction: Takayasu's arteritis (TA) is a large-vessel vasculitis affecting aorta and its branches. Management of TA patients is challenging because of paucity of clinical symptoms and signs of disease activity and lack of reliable parameters to measure disease activity. The aim of the treatment is to suppress systemic and vascular inflammation using both glucocorticoids (GCs) and immunosuppressive agents (IS). There are no randomized trials comparing efficacy of different IS agents. Methotrexate (MTX), azathioprine (AZA), and mycophenolate mofetil (MMF) are commonly used the first-line IS agents. Leflunomide (LEF), tocilizumab, and anti-tumor necrosis factor agents have been used in refractory cases. Herein, we describe two cases of TA who were successfully treated with LEF.

Case Report: Case 1 was a 57-year-old female who presented with disease duration of 5 years. She was on deflazacort 6 mg per day and was intolerant to multiple IS agents (MTX, AZA, and MMF). She was started on LEF 20 mg per day. Case 2 was a 61-year-old female who presented with disease duration of 4 months. At presentation, she was not on any IS agents. Based on previous experience, LEF was started as the first line in combination with MTX. GCs were not used in the treatment. In both the patients, serial erythrocyte sedimentation rate and C-reaction protein normalized. Positron emission tomography-computed tomography repeated at 24 months (Case 1) and after 18 months (Case 2) showed significant regression of changes. The duration of treatment with LEF was 44 and 29 months, respectively. Both the patients did not have any LEF-related adverse effects.

Conclusion: LEF can be considered as an IS agent in refractory TA. There is need for further studies to evaluate the use of LEF in the treatment of TA either alone or in combination with other agents such as MTX.


  PC0090: Early Diagnosis predicts better therapeutic response in Rheumatoid Arthritis Top


Dhaiwat Shukla, Nibha Jain, Puja Srivastava, Sapan Pandya; Department of Rheumatology, VS Hospital, Ahmedabad, Gujarat, India

Background: Early diagnosis and treatment in rheumatoid arthritis (RA) is associated with less morbidity and better outcomes.

Methods: All consecutive patients of RA (diagnosis based on the American College of Rheumatology 2010 Criteria) attending outpatient department at VSGH were included in the study. Patients are divided into two groups based on their duration of illness (early <2 years and late >2 years). Their clinical profile was recorded in predefined case record forms (laboratory investigation and disease activity parameters [health assessment questionnaire (HAQ) and disease activity score in 28 (DAS28)] and erythrocyte sedimentation rate/C-reactive protein]). Disease activity was re-assessed at 6 months of follow-up and difference in disease activity was compared between early and late RA.

Results: Both the groups early as well as late RA were comparable at baseline in terms of age, gender, treatment profile, DAS28, and HAQ scores. However, significant difference was noted at 6 months of follow-up in DAS28 and HAQ scores, both being higher in late RA. Patients diagnosed early responded better in terms of patient-reported outcome and DAS28.



Conclusion: Early diagnosis and treatment are associated with better outcomes. The differences are remarkable even as early as 6 months.


  PC0091: Essential thrombocytosis mimicking as Buerger's disease Top


Hrudananda Bhuyan, Anchal sahoo, Manoj Kumar Parida, Saumya Ranjan Tripathy, Bidyut Kumar Das; SCB Medical College and Hospital, Cuttack, Odisha, India

Introduction: We present a case diagnosed initially as Buerger's disease who after 6 years was established to have essential thrombocytosis (ET).

Case Report: A 35-year-old male presented with claudication and pain of left foot with bluish discoloration of toes followed by similar complaints in the right foot 6 months later. With a positive history of smoking, absence of other thrombophilic causes, and demonstration of reduced blood flow in lower limb arteries and ultrasonographic impression, diagnosis of Buerger's disease was made. The patient was discharged on antiplatelets and instruction to abstain from smoking. For the next 6 years, he was relatively asymptomatic. For the last 1 month, there was relapse of symptoms of claudication only in the left lower limb. On re-evaluation, the patient was found to have absent dorsalis pedis, posterior tibial, popliteal, and femoral pulse bilaterally with prominent abdominal aortic pulsation. Hematological evaluation revealed normal inflammatory markers (erythrocyte sedimentation rate and C-reactive protein), hemoglobin-16.3 gm/dl, total leukocyte count-15,610/cumm, and platelet counts of 11.02 lakhs/cumm. Liver function test and renal function test were normal. Bone marrow aspiration and biopsy showed increased numbers of megakaryocytes with large, hyperlobulated nucleus with few showing Staghorn configuration, suggestive of ET. JAK2V617F mutation was positive confirming the diagnosis of ET. Ultrasonography showed splenomegaly. Arterial Doppler and computed tomography angiography demonstrated thrombosis of the lower abdominal aorta, bilateral common iliac arteries (R > L) with extensive collaterals. The patient was started on hydroxyurea 500 mg OD along with antiplatelets.

Discussion: Buerger's disease presents in young individuals with a history of smoking with limb claudication. On the other hand, ET is a nonreactive chronic myeloproliferative disease with sustained megakaryocytic proliferation with increased circulating platelets which can have both thrombotic and hemorrhagic manifestations.

Conclusion: ET must be included in the differential of thrombotic conditions. Total platelet counts can help suspect ET early and avoid late diagnosis.


  OPC0286: Retrospective study of long-term course and outcome of idiopathic inflammatory myopathies: A tertiary care teaching center experience Top


Aswin M Nair, Ruchika Goel, Pramod Chebbi, Ashish Mathew, Arvind Ganapati, John Jude1, Debashish Danda; Departments of Clinical Immunology and Rheumatology and 1Microbiology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of disorders with variable clinical profiles and outcomes across various populations.

Objective: The objective was to study the long-term outcome of patients with IIM at Christian Medical College, Vellore.

Methods: Patients aged ≥18 years with diagnosis of IIM, seen as outpatients and inpatients between January 2010 and July 2018, were identified from our institutional electronic records. Baseline and follow-up data on clinical profile, laboratory parameters, treatment details, as well as outcome data of patients having completed at least 1 year of follow-up were analyzed. Depending on the clinical response, the patients were grouped into complete, incomplete, and non-responders.

Results: Of 334 patients with IIM identified, 167 (50%) with follow-up data available were included. Mean (standard deviation) age of these patients was 38.6 (11.79) years with two-thirds being females (112 [67%]). Median disease duration before diagnosis was 9 (3–18) months. Dermatomyositis constituted majority of our patients (99 [59.3%]). Interstitial lung disease was seen in 33.5%, and anti-Mi2 (17.4%) was the most frequently encountered myositis-specific antibody. Methotrexate (44.9%) and mycophenolate mofetil (MMF) (37.7%) were the common immunosuppressants used at baseline. During a median follow-up duration of 33 (interquartile range [IQR]: 22–56) months, median MMT 8 improved from 54.4 (IQR: 45.8–68.5) to 80 (IQR: 79–80) and serum creatine phosphokinase creatine phosphokinase levels decreased from 411 (IQR: 87–2094) U/L to 110 (IQR: 52.8–192.5) U/L (P < 0.0001). Median steroid intake reduced from 50 (IQR: 35–60) mg/day to 2.5 (IQR: 0–7.5) mg/day (P < 0.0001). Overall, complete responders comprised 100 (59.9%), incomplete responders 65 (38.9%), and non-responders 2 (1.2%) patients. Discontinuation of steroids and second-line agent was feasible in 8 (8%) of complete responders. Malignancy was diagnosed in 4 (2.4%) patients and one expired during follow-up.

Conclusion: Long-term survival was good in our cohort of IIM with majority responding adequately to therapy.


  OPC0259: Acute pancreatitis in systemic lupus erythematosus: A multicentric retrospective analysis Top


Hafis Muhammed, Mohannad Irfaan Korvi1, Sheba Charles2, Preksha Dwivedi3, Pallavi Pimpale Chavan4, Amit Sharma5, Avinash Jain, Liza Rajasekhar1, Vineeta Shobha2, Varun Dhir3, Aman Sharma3, Ramnath Misra, Amita Aggarwal; Sanjay Gandhi Postgraduate Institute, Lucknow, Uttar Pradesh, 1Nizam Institute of Medical Sciences, Hyderabad, Telangana, 2Saint John's medical college, Bengaluru, Karnataka, 3Postgraduate Institute of Medical education and research, Chandigarh, 4Jaslok hospital and research center, Mumbai, Maharashtra, 5Fortis Escorts Hospital, Jaipur, Rajasthan, India

Introduction: Acute pancreatitis (AP) is a rare (0.1%) manifestation of systemic lupus erythematosus (SLE) and has significant mortality. Thus, we studied its presentation and outcome in patients with SLE seen in centers across India.

Methods: On behalf of SLE-SIG group, clinicians were invited to send data of patients with SLE who had AP in the past. Diagnosis of AP required the presence of two of the following three criteria: acute-onset epigastric pain, elevation in serum lipase or amylase (≥3 times normal), and characteristic findings of AP on imaging.

Results: There were 47 episodes of AP in 45 patients (40 females, mean age 26.2 ± 9.3 years). 97.6% had active lupus (SLE disease activity index [SLEDAI] > 4) with one-third having nephritis and one-fourth having central nervous system disease. 57.4% developed AP within 1 year of diagnosis of SLE. Mean SLEDAI was 17.4 ± 7.6, and 29 patients were on corticosteroids, four on cyclophosphamide, three on azathioprine, and two on mycophenolate mofetil. Among known factors, methotrexate, furosemide, gallstone, and alcohol were implicated in one patient each. All had abdominal pain while 55.3% had abdominal distension and 10.6% had jaundice. Thirty-seven patients had elevated enzymes and 40 had evidence of AP on imaging. Twenty-four patients had mild, 6 had moderate, and 17 had severe AP (Atlanta Classification). There was no difference in lupus features or activity between the three groups. All received immunosuppression intravenous methylprednisolone or dexamethasone (68.1%), cyclophosphamide (34.0%), or oral prednisolone (93.6%). Three patients had necrotizing pancreatitis, 15 had abdominal collections, 13 each had ascites and pleural effusion, 11 had acute respiratory distress syndrome, and 8 had sepsis (3 culture positive) as complications. Eight patients with severe AP died of multiorgan failure. On follow-up, two patients developed diabetes while one had malabsorption.

Conclusions: Pancreatitis is an early manifestation of SLE and is associated with active disease. High mortality rate is seen particularly with severe pancreatitis.


  OPC0260: Rheumatoid arthritis - high prevalence of Epstein-Barr virus and association with severitry of disease Top


Samir Kumar Hota, Saumya Ranjan Tripathy, Prakash Kumar Sahoo, Aditya Panda, Manoj Parida, Bidyut Kumar Das; SCB Medical College Cuttack, RMRC Bhubaneswar, Khallikote University, Bhubaneswar, Odisha, India

Background: Epstein–Barr virus (EBV) has been hypothesized to be one of the etiologic agents for rheumatoid arthritis (RA). However, the role of EBV in severity of disease in RA has been explored to a limited extent.

Aims and Objectives: The objectives were to determine the prevalence of EBV infection in RA and to compare the severity of RA between patients with EBV-positive and EBV-negative status.

Materials and Methods: In this pilot study, 25 patients with RA admitted in the Department of Rheumatology, SCBMCH, during January–March 2018, fulfilling the American College of Rheumatology/European League Against Rheumatism Criteria for RA were recruited. Twenty age- and sex-matched healthy controls were recruited for comparison. Anti-EBV immunoglobulin M (IgM) antibodies were used to detect the presence of EBV infection. Erythrocyte sedimentation rate (ESR) (Westergren), C-reactive protein (CRP) (quantitative), disease activity score in 28 (DAS-28), and health assessment questionnaire (HAQ) were used to assess RA disease activity.

Results: Twenty-five patients with RA and 20 healthy controls were recruited. The mean age of patients was 42.5 ± 11.9 mg/dl. The female:male ratio was 4:1. Out of 25 RA patients, 11 (44%) were positive for EBV-IgM antibodies versus 3 (15%) in controls (P < 0.01). The DAS-28 in EBV-positive patients and EBV-negative patients was 6.95 ± 0.75 and 6.59 ± 0.35, respectively (P > 0.05). HAQ score was higher in EBV-positive patients compared with EBV-negative patients (2.18 ± 0.17 vs. 1.85 ± 0.20) (P < 0.001). ESR (87.27 ± 38.02 vs. 75.36 ± 24.03), CRP (69.97 ± 53.79 vs. 57.74 ± 52.16) and titer of rheumatoid factor (117.3 ± 67.17 vs. 77.39 ± 73.54) were higher in EBV-positive patients compared with EBV-negative patients although statistical significance was not reached. Extra-articular manifestations (rheumatoid nodules) were more common in patients with EBV-positive status (n = 4) compared with patients with negative EBV (n = 1). Interstitial lung diseases were present in one patient each with EBV-positive and EBV-negative status.

Conclusion: Patients with RA who are positive for EBV have higher disease activity, poorer quality of life, higher degree of inflammation (ESR and CRP), and more severe disease.


  OPC0276: Multicentric Castleman's disease with lymphocytic interstitial pneumonia: Diagnostic challenge Top


Sonal Mehra, Raman Arora, Esha Kaul, Roopam Deka, Suhas Singla; Jaypee Hospital, Noida, Uttar Pradesh, India

Case Report: A 54-year-old female presented with a disease duration of 2 years of low-grade fever, polyarthralgia, abdominal pain, weight loss 22 kg, and sicca with progressive dyspnea. On evaluation, there was pallor with bilateral crepts with hepatomegaly and splenomegaly. Hb 10.3 g/dl and total counts 3050/cumm and ALC 610/cumm, erythrocyte sedimentation rate 80 and platelets 1.82lac. High-resolution computed tomography showed lymphoid interstitial pneumonia pattern pneumonia. The positron emission tomography showed mediastinal, retroperitoneal, and cervical lymphadenopathy with pleural thickening, hepatomegaly, and gross splenomegaly. The possibilities considered were sarcoidosis, lymphoproliferative disorder, tuberculosis, connective tissue disease, or IgG4 disorder. Antinuclear antibody was 1:80 cytoplasmic speckled and extractable nuclear antigen Sm/u1rnp ro-52 and histone positivity. Complements and anti-dsDNA were normal. Serum angiotensin-converting enzyme was raised 95. Direct Coomb's test was positive. The rheumatoid factor and anti-cyclic citrullinated peptide were negative. Serum IgG4 and anti-neutrophil cytoplasmic antibody were negative. Mantoux and viral markers were negative. The urine routine was normal. The immunofixation electrophoresis showed monoclonal band immunoglobulin M positive with lambda band. Lymph node biopsy showed features of hyaline vascular variant of Castleman's disease. The pleural nodule biopsy showed microgranulomas with lymphomononuclear cell infiltrate with epithelioid histiocytes with Ziehl–Neelsen stain negative. Bone marrow biopsy was normal cellularity with few aggregates of lymphoid cells, and reticulin fibrosis and flow cytometry showed increase in B-lymphoid cells with dual expression of light chains likely a clonal B-cell population.

Discussion: The patient was treated as a multicentric Castleman's disease. This presents with fever, night sweats, fatigue, weight loss, diffuse lymphadenopathy, hepatosplenomegaly, and pleural effusion/effusion of serous cavities. Some cases may have TAFRO syndrome with thrombocytopenia, anasarca, myelofibrosis/fever, renal dysfunction/reticulin fibrosis, and organomegaly. Lymphocytic interstitial pneumonia is seen in HIV, primary Sjogren's syndrome, and other connective tissue diseases. She received rituximab 2 g and steroids.

Conclusion: Multicentric Castleman's disease can mimic autoimmune connective tissue and false-positive immunological tests should be interpreted in context with tissue biopsy, immunohistochemistry and clinical profile for optimal diagnosis and management.


  OPC0263: Role of PET scan in IgG4 related disorders Top


Sonal Mehra, Suhas Singla, Raman Arora, Jaypee Hospital, Noida, Uttar Pradesh, India

Case 1: A 56-year-old male, with a disease duration of 7 months of lacrimal gland enlargement, presented with enlarged submandibular and parotid glands. Renal biopsy showed hypocomplementemia tubulointerstitial nephritis with IgG4-positive cells, storiform fibrosis, and palisaded interstitial fibrosis with kappa light-chain monoclonal gammopathy (kappa/lambda ratio 9.17). Serum IgG4 was 479 mg/dl. He received rituximab with prednisolone followed by mycophenolate mofetil (MMF). Follow-up positron emission tomography (PET) scan [Figure 1] showed significant improvement, kappa/lambda ratio reduced to 1.6, and IgG4 became normal.



Case 2: A 42-year-old male with a disease duration of 6 years of dyspnea and stridor. PET showed constrictive pericarditis with pseudotumor of the right ventricle with tracheal stenosis and fibrosing mediastinitis and pulmonary artery stenosis. The IgG4 level was 116.5 mg/dl (4–85). He improved on prednisolone 40 mg.

Case 3: A 42-year-old female with a disease duration of 17 years presented with weight loss and abdominal pain. She was diagnosed with retroperitoneal fibrosis with recurrent hydroureteronephrosis. Biopsy showed retroperitoneal fibrosis and few lymphoplasmacytic infiltrations and serum IgG4 posttreatment which was normal. She was treated with MMF and steroids.

Case 4: A 20-year-old male with a duration of 4 years with recurrent abdominal pain diagnosed with two episodes of autoimmune pancreatitis with raised serum IgG of 4 295 mg/dl.

Case 5: A 40-year-old female with a disease duration of 2 months presented with low-grade fever, weight loss, loss of apetite, and polyarthralgia. Magnetic resonance cholangiopancreatography showed chronic atrophic-calcific pancreatitis. Serum IgG4 was 409 mg/dl. She is on pancreatic enzyme replacement therapy.

Discussion: IgG4-related disorders present autoimmune pancreatitis, sclerosing cholangitis, hilar lymphadenopathy, sialoadenitis, retroperitoneal fibrosis, interstitial pneumonia, and prostatitis, and the 30%–40% of them relapse despite steroid therapy. 18F-labeled fluoro-2-deoxyglucose (F-FDG) PET/computed tomography (CT) can detect lesions sensitively because of abundant of inflammatory cells.

Conclusion: F-FDG PET/CT is a useful tool for assessing organ involvement, monitoring therapeutic response, and guiding interventional treatment of IgG4-related disorder and assessment of relapses.


  OPC0275: Prevalence of ILD In Lupus And Its Serological And Systemic Association: A Cross Sectional Study at a Tertiary Care Centre In India Top


Lucky Sharma, Dhiren Raval, Muzaffar Bindro, Gayatri Ekbote, Natasha Negalur, Dhaval Tanna, Wasim Kazi, Durga Rao, Rohit Bajaj, Rajiva Gupta; Department of Rheumatology and Clinical Immunology, Vedanta, The Mendicity

Introduction: Systemic lupus erythematosus (SLE) is a heterogeneous disease of unknown etiology. Pulmonary involvement in isolation or along with the involvement of other organ systems in SLE is not uncommon. The prevalence of interstitial lung disease (ILD) is however low (3%–15%) in lupus than in other connective tissue diseases. ILD in patients with lupus is usually mild; however, it can be progressive in some patients keeping such patients at increased risk of morbidity and mortality. Thus, the objective of our study is to determine the prevalence of ILD and its clinical and serological association as well.

Methods: This is a cross-sectional observational study conducted between September 2015 and July 2018 at Medanta, The Medicity. All SLE patients who fulfilled the American College of Rheumatology 1997 Criteria who underwent high-resolution computed tomography (HRCT) chest were included in the study. The demographic, clinical, and serological profile was recorded and reviewed. Ethical approval was taken from the institutional review board.

Result: Out of the 356 SLE patients, 289 who underwent HRCT were enrolled. There were 42 (14.5%) males and 247 (85.5%) females. The prevalence of ILD was found to be 7.9% (n = 23/289) in our cohort. Anti-SSA Ro 60KD was found to be positive in 12 patients with ILD (n = 12/23); however, it was not statistically significant. There was no statistical difference noted in other serological profile of patients with/without ILD. We did not find any difference in the manifestations of the observed systems in patient with/without SLE. Out of the 23 patients who had ILD, only three patients had pulmonary arterial hypertension (PAH) and 20 patients had isolated ILD with normal pulmonary arterial systolic pressure, though it was not found to be statistically significant. The tables are attached herewith.

Conclusion: The prevalence of ILD was 7.9% in our lupus cohort. There was no statistically significant correlation with serology, clinical manifestations of various systems observed, and PAH.




  OPC0266: Adjunct Vitamin D therapy maintains low SLE disease activity state Top


Mohapatra S, Tripathy R, Panda AK, Parida M, Tripathy SR, Das BK; SCB Medical College Cuttack, Khalikhote University Berhampur, Odisha, India

Introduction: Various autoimmune disorders (multiple sclerosis, autoimmune type 1 diabetes, rheumatoid arthritis, systemic lupus erythematosus [SLE], etc.) have been associated with Vitamin D deficiency. Negative correlation between serum Vitamin D levels and SLE disease activity has been shown in previous studies.

Aims and Objectives: The aim of the study was to assess the disease activity in SLE patients receiving adjunct Vitamin D in addition to standard care of treatment.

Methodology: A total of 251 patients who fulfilled the SLICC criteria for SLE, on standard care of treatment and adjunct 1000 IU Vitamin D daily, were included. Overlap syndromes were excluded. One hundred age- and sex-matched healthy controls not receiving Vitamin D were included for comparison. Serum Vitamin D was measured using competitive ELISA. SLE disease activity index (SLEDAI)-2K, serum C3 and C4, and serum anti-dsDNA were used to assess SLE disease activity.

Results: Out of 251 patients, 241 were females and 10 males (female:male = 24:1). Mean age was 29.5 ± 8.8 years. The clinical profile of SLE patients in this study included a history of mucocutaneous manifestations in 87.4% of patients, nephritis in 64.5%, neuropsychiatric lupus in 20%, serositis in 4%, and cardiovascular involvement in 4%. The mean duration of the disease was 56.1 ± 41.2 months. The mean SLEDAI-2K score was 2.1 ± 2.43. Mean SLICC/American College of Rheumatology damage index was 0.33 ± 0.63.The mean serum Vitamin D level in SLE patients (21.3 ± 13.8 ng/ml) was higher than in controls (12.4 ± 10.2 ng/ml) (P < 0.001). Serum C3 and C4 levels were 93.3 ± 45.8 mg/dl (normal 75–180 mg/dl) and 18.3 ± 10.97 mg/dl (normal 10–40 mg/dl), respectively. The mean serum dsDNA was 97.95 ± 123.2 IU/ml (normal < 100 IU/ml). Serum Vitamin D correlated positively with serum C3 (r = 0.09; P = 0.20) and serum C4 (r = 0.122; P = 0.11) and negatively with SLEDAI-2k (r = −0.02; P = 0.85) and serum anti-dsDNA (r = −0.01; P = 0.95).

Conclusion: Vitamin D would provide a low cost, lesser risk, adjunct to standard care of therapy and help patients have a better life.


  OPC0267: Scleroderma renal crises: High mortality despite angiotensin-converting enzyme inhibitors in India Top


Preksha Dwivedi, Adarsh M B, Nupoor Acharya, Raja Ramachandran1, Ritambara Nada2, Shefali Sharma, Aman Sharma, Sanjay Jain, Varun Dhir; Departments of Internal Medicine, 1Nephrology and 2Pathology, PGIMER, Chandigarh, India

Background: Scleroderma renal crisis (SRC) is a severe and life-threatening manifestation of systemic sclerosis (SSc). Even with use of angiotensin-converting enzyme (ACE) inhibitors and dialysis, the outcome in western centers shows mortality of 65% at 5 years. There are limited data from India.

Objective: The objective of the study was to assess outcome of SRC.

Methods: In single-center, retrospective study, we analyzed the clinical characteristics, triggering factors, treatment, and outcome of SRC. Records of all SSc patients (diagnosed by American College of Rheumatology 2013 Criteria) who were admitted in PGIMER, Chandigarh, in the last 5 years (2014–2018) were scrutinized and patients with SRC were included. Updated Consensus Classification (2014) was used to define SRC patients.

Results: Out of 94 admissions of SSc during 5 years, 15 patients had SRC. Mean (±standard deviation) age at diagnosis was 47.3 ± 9.7 years, with 14 patients of diffuse cutaneous SSc. Mean disease duration was 26.5 ± 28.3 months, with six patients having <1 year. Ten patients had a history of exposure to steroids in the prior 3 months, with a median dose of 30 mg (range 5–60 mg) with seven being on dose more than 20 mg. Pericardial effusion was present in five patients with dilated cardiomyopathy in two. Seizures and hypertensive retinopathy were present in two and five patients, respectively. Mean systolic and diastolic blood pressure at diagnosis was 160.93 ± 26.9 mmHg and 92.4 ± 12.4 mmHg, respectively. Mean serum creatinine was 6.12 ± 3.3 mg/dl. Evidence of thrombotic microangiopathy was present in five patients. All patients received ACE inhibitors and ten patients required dialysis. During the follow-up, ten died within 2 years with mean survival being 13.1 months (95% confidence interval 7.1–19.0 months) .

Conclusion: SRC occurs as an early manifestation in SSc. Prior steroid use was found in most patients. Despite the use of ACE inhibitors, two-third patients died in 2 years.


  OPC0269: Lupus nephritis in a pediatric lupus patient cohort Top


Deepak Rath, Pradyot Sinhamahapatra, Parasar Ghosh, Alakendu Ghosh; Department of Rheumatology, IPGMER and SSKM Hospital, Kolkata, West Bengal, India

Background: Childhood-onset lupus is a challenge for a diagnostician and for a clinician treating a patient. The disease has a multifaceted presentation with a multitude of organ involvement. The patient has multiple visits to varying specialists before a unifying diagnosis is made.

Objectives: The objectives were to identify the varying organ involvements in the patients diagnosed as childhood-onset lupus and to identify factors predicting renal involvement.

Methods: Sixty patients discharged with diagnosis of systemic lupus erythematosus (SLE), aged below 17 years of age, between December 2015 and July 2018 were identified and the discharge tickets were evaluated. Data were extracted and analyzed.

Results: Six males and 54 females were identified with a diagnosis of SLE at the time of discharge. The patients were diagnosed on an average of 6 months after symptom onset. Ninety-two percent of the patients had fever followed by malar rash-80%, oral ulceration-65%, lymphadenopathy-(42%. Constitutional features were present in 93% of the patients, while hematologic manifestations were seen in 70%. Renal involvement was observed in in 75%. Sixty percent of patients underwent renal biopsy (4 of 6 males, 32 of 54 females).

Conclusions: Class IV lupus nephritis was the most common biopsy finding (42%), followed by Class II (22%) and then Class III/V. All males had proliferative lupus nephritis, while 66% of females undergoing biopsy had proliferative lupus nephritis.


  OPC0271: The profile of cardiovascular involvement in SLE at a tertiary care centre from eastern India Top


Sweekruti Jena, Mrunmaya Ricki Jena, Rina Tripathy, Saumya Ranjan Tripathy, Aditya Panda, Manoj Parida, Bidyut Kumar Das; SCB Medical College Cuttack, Khallikote University, Berhampur, Odisha, India

Background: The cardiovascular system is affected in systemic lupus erythematosus (SLE) by disease itself, state of chronic inflammation, and side effects of treatment given.

Aims and Objectives: The aims and objectives were to find the burden of cardiovascular involvement in SLE and to correlate cardiovascular manifestations with SLE disease activity (SLE disease activity index [SLEDAI]-2K) and damage (SLICC/American College of Rheumatology [ACR] damage index).

Materials and Methods: Seventy-five consecutive SLE patients between 15 and 55years, with disease duration <5 years, admitted to the department of Rheumatology ward, SCBMCH, were included. Patients with overlap syndromes, history of cardiac disease, end-stage renal disease, chronic liver disease, or type 2 diabetes were excluded. Clinical examination, fasting serum lipid profile, electrocardiography (ECG), two-dimensional-echocardiography (2D-ECG), carotid intimal-media thickness, (CIMT) and serum troponin-I were used to assess the cardiovascular status of the patients.

Results: The mean age of patients was 28.5 ± 7.9 years. The female:male ratio was 14:1. The mean SLEDAI was 7.3 ± 4.9 and mean SLICC/ACR damage index was 0.78 ± 1.2. The most common autoantibodies found in patients with cardiovascular involvement were antinucleosome and anti-Ro52 antibodies. Raised systolic blood pressure (BP) was found in 42% and raised diastolic BP in 28% patients. Increased serum low-density lipoprotein, hypertriglyceridemia, and low serum high-density lipoproteins were found in 29%, 47%, and 51% patients, respectively. ECG revealed tachycardia in 32% while bradycardia, left axis deviation, and right atrial abnormality were found in one patient each. 2D-ECG revealed pericardial effusion in 15%. Aortic valves, mitral valves, and tricuspid valves were affected in 1.33%, 10.66%, and 6.66% patients respectively. Systolic dysfunction, diastolic dysfunction, and cardiomyopathy were found in 2.66% patients each. Pulmonary arterial hypertension was detected in 5.33% patients. None had evidence of vegetation. No increase in CIMT or serum troponin-I was detected. SLE patients with cardiovascular involvement had higher SLEDAI-2K (P = 0.002) and SLICC-ACR damage index (P = 0.01) compared with patients without cardiovascular involvement.

Conclusion: Cardiovascular involvement is associated with high SLEDAI-2K and SLICC/ACR damage index. The presence of antinucleosome and anti-Ro52 antibodies may predispose to cardiovascular involvement.


  OPC0272: Systemic sclerosis: Serum transforming growth factor beta-1 and severity of disease Top


Swati Parida, Snehalata Devi1, Rina Tripathy2, Rasmi Ranjan Sahoo3, Aditya Kumar Panda4, Saumya Ranjan Tripathy5, Bidyut Kumar Das5; Departments of General Medicine, 1Medicine, 2Biochemistry and 5Rheumatology, SCB Medical College, Cuttack, Odisha, 3Department of Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, 4Centre for Life Sciences, Central University of Jharkhand, Ranchi, Jharkhand, India

Background: Transforming growth factor beta-1 (TGF-β1) is suggested to play an important role in the pathogenesis of systemic sclerosis (SSc). TGF-β1 stimulates synthesis of extracellular matrix, fibroblast proliferation, and enhances matrix stiffness.

Objectives: The objective was to correlate serum TGF-β1 with various clinical manifestations of SSc.

Materials and Methods: Patients with SSc attending the Rheumatology Department, SCB Medical College, fulfilling the American College of Rheumatology Criteria (2013) for SSc were recruited for this study. Exclusion criteria included patients with overlap syndromes such as SSc with dermatomyositis, systemic lupus erythematosus, Sjogren's syndrome, and rheumatoid arthritis. Eight healthy controls were recruited for comparison. TGF-β1 levels in the plasma were quantified by ELISA and data were analyzed by GraphPad Prism version 7.05.

Results: Seventy-five patients (56 females; 19 males) were recruited. Diffuse cutaneous SSc (SSc-dc) was present in 61 patients (81%) and limited cutaneous SSc (SSc-lc) in 14 patients (19%). Serum TGF-β1 in patients with SSc was 649 ± 636.1 pg/ml versus 1118 ± 584.3 pg/ml in healthy controls (P < 0.05). In SSc patients with severe skin involvement (modified Rodnan skin score [MRSS] ≥20), there was higher concentrations of circulating TGF-β1 than those with mild-to-moderate skin involvement (MRSS <20) (P = 0.28). There was a positive correlation between serum TGF-β1 levels and MRSS (r = 0.07) (P = 0.58). SSc patients with low-density lipoprotein (ILD) had higher serum TGF-β1 levels than patients without interstitial lung disease (ILD) (P = 0.19). Serum TGF-β1 was significantly higher in SSc patients with pulmonary arterial hypertension (PAH) compared with patients without PAH (P = 0.01). There was no significant difference in TGF-β1 levels in patients with or without antitopoisomerase antibody (P = 0.86).

Conclusion: Serum TGF-β1 is lower in SSc than healthy individuals. We hypothesize that in active disease, TGF-β1 is sequestered by binding with TGF-β1 receptors. However, patients with more severe disease as reflected by the presence of higher MRSS, ILD, and PAH had higher levels of serum TGF-β1.


  PC0092: Adult-onset Still's disease with myocarditis: An unusual complication Top


Anchal Sahoo, Sai Swaroop, Saumya Ranjan Tripathy, Manoj Kumar Parida, Bidyut Kumar Das; SCB Medical College, Cuttack, Odisha, India

Background: Adult-onset Still's disease (AOSD) is a systemic autoinflammatory disease characterized by high-grade fever, arthralgia, and salmon-colored maculopapular rash. Cardiac involvement is rarely reported. We present a case report of a patient with AOSD with myocarditis that proved fatal.

Case Report: A 48-year-old female presented with chief complaints of continuous fever, symmetrical polyarthralgia for 1 month, without rash, oral ulcer, and alopecia. She was treated in a private setup as a case of pyrexia of unknown origin and was put on multiple antibiotics. She was admitted to our hospital because her fever did not subside. Hematological investigations available with the patient revealed hemoglobin (Hb)-8.7 mg/dl, total leukocyte count (TLC)-12,700/cumm, serum glutamic pyruvic transaminase -56 mg/dl, serum glutamic oxaloacetic transaminase-136 mg/dl, erythrocyte sedimentation rate-Westergren-80 mm 1st h, and C-reactive protein-12.6 mg/l. Rheumatoid factor and antinuclear antibodies were negative. Serum ferritin was 32,000 μg/l (normal: 30–300 μg/l). Ultrasonography revealed mild hepatosplenomegaly. She was diagnosed as a case of AOSD. She became afebrile with prednisolone 1 mg/kg per oral per day. On day 4, she developed loose stools, put on antibiotics, and improved. Two days later, she developed severe epigastric pain with shortness of breath. Electrocardiography showed low voltage complexes. She was started on conservative treatment and investigations sent to rule out cause for acute abdomen. She deteriorated suddenly and expired. Her reports postdeath showed Hb-8.7 mg/dl, TLC 23500/cumm (neutrophil-85%; lymphocyte-15%), serum amylase-34 IU/l, lipase-19 IU/l, serum procalcitonin-0.27 ng/ml (<0.5 ng/ml-unlikely to be systemic bacterial infection), and serum troponin (Trop)-T -441 pg/ml (normal <14.5 pg/ml).

Discussion: The absence of drop in hemoglobin, normal procalcitonin, and normal serum amylase and lipase ruled out gastrointestinal bleed, sepsis, and pancreatitis. The final diagnosis given was AOSD with myocarditis. The probable cause of death could be ventricular fibrillation.

Conclusion: Myocarditis, though rare, can be a fatal complication of AOSD, and hence, routine cardiac screening by echocardiography and serum Trop is essential.


  OPC0273: Late referral of lupus to rheumatologists in India: A study of lupus cohort Top


Chandrashekara S, Vikram Haridas, Ashwini Kamath, Apurva Khare, Utkarsha Patil; ChanRe Connective Tissue Disease Consortium (CCTDC)

Introduction: Systemic lupus erythematosus (SLE) is one of the prototype connective tissue diseases (CTDs). The early recognition and referral play a paramount role in patient care. As a first step to understand the patients care in India, we analyzed the collected data to evaluate the delay in referral of lupus patients to a specialist and their disease severity.

Methods: ChanRe CTD Consortium (CCTDC), a group of practicing rheumatologists across India, has created a database of lupus patients to carry out further studies. The cross-sectional study of the lupus cohort, at the time of reporting, comprised 200 patients. All the patients who fulfilled the 2012 SLICC SLE criteria for SLE were recruited after obtaining their informed consent. The SLE disease activity index was calculated based on the retrieved chart and the duration of the symptoms attributed to lupus.

Results: CCTDC, the rare disease registry, recruited a total of 200 SLE patients. The mean age of the study cohort was 31 (11–74) years, and the male-to-female ratio noted was 10:190. The referral time to the specialist was 55 (1–307) months with majority reaching after 1 year. More than 50% of the patients had moderate-to-severe disease, with 30% having severe disease.

Conclusion: Lupus patients are often referred to tertiary care after noting a severe disease activity. It is necessary to improve the early recognition of lupus at the level of primary care.


  OPC0274: Pancreatitis-panniculitis-polyarthritis syndrome: An unusual presentation of acute pancreatitis Top


Joydeep Samanta, Sandeep Nagar, Uma Kumar, Anoop Saraya1; Departments of Rheumatology and 1Gastroenterology and Human Nutrition, All India Institute of Medical Sciences, New Delhi, India

Case Report: A 40-year-old army personnel by occupation was admitted under us with complaints of recurrent pain abdomen for 2 years with progressively deforming arthritis involving moderate-to-large joint for 1½ years. Pain abdomen started 2 years back was severe, radiating to back without any relationship with food. He was diagnosed to have acute necrotizing pancreatitis and managed conservatively. He had recurrent attacks of pain abdomen with multiple hospital admissions, needed endoscopic retrograde cholangiopancreatography, and stenting once due to pancreatic duct disruption. He had some nodular lesion over his extremities; biopsy showed features suggestive of panniculitis. Within 6 months of onset of abdominal pain, he developed arthritis of his both ankle joint followed by involvement of bilateral knee and elbow joints without any small joints involvement, morning stiffness, or inflammatory low back pain. He was treated outside, but it was persistent and progressive leading to deformities of the involved joints. Due to recurrent attacks of pain abdomen and joint deformity, he came to our institute. He had both elbow, knee joints deformity with bilateral knee and left ankle joint synovitis, and no involvement of small joints. For recurrent pain abdomen, gastroenterology evaluation was done and diagnosed as chronic pancreatitis. His routine investigations including rheumatoid factor, anti-cyclic citrullinated peptide antibody, human leukocyte antigen B27, and X-ray sacroiliac joint were normal. Magnetic resonance imaging of elbow and knee joint showed features of multiple areas of large erosions with possibility of osteomyelitis changes. Bone biopsy showed no evidence of infection. Considering previous history of necrotizing pancreatitis with a history of duct disruption, deforming large joint arthritis, and panniculitis, diagnosis of pancreatitis-panniculitis-polyarthritis syndrome was made. He was managed conservatively with analgesic and physiotherapy for his joint symptoms. Chronic pancreatitis was adequately managed by a gastroenterologist.

Discussion: Pancreatitis-panniculitis-polyarthritis is a rare complication of acute necrotizing pancreatitis, but exact pathophysiology is still elusive. It is postulated that leakage of pancreatic enzymes especially lipase consequent to pancreatic duct disruption is responsible for extensive fat necrosis and consequent deforming arthritis and panniculitis. Management is mainly conservative with adequate management of underlying pancreatic disease.


  OC0056: A unique case of systemic lupus erythematosus presenting with lupus hepatitis and autoimmune myelofibrosis: A case report Top


Anamika Das, K. G. Lynrah, I. Tiewsoh, P. K. Bhattacharya, Bhupen Barman; NEIGRIHMS, Shillong, Meghalaya, India

Introduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease of unknown cause that can affect virtually any organ of the body. The clinical heterogeneity of SLE and the lack of pathognomonic features or tests pose a diagnostic challenge for the clinicians

Case Report: An 18-year-old female student was admitted to a tertiary care center in Northeast India with complaints of bleeding manifestations on and off for 4 years with an aggravation of her bleeding manifestations with recurrent epistaxis in 2 weeks before her admission which was associated with low-grade fever, vomiting, vague abdominal pain, and joint pain. She was initially diagnosed as a case of immune thrombocytopenic purpura on the evaluation of thrombocytopenia and was on steroids to which she had shown a partial response. Her current presentation showed a picture of pancytopenia with hepatitis. She was evaluated for the presence of acute infection and an autoimmune etiology. Preliminary workup for an infectious etiology was negative. A bone marrow examination was done in view of pancytopenia which showed a picture of myelofibrosis. Autoimmune workup showed positive antinuclear antibodies and double-stranded DNA and reduced complement levels. Markers for autoimmune hepatitis antismooth muscle antibodies, antimitochondrial antibodies, and Anti-liver-kidney microsomes antibodies were negative. The findings were typical of SLE with flare, however, with unique findings of autoimmune myelofibrosis with pancytopenia and lupus hepatitis. She was treated with pulse therapy of methyl prednisolone for 3 days followed by oral steroids and mycophenolate mofetil to which she responded with normalization of liver enzymes and correction of pancytopenia.

Discussion: SLE has been known to present in unique and varied patterns. However, presentation as a case with acute flare with autoimmune myelofibrosis along with hepatitis has rarely been documented in literature.


  OPC0283: Demographic and clinical profile of interstitial lung disease in long-standing rheumatoid arthritis Top


Wasim Kazi, Dhaval Tanna, Natasha Neglur, Rohit Bajaj, Vinay Singal, Rajiva Gupta; Medanta - The Medicity, Gurgaon, Haryana, India

Background: Interstitial lung disease (ILD) is a frequent extra-articular manifestation of rheumatoid arthritis (RA). Among all connective tissue disease-ILD, 25%–30% of RA patients are found to be associated with ILD. We wanted to study who develops ILD and what is prevalence of other extra-articular manifestations in long-standing RA patients with ILD.

Methods: We included all 130 patients of RA who presented consecutively to outpatient department and inpatient department of Medanta Hospital, from August 2017 to April 2018. The disease characteristics including rheumatoid factor (RF)and cyclic citrullinated peptides (CCP) positivity, comorbidities, articular and extra-articular complications diagnosed on the basis of clinical or laboratory data were noted. Only patients with disease duration of more than 10 years who were on disease-modifying antirheumatic drugs were selected. The study was approved by the Institutional research board. Ethics committee clearance was also taken (Reference No.-MICR-789/2017).

Results: Disease characteristics of the patients having ILD versus those who did not were studied.

Conclusion: ILD was significantly associated with a longer disease duration and RF/CCP-positive status. All patients who developed ILD, in our cohort, were RF and/or CCP positive. Furthermore, mean age of ILD patients was significantly higher. Osteoporosis was seen more frequently in patients who had ILD.


  OPC0303: Descriptive analysis of malignancies in systemic autoimmune rheumatic disorder: A 5-year audit Top


Rahul Sahu, Arvind Ganapati, Debashish Danda; Department of Clinical Immunology and Rheumatology, CMC Vellore, Tamil Nadu, India

Background: Systemic autoimmune rheumatic disorders (SARD) are associated with higher incidence of malignancies.

Aim: The aim was to study the prevalence of various malignancies among SARD in a tertiary care teaching hospital setting.

Methods: This was a retrospective study in a tertiary care teaching hospital setting of Southern India involving outpatients and inpatients of the Rheumatology Department between August 2013 and July 2018. Electronic medical records were scouted for search criteria of “malignancy,” “carcinoma,” “lymphoma,” “cancer,” and clinical details of patients with positive search criteria were recorded.

Results: Out of total 18,923 SARD patients, 55 were found to have 56 malignancies (1 malignancy in 338 patients); 38 (69%) of them were females. Mean age at malignancy diagnosis and median time span between the diagnosis of SARD and malignancy were 50.52 ± 13.6 and 2 years (0, 11), respectively. Highest prevalence of malignancy was found with dermatomyositis (7 malignancies among 155 dermatomyositis patients, 45.16/1000 patients) followed by primary Sjögren's syndrome (21.12/1000 patients) and undifferentiated connective tissue disease (5.46/1000 patients). Overall, the most common malignancies observed were lymphoma and carcinoma breast (12 and 7 cases, respectively). Most common malignancy in dermatomyositis was carcinoma breast (71.4%). Highest prevalence of lymphoma was seen among Sjögren's syndrome patients (2.71/1000 patients).

Conclusion: Lymphoma was the most common malignancy seen among our SARD patients cohort, with highest prevalence among primary Sjögren's syndrome. Dermatomyositis was the most common SARD associated with malignancy with breast cancer being the most common.


  OPC0291: Epidemiologic data of patients of adult-onset Still's disease receiving tocilizumab in the Department of Rheumatology, IPGMER and SSKM Hospital, Kolkata Top


Ankit Patawari, Deepak Rath, Alakendu Ghosh, Pradyot Sinhamahapatra, Parasar Ghosh; Department of Rheumatology, IPGMEandR and SSKM Hospital, Kolkata, West Bengal, India

Background: Adult-onset Still's disease (AOSD) is an uncommon cause of fever and arthritis and is often misdiagnosed initially. First-line therapy in AOSD is based on corticosteroids, which often requires high doses for a long period, with the subsequent risk of side effects. We present our cohort of patients who had been diagnosed as AOSD based on their clinical, physical, and serological parameters and received tocilizumab.

Objectives: The objective was to assess the response to tocilizumab in patients of AOSD.

Methods: This was a longitudinal observational study of patients of AOSD receiving tocilizumab admitted to the rheumatology ward.

Results: Twelve patients diagnosed as AOSD receiving tocilizumab was analyzed. There were nine females and three males. The mean age at presentation was 28.81 ± 10.56 years (range: 17–62 years). Patients presented at an average of 6 weeks of fever (range: 2–24 weeks) to us for evaluation. Arthritis was seen in 87.5%, sore throat was present in 84.1%, and skin rash was seen in 65.6% of the population. The incidence of hepatomegaly, splenomegaly, and lymphadenopathy was 62.5%, 46.9%, and 43.8%, respectively. There was a decrease in fever, cutaneous manifestations to 5.9%, joint manifestations had decreased to 32.4% and lymphadenopathy to 0% after tocilizumab therapy. Mean hemoglobin was 8.6 ± 1.6 g/dl, mean total leukocyte count: 17,379 ± 7199/μl, and mean ferritin levels were 9775 ng/ml. A dramatic reduction in laboratory markers of inflammation, including C-reactive protein, erythrocyte sedimentation rate, and ferritin level, was achieved. 78.1% of the patients were treated with concomitant steroids; median dose was, however, reduced from 13.8 to 2.5 mg/day while on follow-up.

Conclusions: Tocilizumab treatment was associated with rapid and maintained clinical and laboratory improvement in patients with AOSD. Tocilizumab also had a significant corticosteroid-sparing effect. The prednisone dosage was reduced significantly during tocilizumab therapy.


  OPC0288: Co-trimoxazole prophylaxis prevents major infective episodes in SLE patients on immunosuppression Top


Salil A Ganu, Ashish J Mathew, B Antonisamy1, L Jeyaseelan1, Debashish Danda; Departments of Clinical Immunology and Rheumatology and 1Biostatistics, Christian Medical College, Vellore, Tamil Nadu, India

Background: Both disease and its treatment can render systemic lupus erythematosus (SLE) patients susceptible to common infections. This study aims to assess prophylactic role of co-trimoxazole in preventing infections.

Objectives: The objective was to compare the occurrence of major as well as minor infections between SLE patients on co-trimoxazole prophylaxis and those on no prophylaxis.

Methods: In this cross-sectional observational study, SLE patients from outpatient department or inpatient department under the Rheumatology Department of CMCH during the study period were screened. SLE patients on mycophenolate and tapering steroid for at least 1 year were included in the study. The relevant demographic, clinical, and laboratory data were collected and the events of “major infections” (events requiring the use of intravenous antibiotics or hospitalization”) were noted. Episodes of “minor infections” (requiring use of oral antibiotics) during the past 1 year were recorded by interviewing patients. Analysis for major infections was done by comparing the median (interquartile range) of standardized episodes of infection (i.e., total episodes of infection divided by treatment duration per patient) between the prophylaxis group and no prophylaxis group using Mann–Whitney U-test. The proportion of patients having minor infections during the last 1 year were also compared between the prophylaxis and no prophylaxis groups using Chi-square test.

Results: The standardized rates of episodes of major infections (mean [standard deviation]) were significantly lower in the co-trimoxazole prophylaxis group as compared to no prophylaxis group (0.03 [0.25] vs. 0.77 [1.13]; [P < 0.001]). The proportion of patients having minor infections during preceding 1 year, however, were not significantly different (P = −0.141), between co-trimoxazole prophylaxis group (21.4%) and no prophylaxis (20.4%) group.

Conclusions: Use of co-trimoxazole in SLE patients on immunosuppression prevents episodes of major infections.

[TAG:2]OPC0289: Mycophenolate (mycophenolic acid) area under the curve concentrations and treatment response in Takayasu's arteritis [/TAG:2]

Shivraj Padiyar, Ruchika Goel, Binu S Mathew, Debashish Danda1; Departments of Clinical Immunology and Rheumatology and 1Clinical Pharmacology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Mycophenolate (mycophenolate mofetil and mycophenolate mofetil-sodium) is a widely used immunosuppressant in Takayasu's arteritis (TA). Currently, there are no data on optimal dose in patients with TA. Therapeutic concentrations of mycophenolic acid (MPA) as used for lupus nephritis are utilized as a cutoff for TA.

Aim: The aim was to study the plasma concentrations of mycophenolate to optimize its dose in TA.

Methods: This was a retrospective study of prospectively documented data from September 2013 to August 2018 carried out in a tertiary care teaching hospital in Southern India. Patients of TA fulfilling the American College of Rheumatology 1990 criteria were recruited. Plasma MPA concentrations were determined as 6-h area under curve (6-h AUC) by high-performance liquid chromatography using a standardized protocol. The MPA levels, clinical response Indian Takayasu activity score 2010 (ITAS 2010), erythrocyte sedimentation rate/C-reactive protein (CRP), and ITAS-A (CRP) were retrieved from hospital records and analyzed in relation to clinical course in terms of responders and nonresponders.

Results: A total of 76 patients (mean age 33.75 ± 11 years, male:female = 1:6) had undergone 6-h AUC and were studied. Type 5 was the most common type (n = 42, 58.3%). Only two patients had MPA concentrations of <30 mg.h/L, which is the standard laboratory cutoff for lupus patients. Since most of the nonresponders had concentrations of >30 mg.h/L, it makes this cutoff level redundant for our TA population. We found that the best cutoff for MPA AUC that differentiates responders from nonresponders is 47.5 mg.h/L (AUC 0.669,0.51–0.83; P = −0.057) unlike the standard laboratory cutoff of 30 mg.h/L used for lupus. 73.5% of patients with AUC above this value were responders and 26.5% are nonresponders, whereas 38.5% with AUC below this value were responders and 61.5% were nonresponders (P = 0.025).

Conclusion: MPA concentrations of 46.7 mg.h/L as cutoff correlates with TA disease activity instead of the usual cutoff of 30 mg.h/L used for lupus.


  OPC0310: A rare association of anti-glomerular basement membrane antibody-negative, biopsy-proven Goodpasture disease with myocarditis and unusual skin lesions: A case report Top


Shambaditya Das, Chirantan Mandal, Sayan Saha, Subhasis Neogi, Niladri Sarkar; Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

Case Report: A 24-year-old female with no previous comorbidities presented with a rapidly developing oliguria and hematuria (rapidly rising serum creatinine; red blood cell [RBC] cast, dysmorphic RBC, and subnephrotic range proteinuria in urine suggestive of rapidly progressive glomerulonephritis), cough without hemoptysis (low hemoglobin levels, baseline hemoglobin not available-? pulmonary hemorrhage), with chest pain, severe shortness of breath and orthopnea having features of congestive cardiac failure (arterial blood gas showed Type 1 respiratory failure, electrocardiogram had Pan ST-T changes, Trop-i markedly raised, Echocardiography shows global hypokinesia-features suggestive of myocarditis). The patient also had a fleeting, asymmetrical polyarthralgia, erythematous, and nonblanching skin lesions mainly in lower limbs and buttocks (skin biopsy suggestive of “perforating disorder” with no evidence of vasculitis). Antinuclear antibodies, dsDNA, antineutrophil cytoplasmic antibody, MPO, PR3, HBsAg, and anti-HCV were all negative. Complements were normal. Blood cultures showed no growth. Anti-glomerular basement membrane (GBM) was negative. Renal biopsy was done and showed necrotizing and crescentic glomerulonephritis with linear immunoglobulin G staining consistent with anti-GBM disease. The patient was treated with pulse methyl prednisolone, cyclophosphamide, underwent Plasmapheresis, and responded clinically and biochemically. Goodpasture is a rare but grave condition that requires early recognition and aggressive management. However, in the face of negative antibody and atypical presentation, it is often difficult to pinpoint, and we have to rely entirely on renal biopsy for diagnosis. There has been only a handful case reports of antibody-negative Goodpasture in the world, and to the best of our knowledge, there have been no case reports mentioning the association of Goodpasture disease with myocarditis or perforating disorder in the skin.


  OPC0299: Case Reports: Ocular cicatricial pemphigoid Top


Mamatha Panathula, Mayur Morekar, Tanuj R Sharma, Anamika Agarwal1; Bombay Hospital and Medical Research Centre, 1TNMMC and BYL Nair Hospital, Mumbai, Maharashtra, India

Case 1: A young female in twenties, with biopsy-proven ocular cicatricial pemphigoid (OCP), bilateral cataract, presented with severely painful, red eyes. She was started on immunosuppression with oral steroids and oral methotrexate for immunomodulation, steroid-sparing. Because of only partial response, she was shifted to azathioprine. She developed leukopenia although clinical response was good. She was then given intravenous (IV) methyl prednisolone and IV cyclophosphamide (CYC) pulses over 6 months. Response was excellent. The surface being stable, bilateral cataract surgery with intraocular lens implantation was performed. She is stable on low-dose azathioprine.

Case 2: A female in fifties, diagnosed clinically as OCP, on low dose of oral CYC, presented with continued severe ocular symptoms. History and records were reviewed - she had improved 60%–70% with the ongoing treatment. Hence, her oral CYC dosage was increased for better immunomodulation. At follow-up, she is asymptomatic.

Case 3: A female, in seventies, incapacitating symptomatic for 4–5years, was clinically diagnosed OCP. She presented with bilateral debilitating ocular disease with impending corneal melt in her right eye. Past treatments included methotrexate, azathioprine, mycophenolate, with no response. She was given IV methylprednisolone, IV CYC pulse doses, oral steroids. Clinical response was excellent with stabilization of bilateral ocular surfaces, to achieve stable vision. She was continued on oral CYC.

Discussion: OCP, a debilitating ocular surface disorder, is diagnosed clinically or with conjunctival biopsy. Severe symptoms incapacitate the patients making them dependent in day-to-day activities. OCP can cause blindness. Our patients had slow onset of symptoms over 1–3 years, were initially extensively investigated, but treated sub-optimally with topical/oral steroids, with/without immunomodulation. Hence, they had relentless, disease progression.

Conclusion: Early, aggressive management of OCP with immunomodulation, including biologics if necessary, can prevent debility and blindness.


  OPC0293: Effect of tocilizumab in refractory aortoarteritis: Experience from a single tertiary center Top


Rohit Bajaj, Natasha Negalur, Gayatri Ekbote, Lucky Sharma, Shruti Bajad, Rajiva Gupta; Department of Rheumatology, Medanta - The Medicity Hospital, Gurugram, Haryana, India

Background: Glucocorticoids are an effective therapy in Takayasu's arteritis (TA), but role of other immunosuppressants needs to be studied. Tocilizumab has recently been studied as maintenance therapy in TA in Japan, but no such studies are available in India.

Aim: The aim was to study the effect of tocilizumab in patients of refractory TA.

Methods: All consecutive patients of TA attending the rheumatology outpatient as well as inpatient department of Medanta - The Medicity Hospital from a period of August 2016 to June 2018 were included. Five patients who received tocilizumab for refractory or relapse disease were assessed. These patients were followed up for minimum 6 months. At each visit, disease activity assessment was done by Indian Takayasu activity score (erythrocyte sedimentation rate/C-reactive protein). The study was approved by the institutional review board.

Results: Five patients of TA received tocilizumab infusions (8 mg/kg) every 4 weeks in our center. Four patients had refractory disease on steroids and oral immunosuppressants and one patient had disease relapse (in the form of anterior ischemic optic neuropathy). First patient developed severe abdominal pain with first infusion and further infusions could not be given as patient was lost to follow-up. Second patient took four infusions, showed good clinical response, and had to stop because of affordability issues. Third patient was given extended treatment for 8 months due to persistent disease activity. Fourth patient was given six infusions and is in remission till now. Fifth patient developed pyelonephritis after four infusions and had to stop.

Conclusion: Tocilizumab appears to be an effective therapy in TA. It still raises concerns about tolerance and adverse effects mainly infections. Further larger studies are needed to confirm.


  OPC0294: Study of serum calprotectin levels in rheumatoid arthritis and its correlation with the severity of rheumatoid arthritis Top


Satinder Deswal, R P Saini, Arun Gogna, B C Kabi; VMMC and Safdarjung Hospital, New Delhi, India

Background: Calprotectin is a calcium-binding protein secreted predominantly by neutrophils and monocytes, specifically released during interaction of monocytes with inflammatory activated endothelium, probably at site of local inflammation. Elevated calprotectin serum concentration may be an important serum marker of the extent of local inflammation in the affected joints. Clinical severity of rheumatoid arthritis (RA) is measured by various scores which include clinical disease activity index (CDAI) and disease activity score 28 (DAS28).

Aims and Objectives: The aim and objective were to study the serum calprotectin levels in patients of RA and its correlation with clinical severity measured by CDAI and DAS28 (erythrocyte sedimentation rate [ESR]).

Materials and Methods: The present observational cross-sectional study conducted in the Department of Medicine and Rheumatology Clinic, VMMC and Safdarjung Hospital, New Delhi, included 65 patients of RA satisfying the inclusion and exclusion criteria over a period of 18 months. The study was performed according to the principles of the Declaration of Helsinki. Inclusion criteria were patients with age above 18 years, any gender, and fulfilling the (ACR)/European League Against Rheumatism 2010 Classification. Exclusion criteria were patients with any other autoimmune disorder, chronic kidney disease or infection.

Observations: The median age was 35 years with a female-to-male ratio being 8:1.The present study shows that the mean serum calprotectin level of patients with low disease activity according to CDAI versus DAS score was 3133.33 (32.3%) and 3200 (4.6%), moderate 4728.93 (43.07%) and 4137.5(49.2%), high 7046 (23.07%) and 6813.04 (10.7%), respectively, and the correlation was statistically significant (P = −0.0001).

Discussion: As DAS 28 (ESR) may over/under-represent disease activity while CDAI is just sum total of SJC, TJC, PGC, and EGC. In clinical practice, some patients have normal or low levels of ESR or C-reactive protein (CRP) despite extensive arthritis. Therefore, serum calprotectin levels could be useful an inflammatory marker in these patients.

Conclusion: The present study revealed a significant positive correlation between serum calprotectin levels and other markers of disease activity, i.e., ESR, CRP, CDAI, and DAS28. Serum calprotectin levels did not differ with age.


  OPC0295: Hypertension in Takayasu's arteritis: Not always due to renal artery involvement Top


Rohit Bajaj, Natasha Negalur, Dhaval Tanna, Wasim Kazi, Durga Rao, Shruti Bajad, Vinay Singhal, Rajiva Gupta; Department of Rheumatology and Clinical Immunology, Medanta - The Medicity Hospital, Gurugram, Haryana, India

Background: Takayasu's arteritis (TA) is a chronic inflammatory disease affecting the aorta and its branches. Hypertension is usually seen in Type III, IV, and V subsets of TA mainly attributed to the involvement of renal arteries or suprarenal aorta causing renal hypoperfusion, resulting in activation of renin-angiotensin system (RAS).

Aim: The aim was to study the prevalence of systemic hypertension in Type I and II subsets of disease.

Methods: All consecutive patients of TA attending the Rheumatology Outpatient as well as Inpatient Department of Medanta - The Medicity Hospital from a period of August 2016 to June 2018 were included. Their demographic details and clinical, radiological, and laboratory parameters were assessed. The study was approved by the institutuional review board.

Results: The study comprised 74 patients of TA, including 16 (21.6%) males and 58 (78.4) females. Twenty-one (28.3%) patients had Type I and Type II subset and 53 (71.7) had Type III, IV, and V subset of disease. Thirty-nine patients were identified with hypertension, 29 out of them had renal artery involvement, and three had extensive abdominal aorta involvement. Seven patients had hypertension without renal artery or abdominal aorta involvement, out of which six had Type II and one patient had Type I disease. All patients of Type I and Type II subsets of disease were within 20–45 years of age group at disease onset and five out of seven had carotid involvement.

Conclusion: Hypertension is one of the common manifestations of TA, and renal artery stenosis is the main cause resulting in activation of RAS. In our study, we found that hypertension is not uncommon in TA even without renal artery involvement. Reduced aortic compliance and carotid baroreceptor insensitivity are the important causative factors.


  OPC0296: Comparison of clinical and serological profile of idiopathic inflammatory myositis patients with and without interstitial lung disease Top


J N Durgarao Yadavalli, Natasha Negalur, Dhiren Raval, Lucky Sharma, Gayatri Ekbote, Shruti Bajad, Rajiva Gupta; Medanta - The Medicity, Gurgaon, Haryana, India

Aim: Interstitial lung disease (ILD) is known to affect idiopathic inflammatory myositis (IIM) in about 20%–40% patients worldwide, but studies from India are lacking. In this project, we aimed to study the clinical and serological characteristics of patients with ILD in IIM.

Methods: This was a prospective observational study carried out at the Department of Rheumatology and Clinical Immunology at Medanta Hospital, Gurgaon, from August 2016 to July 2018. A total of 50 consecutive patients diagnosed with IIM and satisfying the Peter and Bohan criteria were included in the study. These patients were divided into two groups based on ILD assessed by high-resolution computed tomography. Demographics and clinical, laboratory (including serology), and treatment details were noted and compared between the two groups. The study was approved by the hospital institutional ethical committee.

Results: Fourteen (38.8%) patients of IIM had ILD. The mean age was 41.5 (± 14) and 41.2 (± 14.7) years in ILD and non-ILD groups, respectively. Majority were females (11 [78.6%] in ILD vs. 27 [75%] in non–ILD). The median disease duration was similar in both groups (30 [2–180] vs. 33 [1–300] months in ILD vs. non-ILD). Arthritis, Raynaud's phenomenon, digital ulcers and mechanic's hands, Jo-1, and Ro positivity were more commonly associated with ILD. None of the Mi-2-positive patients had ILD. However, dysphagia and neck flexors were more in the non-ILD group. Rituximab was given in 4 (28.6%) and in 2 (5.5%), respectively, in ILD and non-ILD groups.

Conclusion: ILD is associated with arthritis, Raynaud's, Mechanic's hands, and Jo-1 positivity in IIM patients. However, there was no consistent evidence of more severe overall disease in the ILD group. Mi-2 is not associated with ILD.


  OPC0297: Level of education and economic status does not affect treatment outcome of rheumatoid arthritis patients on triple disease-modifying antirheumatic drug therapy: results of cross-sectional study Top


Anupama Mundu, Aswin Nair, Ashish J Mathew, Tunny Sebastian, Debashish Danda; Christian Medical College, Vellore, Tamil Nadu, India

Background: Low socioeconomic status (SES), measured using multiple determinants (e.g., educational background, occupation, household income, ethnicity), has been associated with increased risk and poorer disease outcomes in rheumatoid arthritis (RA). This study was undertaken to investigate the influence of educational background and socioeconomic status (SES) on RA outcome, assessed by disease activity score (DAS28).

Objective: The objective was to identify the influence of education and socioeconomic status on treatment outcome of patients with RA on standard triple-drug therapy.

Methods: All RA patients, both outpatient and inpatient, attending the Department of Clinical Immunology and Rheumatology of Christian Medical College, Vellore, between July 2017 and June 2018, on standard triple drugs for at least 1 year were included. Data on SES measures including highest education in household were recorded for each patient. Based on DAS 28 C-reactive protein (CRP) scores at inclusion, the patients were divided into good (DAS 28 score <2.6) or bad outcome (DAS 28 score >3.2) subsets. SES measures were compared in both groups. Multivariable linear, logistic regression models were used to estimate associations of each SES measure with RA outcome.

Results: In our cohort of 404 patients with a mean age of 48.05 ± 10.78 years and median disease duration of 7 years (interquartile range 4–11), 351 (86.9%) were women. Remission (DAS 28 CRP <2.6) at inclusion was achieved by 112 patients. Mean DAS 28 CRP score was 2.46. Within the groups with DAS 28 CRP score <2.6 and >3.2, there were significant associations between LES and bad disease outcome on univariate analysis; however, on multivariate analysis, there was no significant association between educational level (P = 0.693; 95%confidence interval (CI) 0.446–3.37) or SES (P = 0.214; 95% CI 0.531–16.82) with disease outcome.

Conclusion: In our study, educational background and SES did not influence DAS 28 based on outcomes in patients with RA on triple disease-modifying antirheumatic drug therapy for at least 1 year.


  OPC0298: Association of diet and spices with treatment outcome in Asian Indian patients with rheumatoid arthritis Top


Harshini Shivakumar, Ramya J, Antonisamy B, Jeyasheelan L, Debashish Danda; Christian Medical College, Vellore, Tamil Nadu, India

Background: Influence of diet on inflammation, especially foods such as fish oil and spices such as turmeric, capsaicin, and garlic, are reported in published literature. However, a well-designed study on this subject among Asian Indian patients is lacking.

Objectives: The objective was to analyze whether the type and quantity of intake of various food constituents, with particular reference to Indian spices, make an impact on the control of disease activity in patients with rheumatoid arthritis (RA).

Methods: Patients diagnosed as RA by the American College of Rheumatology 2010 Criteria receiving standard triple-drug therapy between June 2017 and June 2018 for at least 1 year were enrolled. Disease activity was assessed during routine outpatient department visit. They were administered a food frequency questionnaire pertaining to the quality as well as quantity of food intake. Multivariate logistic regression used for analysis.

Results: A total of 400 patients were included with 86.75% females. 67.75% patients were in disease remission, 10% had mild disease activity, and 22.25% had moderate-to-high disease activity; only 18.09% were vegetarians and the rest consumed nonvegetarian food. Median age was 47.99 years (standard deviation 10.67), median duration of illness before presentation to our clinic was 7 years (interquartile range (IQR) 4,10), median erythrocyte sedimentation rate was 37 mm/h (IQR 23,52), median C-reactive protein (CRP) was5.34 mg/L (IQR 2.04, 12.4), and median disease activity score (DAS)28 CRP was 2.07 (IQR 1.64, 2.97). Patients with DAS28 CRP of <2.6 were compared with those >3.2. Statistically higher consumption of ginger, garlic, turmeric, and coriander were noted among patients in remission. Similar results were obtained when patients with DAS28 CRP of <1.4 were compared with DAS28 CRP >5.1. Nonsignificant numerical differences were noted for intake of food constituents such as wheat, total pulse, vegetables, fruit, milk, and fish.

Conclusions: Higher consumption of Indian spices such as ginger, garlic, turmeric, and coriander were found to be associated with better control of disease activity, and hence, the inflammation, as evidenced by DAS28 CRP in patients with RA, receiving standard treatment


  OPC0302: Paraneoplastic syndromes as rheumatic disorders Top


Yogesh Preet Singh, Vaibhavi Velangi;

Introduction: Malignant neoplasms are associated with a wide variety of paraneoplastic rheumatological syndromes. These include dermatomyositis/polymyositis, paraneoplastic vasculitis, lupus-like syndrome, Raynaud's phenomenon, erythema nodosum, digital gangrene; musculoskeletal manifestations may coincide, follow, or antedate the diagnosis of cancer. Awareness that cancer can cause certain nonmetaplastic symptoms is important for early diagnosis and treatment of malignancy. The clinical features and risk factor of 14 patients with a mean age of 43.64 years and smoking history in 21.4% were characterized by a retrospective review. Patients suffering from solid malignancies accounted for 28%, as compared to majority, i.e., hematological malignancies (72%), arthritis (78.5%), fever (61.5%), weight loss (23.07%), skin (15.38%), and muscle weakness/pain (14.28%).

Conclusion: Rheumatic disorder with atypical clinical presentation should alert clinicians to look for occult malignancies. Although paraneoplastic rheumatic syndromes are rare, clinicians should be aware and vigilant that they can be the first sign of malignancy. This is necessary for early diagnosis and treatment.


  PC0095: Late-onset rheumatoid arthritis: An unavoidable truth Top


Reet Banerjee, Raja Bhattacharya; Medical College and Hospital, Kolkata, West Bengal, India

Background: Rheumatoid arthritis (RA) is a chronic multisystem disease characterized by persistent inflammatory synovitis, usually involving peripheral joints symmetrically. RA usually develops in middle-aged adults but may occur at childhood or old age, too. RA is generally described as a disease with two peaks of onset, either late (When begins after the age of 60, known as late-onset RA [LORA]) or early (around 30–55 years, known as early-onset RA [EORA]). With 7.7% of its population being aged more than 60 years, India has acquired the label of an aging nation. Considering that the average age of the population is continuously rising, LORA will gain importance in the near future.

Methods: Between January 2017 and June 2018, 50 patients with age >60 years, presenting with LORA were taken from Rheumatology Outpatient Department of Kolkata Medical College and analyzed for their presenting features, comorbidities such as diabetes, hypertension, and hyperlipidemia, and degree of joint involvement. Patients with polymyalgia rheumatica (PMR), paraneoplastic polyarthritis, and hepatitis B and C were excluded. It was a cross-sectional observational study.

Results: Classical rheumatoid hand deformities, interstitial lung disease, and Sjogren's syndrome were significantly lower in LORA than in EORA. LORA patients had more common weight loss, myalgia, lymphadenopathy, PMR-like symptoms. 70% were seropositive, 20% were disease-modifying antirheumatic drug naïve, 40% were hypertensive, 16% had hyperlipidemia, 36% were diabetic, and 22% had ≥10 joints involvement.

Conclusion: In this predominantly older RA cohort, classical presentation of RA is uncommon and there is more predominance of nonspecific symptoms and cardiovascular comorbidities. Hence, early detection of these subset of patients is difficult but essential to improve their quality of life.


  OPC0314: A single-center retrospective study of outcome of mycophenolate mofetil therapy in patients with antineutrophil cytoplasmic antibody vasculitis Top


Debashis Maikap, Ruchika Goel, Debashish Danda; Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Use of mycophenolate needs to be revisited in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) due to better safety profile.

Aim: The aim was to investigate the efficacy and safety of mycophenolate in patients with AAV.

Methods: Details of patients attending our clinics during 2015–2018 and fulfilling the American College of Rheumatology Criteria for granulomatosis-with-polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic GPA (EGPA) and patients initiated on mycophenolate were noted retrospectively. Sustained complete response (CR) was defined as maintenance of Birmingham vasculitis activity score (BVAS) score of 0 with the absence of new manifestations through the follow-up period. Relapse is defined as recurrence of disease activity or new manifestations. Median time to relapse was calculated using Kaplan–Meier survival plots.

Results: Among 60 patients diagnosed with AAV, 40 patients (male:female17:23, age 50.5 [interquartile range (IQR): 37–59] years, disease duration 5 [IQR:3–9] months) satisfied inclusion criteria. Thirty-four patients had GPA while two each had MPA and EGPA. ANCA was positive in 38 patients (c-ANCA–34, p-ANCA–). Median BVAS, c-ANCA, and p-ANCA levels at the first visit were 17.5 (IQR: 9.5–22), 2 (IQR: 0.75–3), 17.5 (IQR: 3.1–194.5), and 2 (IQR: 2–27.2), respectively. All patients received steroids with a median dose of 60 mg at baseline (IQR: 45–72). Dose of mycophenolate was 2–3 g/day. During a follow-up of 31.5 months (IQR: 21–62), sustained CR was observed in 28 (70%) patients. BVAS, c-ANCA levels, and p-ANCA levels decreased to 0 (IQR: 0–0), P < 0.0001; 3.8 (2–46.5), P = 0.0003; and 2.1 (2–8.65), P = 0.16, respectively, at the last visit. Steroid dose was reduced to 3.5 (IQR: 1–6) mg at the last visit (P < 0.001). Relapse occurred in 12 patients at median of 93 (IQR: 60–94) months. ANCA levels, ear nose throat or renal involvement, and disease duration did not predict relapses. Vasculitis damage index scores at the last visit were 1 (IQR: 0–2). Lobar pneumonia and perianal abscess were observed in one patient each.

Conclusion: In our series of AAV patients treated during the last 3 years, mycophenolate was efficacious in maintenance of remission with 5% incidence of serious infection.


  PC0096: A classic case of primary biliary cirrhosis Top


Naveen Chandra Reddy Kotha; Bhaskar Medical College and Hospital

A 32-year-old male presented to the outpatient department with complaints of (1) fever on and off for 3 months, (2) jaundice for 1 month, and (3) diarrhea and weight loss for 20 days.

Presenting Illness: Fever was on and off for 3 months, history of jaundice for 30 days, diarrhea and weight loss for 20 days. Jaundice was associated with clay-colored stools. On examination, icterus +, dry mouth with poor oral hygiene, and dental caries were noted. Dry eyes and on systemic examination mild hepatomegaly was present On laboratory investigations, CBP was normal, erythrocyte sedimentation rate was 120, liver function test showed serum bilirubin 11.2, direct 11.3 and alkaline phosphatase was 587, serum glutamic oxaloacetic transaminase and serum glutamic-pyruvic transaminase were 99 and 148, respectively. ultrasonography showed mild hepatomegaly; HIV and hepatitis B surface antigen were nonreactive. Serum calcium was low. antinuclear antibody (ANA) by immunofluorescence was positive with 1:80. ANA profile revealed positive anti-Ro, anti-La and antimitochondrial antibodies.He was treated with low-dose oral steroids, ursodeoxycholic acid, and methotrexate.

Discussion: Primary biliary cholangitis is a chronic autoimmune liver disease with a female gender predominance with female:male ratio being at least 9:1 and more common in the middle age peak incidence in the fifth decade of life.


  OPC0313: Clinical features and outcomes in polyarteritis nodosa: A 15-year experience from tertiary care center in North India Top


Vikas Sharma, Saket Jha, Rajiv Ranjan, Sakshi Mittal, Shankar Naidu, Ritambhra Nada, Manphool Singhal, Mahesh Prakash, Varun Dhir, Ranjana W Minz, Sanjay Jain, Aman Sharma; PGIMER, Chandigarh, India

Background: Polyarteritis nodosa (PAN) is a rare systemic necrotizing arteritis of medium- or small-sized arteries, without glomerulonephritis and not associated with antineutrophil cytoplasmic antibody.

Methods: This was a retrospective analysis of patients diagnosed as PAN at PGIMER.

Results: Thirty-seven cases (22 males and 15 females) were diagnosed as having PAN during the study period. The mean age at presentation was 40.81 ± 13.02 years. Seven patients were hepatitis B surface antigen positive. Neurological involvement was the most common clinical feature and was present in 83.7% patients. More than half of patients had constitutional symptoms such as fever and weight loss. Thirty-three patients received treatment, whereas three patients died before treatment was administered and one patient left against medical advice. All patients received steroids, 19 received cyclophosphamide and one was managed with azathioprine. Three cases were found to be ADA2 deficient and were successfully treated with tumor necrosis factor inhibitors. Nearly three-fourth patients recovered.

Conclusion: High-dose steroids with or without steroid-sparing agents are the major form of therapy. Mononeuritis multiplex was seen in more than three-fourth patients and predicted the need for additional immunosuppressive therapy other than steroids even with five-factor score of 0. With treatment, survival rates have considerably improved.


  PC0097: A rare case of Takayasu's disease with triple-vessel coronary artery disease on a background of retroviral disease Top


Asna Shaikh, Ankit Ganatra, Shailee Chandak, Shubhada Kalke, Neelu Bhojani, Kaushik S Bhojani; Kennisha Rheumatology Care and Diagnostics, Mumbai, Maharashtra, India

Introduction: Takayasu's arteritis (TA) is a chronic inflammatory large-vessel vasculitis with greatest prevalence in Asian people. The incidence of coronary artery involvement has been reported to be <10% 1.2.3. Such involvement may present in the form of stenosis, complete obstruction, aneurysm, or coronary steal syndrome. The precipitant factor of the pathological immune response is generally unknown, and different factors have been implicated, including infection. We report a case of a 38-year-old female on highly active antiretroviral therapy who presented to us with intermittent neck pain, upper back pain, and left arm pain in 2014. The absence of brachial pulse, a difference higher than 10 mmHg in the systolic blood pressure between both arms, an elevated C-reactive protein, and characteristic radiological findings were the criteria for the diagnosis of TA. She remained stable on prednisolone and mycophenolate mofetil for 4 years after which she complained of episodic atypical chest pain radiating to neck, left jaw, and scapula. Coronary angiography was done which revealed a triple-vessel disease.

Discussion: Coronary artery disease can occur in <10% of patients with TA. In addition, very few cases have been reported in the literature on a background of retroviral disease. Such patients usually present with cerebrovascular accident, and no case with coronary artery disease has been reported thus far. The lesions are usually ostial in nature with a recurrence of stenosis after angioplasty and hence do better with coronary artery bypass grafting.

Conclusion: To the best of our knowledge, this is the first such case of Takayasu's disease-associated coronary artery disease on the background of retroviral disease and should be suspected as such even in immunocompromised states.


  OPC0307: Clinical study of ankylosing spondylitis with special reference to cardiopulmonary manifestation Top


Nayanmoni Dutta, P Dihingia, Bishal Agarwalla, S M Baruah, T K Das; AMCH, Dibrugarh, Assam, India

Background: Ankylosing spondylitis (AS) is an inflammatory disorder of unknown cause that primarily affects the axial skeleton; peripheral joints and extraarticular structures are also frequently involved. Characteristic cardiac abnormalities in AS include aortitis, aortic regurgitation, and conduction abnormalities that are seen in up to 9% of patients with AS. The pulmonary manifestations of the disease include fibrosis of the upper lobes, interstitial lung disease and ventilatory impairment, sleep apnea, and spontaneous pneumothorax. Objectives: The objective was to study the clinical manifestation of AS with special reference to cardiopulmonary manifestation.

Materials and Methods: This study was a hospital-based observational study carried out on 65 patients of AS, who fulfill the modified New York Criteria and were admitted or attended various outpatient departments of Assam Medical College and Hospital, Dibrugarh, during a period of 1 year from July 2012 to June 2013.

Results: Out of the total 65 patients, 52 were male and 13 were female. Majority of patients had bilateral radiologic sacroiliitis (92.3%). Inflammatory low back pain was the most common symptom. Acute anterior uveitis was the most common extra-articular manifestation, seen in 20% of patients. Other extra-articular manifestations include cardiac and pulmonary involvement, which is seen in 6.15% patients. Out of the 65 patients, cardiac involvement was found in 4 (6.15%) patients. Pulmonary involvement was found in 4 out of 65 patients.

Conclusion: Cardiopulmonary involvement was found in 6.15% of patients. Cardiopulmonary manifestation was seen in patients with longer duration of disease, and majority of them were asymptomatic and were detected only upon investigation.


  OPC0308: Five-year mortality audit of rheumatology in-patients at CMC Vellore Top


R J Sachin, Arvind Ganapati, Debashish Danda; Department of Clinical Immunology and Rheumatology, CMC, Vellore, Tamil Nadu, India

Background: Systemic autoimmune rheumatic diseases are associated with higher mortality, infection, and disease activity being the common causes.

Objectives: The objective was to study associations of mortality in rheumatology in-patients.

Methods: We undertook a retrospective descriptive study of all deaths in rheumatology in-patients services of our tertiary care teaching hospital in Southern India between August 2013 and July 2018. Clinical details that could have impacted the mortality were retrieved from hospital records.

Results: Total rheumatology inpatient admissions during the last 5 years were 3502, and total mortality was 54/3502 (1.54%). Out of the total fatalities, 44 (81.5%) were females. Majority (85%) succumbed in intensive care unit, whereas 15% deaths were in the wards. Systemic lupus erythematosus (SLE) contributed to most of the mortalities (n = 29, 53.7%), followed by vasculitis (n = 6, 11.1%) and diffuse systemic sclerosis (n = 5, 9.2%). Mortality rates of SLE, vasculitis, and diffuse systemic sclerosis out of total number of in-patients with these diagnosis during this study period were 3.7% (29/774), 3.4% (6/177), and 13.1% (5/98), respectively. The most common cause of death was infection (29/54, 53.7%). Among them, five had leukopenia and 12 had neutrophilic leukocytosis. In patients with infection, 81% had raised C-reactive protein (n = 27) and procalcitonin (n = 21) levels. The culture positivity for blood, urine, and sputum was 59.2% (n = 27), 76.5% (n = 17), and 71.4% respectively, with Gram-negative bacilli (GNB) being isolated in 60.9%. The second most common cause of mortality was disease activity (15/54, 27.7%). Among SLE, all patients who died were females; 17/29 (58.6%) died at first presentation; 22 of them had renal, 17 hematological, 14 cardiac, and 13 central nervous system involvement. The median cumulative steroid intake in SLE patients was 8595 mg (n = 16, 3055, 14,332) of prednisolone; with median SLE disease activity index of 17 (n = 17, 8, 23).

Conclusions: Mortality among rheumatology inpatients is low (1.5%) in our setting, SLE contributing the majority; GNB sepsis was the most common attributable factor, followed by high disease activity.


  OPC0315: Mycophenolate is effective as induction therapy in primary angitis of central nervous system Top


Shyamashis Das, Ashis Datta, Sukalyan Purkayastha, A Shobhana; Department of Rheumatology, Institute of Neurosciences, Kolkata, West Bengal, India

Objective: The objective was to observe the efficacy of mycophenolate in primary angiitis of central nervous system (PACNS) in adult patients as induction therapy in an open-label prospective study.

Methods: From November 2016 to August 2018, 8 patients (6 females) with age range of 34–50 years and minimum follow-up 6 months were collected. Patients were classified as PACNA based on Salvarani criteria. All of them had changes in digital subtraction angiography (DSA) highly suggestive of vasculitis (narrowing, occlusion, or dilation in a segmental pattern affecting multiple cerebral arteries in the absence of changes consistent with atherosclerosis) with raised protein in the cerebrospinal fluid. All patients were given initial methylprednisolone pulse followed by oral prednisolone (1 mg/kg/day) in tapering dose.

Results: All patients were followed up for a median period of 11 months (range: 6–19 months). The most common clinical feature was headache. Brain imaging showed infarcts in four, hemorrhagic infarct in two, and only hemorrhage in two patients. Prednisolone was discontinued in four patients and continued in low dose (2.5–10 mg/day) in rest of them. All of them were on mycophenolate 2 g/day. Inflammatory markers (erythrocyte sedimentation rate/C-reactive protein) were elevated in seven patients and were normalized at 6 months. There was no recurrence or worsening of symptoms in any of the patients till the last follow-up. All of them showed clinical improvement in terms of improvement of headache and neurodeficit. Only one patient has mild residual hemiparesis. DSA was repeated in three patients (at 7, 9, and 14 months) and that showed improvement in one and nonprogression of angiographic abnormalities in two. Median period of mycophenolate mofetil treatment was 11 months (range: 6–19 months). There was no report of significant drug-related toxicity.

Conclusion: We report the first series of PACNS from India treated with mycophenolate. Mycophenolate seems to be efficacious and safe as induction therapy in PACNS.


  OPC0317: Bad obstetric history, a different viewpoint: Experience from a rheumatology unit Top


Asna Shaikh, Ankit Ganatra, Shailee Chandak, Neelu K Bhojani, Shubhada Kalke, Kaushik S Bhojani; Kennisha Rheumatology Care and Diagnostics, Mumbai, Maharashtra, India

Aim: The aim was to analyze the causes and treatment outcomes in patients with bad obstetric history.

Materials and Methods: Patients with miscarriages between 2011 and 2015 were included. Those with no evidence of connective tissue disease or antiphospholipid antibody syndrome were investigated for thrombophilic states, namely hyperhomocysteinemia, protein C deficiency, and protein S deficiency. Patients with obstetric causes were excluded.

Results: One hundred thirty-five patients with 339 pregnancies were evaluated. Two hundred seventy-two miscarriages (80.23%) were found, of which 204 were in the first (75%), 48 second (17.64%) and 20 third (7.35%) trimester, respectively.

Conclusion: Protein C and S deficiency and hyperhomocysteinemia should also be considered as causes of recurrent pregnancy loss. Role of B12 and D3 deficiency needs further research.


  OPC0316: Toxic epidermal necrolysis versus lupus: A great diagnostic dilemma Top


Meghna Dutta, Satyabrata Ganguly, Asif Iqbal, Enam Murshed Khan, Sraboni Ghosh Zoha, Jayanta Kumar Gupta and Suddhasatwya Chatterjee; Apollo Gleneagles Hospitals Limited, Kolkata, West Bengal, India

Discussion: Toxic epidermal necrolysis (TEN) is a common, yet one of the most dreaded dermatological emergencies. TEN-like rash of lupus, a rare entity, is clinically indistinguishable from drug-induced TEN. Unless there is a high index of suspicion, the subtle features of systemic lupus erythematosus (SLE), complemented with laboratory findings, can be easily overlooked in a sick patient presenting with vesiculobullous lesions with peeling of skin, screaming for urgent management.

Case Report: We came across a 24-year-old nulliparous female who was 14 weeks pregnant and presented with vesiculobullous lesions all over the body and oral ulceration. She gave a history of usage of doxylamine succinate + pyridoxine hydrochloride. Antinuclear antibodies (ANAs) by Hep2 in 1:100 dilution were 3+ with immunofluorescence showing fine granular pattern; anti-SS-A and anti-SS-B were strong positive; and antiphospholipid immunoglobulin (Ig)G and IgM levels were normal. Punch biopsy taken from a lesion over the left arm revealed full-thickness epidermal necrosis and a subepidermal cell-poor bulla-containing fibrin.

Discussion: The purpose behind the presentation of this case is that differentiating TEN-like lesions of SLE from drug-induced TEN is a challenge in the practical scenario because TEN is known to occur with increased frequency in connective tissue disease. Primary Sjogren's syndrome was also a differential diagnosis as per the ANA pattern.


  OPC0318: A clinico-serological profile of inflammatory myositis: An experience from rheumatology clinic Top


Nachiket Kulkarni, Anuradha Venugopalan, Arvind Chopra; Centre for Rheumatic Diseases, Pune, Maharashtra, India

Aim: The aim was to study the clinico-serological profile of inflammatory myositis.

Materials and Methods: We present data from 106 patients of connective tissue disorders with dominant inflammatory myopathy (IM) evaluated in CRD where we have patient database since 1996. Standard investigations and autoantibody (AAb) assessment were done (SM, U1-snRNP, SSA-Ro60, SSB, SSA-Ro52, PCNA, PM-Scl, Scl 70, CENP B, Jo-1, Mi2, dsDNA, and Ku).

Results: Antinuclear antibody (ANA) positivity was noted in (46%) in patients with IM. The most prevalent AAb was against Ro52 (10%). Exclusive AAb was found in 24%. AAb noted in patients with predominant dermatomyositis rash were Mi2, SSA-RO60, Ro52, and Ku. Those with predominant polymyositis were Jo1 and CENP B. Overlap myositis patients had the presence of SM, U1-snRNP, Ro 52, SSA-Ro60, PM-Scl. Mi2 positivity was noted in 5%. Jo1 positivity was noted in 12% of patients with features of IM, interstitial lung disease (ILD), RP, and good therapeutic response. Patients with malignancy were negative for ANA and tested AAb. ILD was with the presence of Jo1, Ro52, Ku, Mi2, Scl 70.

Conclusion: The serological signature in IM demonstrated some characteristic association with clinical profile. Notable were ILD with Mi2, Jo1, and R052 and overlap profile with Ro52. Patients with malignancy were negative for tested AAbs.


  OPC0319: Effect of sulfasalazine on fertility of spondyloarthropathy patients Top


Ankit Ganatra, Aasna Shaikh, Shailee Chandak, Shubhada Kalke, Neelu Bhojani, Kaushik Bhojani; Kennisha Rheumatology Care and Diagnostics, Mumbai, Maharashtra, India

Introduction: It is known that sulfasalazine can result in reversible oligospermia in patients of spondyloarthropathy. However, in our experience, a couple of patients did not show reversibility on discontinuation of the drug. This prompted the suspicion that the disease may also play a role in causing oligospermia. To study this aspect, a baseline semen examination of all young male patients was carried out and then repeated while on treatment with sulfasalazine.

Patients and Methods: Semen examination of 197 patients was evaluated at initial presentation to our outpatient department. Normal sperm count was defined as more than 50 million/ml. Those having normal sperm count and motility were initiated on sulfasalazine therapy, and semen examination was repeated at 4 months on treatment. Those having oligospermia at baseline were not initiated on sulfasalazine.

Results: 99/197 patients had never received sulfasalazine or had been off the drug for more than 6 months. 66/197 patients (33.50%) were found to have oligospermia. 61/197 (30.96) patients on sulfasalazine at initial presentation had oligospermia while 16/61 (26.23%) developed it after treatment with sulfasalazine. 10/66 patients improved with the disease treatment.

Conclusions: It is possible that a significant proportion of patients suffer from oligospermia due to the effect of the disease itself. We suggest that all young male patients suffering from spondyloarthropathy be subjected to a baseline semen examination before treating them with sulfasalazine.


  OPC0321: Short-term safety and efficacy of combination of methotrexate with apremilast in patients with psoriatic arthritis Top


Vishad Viswanath, G J Sangeetha, S Arathy, Surabhi Subin; Institute for Rheumatology and Immunology Sciences, Thiruvananthapuram, Kerala, India

Introduction: Apremilast is an orally active small molecule inhibitor with proven efficacy in psoriasis and psoriatic arthritis. There is a paucity of data on its safety and efficacy when used in combination with methotrexate. Herein, we report the short-term safety and efficacy of combination therapy of apremilast with methotrexate in the treatment of psoriatic arthritis.

Methodology: This is a retrospective review of 15 (male:female 7:8) patients with psoriatic arthritis (Classification Criteria for Psoriatic Arthritis Criteria) seen between December 2017 and August 2018. All patients who received a combination of apremilast with methotrexate were included. Eleven patients were on prior treatment with methotrexate and four received combination upfront.

Results: Mean age was 51 years (standard deviation [SD] 10.44). Disease duration was 40.5 months (31.82). The mean duration of treatment was 23.9 weeks (range 18–30 weeks) and the mean dose of methotrexate at baseline was 18.3 mg (SD 6.34). 3/15 patients in the study group discontinued treatment due to side effects (abdominal distention and gastritis in 3 of them and headache in 2). All discontinuation occurred in the first follow-up after initiating treatment. One other patient developed headache of migrainous nature who responded well to flunarazine and continued treatment. Transaminitis was seen in one but recovered following reducing the dose of methotrexate. All except one patient who continued treatment showed improvement in PSDAS scores. The mean change in disease parameters pre- and post-treatment was as follows: 78 joint TJC (8.5 and 3.0), 77 joint SJC (7.9 and 4.5), PSDAS (3.9 and 2.42), C-reactive protein (19 mg/dl and 6.0 mg/dl), health assessment questionnaire score (2.4 and 0.46), PASI (0.79 and 0.27), and mean corticosteroid usage (3.9 mg equivalent of prednisolone and 2.7 mg). Methotrexate dose was reduced in 7/11 patients and regular nonsteroidal anti-inflammatory drug usage was discontinued in 5/6 patients who were taking it at baseline.

Conclusion: Combination of apremilast with methotrexate appears to be safe and effective at least in the short term.


  OPC0322: Significance of asymptomatic antinuclear antibodies positivity in patients referred for bad obstetrics history Top


Ankit Ganatra, Aasna Shaiks, Shailee Chandak, Kaushik Bhojani, Shubhada Kalke, Neelu Bhojani; Kennisha Rheumatology Care and Diagnostics, Mumbai, Maharashtra, India

Aim: The aim was to study the incidence and significance of asymptomatic antinuclear antibodies (ANA) positivity as a cause of bad obstetrics history (BOH).

Materials and Methods: Patients referred with BOH between 2011 and 2015 were studied. Those patients with well-known causes of BOH such as antiphospholipid antibody syndrome (APS) and connective tissue disease (CTD) were excluded. Only those patients who were asymptomatic for a CTD but had tested positive for ANA by immunofluorescence were included and analyzed. Some of these patients were further tested for antigen subsets as well.

Results: Out of 135 patients referred, 46 were asymptomatic ANA positive (34.07%). Mean age was 32.65 years and mean pregnancy loss was 2.04. Out of 94 miscarriages, 74 were in the first trimester (78.7%), 12 (12.76%) second trimester, and 5(5.3%) third trimester. Thirty of the 46 patients underwent testing for antigen subsets (65.22%). Positive ANA was often found to be associated with other abnormalities such as elevated homocysteine (>10 umol/L) in 14 patients (30.43%), Vitamin D3 deficiency (<30 ng/ml) in 29 (63.04%), Vitamin B12 deficiency (<300 pg/ml) in 14 (30.43%), protein C in 2 (4.34%), and protein S in 7 (15.21%) patients. Thyroid-stimulating hormone was elevated in one patient (2.17%). Isolated ANA abnormality as a cause of BOH was present in seven patients (15.21%). Sixteen (34.78%) patients had successful outcomes, 4 (8.7%) patients were pregnant when assessed, 6 (13.43%) had miscarriages despite treatment; 17 (36.96%) were on treatment. Outcome was not known in 5 (10.86%) patients.

Conclusion: Patients presenting with BOH should be assessed for ANA as a cause even if asymptomatic. Significance of weak-positive ANA in patients of BOH may need further research.


  OPC0324: Clinico-pathological profile of lupus nephritis patients and short-term treatment outcome: A study from Northeast India Top


Jamil. P K Bhattacharya, Bhupen Barman, L N Phibakordor, M V Subramanya, Nilanjan Mazumdar; NEIGRIHMS, Shillong, Meghalaya, India

Aims and Objectives: The aim and objective were to study the clinico-pathological profile of lupus nephritis patients and to assess the short-term treatment outcome.

Methodology: This was a prospective observational study that includes only adult patients with diagnosis of biopsy-proven lupus nephritis. Data related to the demographic details of the patients were collected along with clinical data, laboratory reports, and kidney biopsy reports. Patients with class III and class IV lupus nephritis were put on immunosuppressive therapy with either pulse cyclophosphamide with steroid or mycophenolate mofetil (MMF) with steroid. Treatment response at the end of 6 months of initiation of therapy in terms of remission in both groups is assessed.

Results and Observations: A total of 65 patients from both gender were included in the present study. A total number of female and male patients were 62 (95.4%) and 3 (4.6%) respectively, and female-to-male ratio was 21.6:1. Majority of the patients belonged to age group of 18–30 years of age (78.5%) followed by 31–40 years (10.8%) and 41–50 years (9.2%). On the basis of renal biopsy report, majority of patients had class IV Lupus nephritis (50.8%) followed by class III (30.8%), then class II (07%), and class V (7.7%). Complete remission wad achieved in 52.9% in cyclophosphamide group and 58.3% in MMF group among the patients who were followed up for 6 months of initiation of treatment.

Conclusion: Majority of the patients diagnosed with lupus nephritis at presentation were found to have class III and IV lupus nephritis. Remission was achieved in little more than half of the patients at the end of 6 months.


  OPC0323: Incidence of bad obstetric history in patients with antiphospholipid syndrome: Experience from a single rheumatology center Top


Asna Shaikh, Ankit Ganatra, Shailee Chandak, Neelu K Bhojani, Shubhada Kalke, Kaushik S Bhojani; Kennisha Rheumatology Care and Diagnostics, Mumbai, Maharashtra, India

Aim: The aim was to study the analysis of bad obstetrics history (BOH) in patients with primary and secondary antiphospholipid syndrome (APS).

Materials and Methods: Patients with APS from 2011 to 2015 were classified into primary and secondary APS [Tables 1 and 2]. The BOH group was analyzed for differences in presentation and antibody positivity.

Results: N = 49. BOH = 24 (48.97%). Non BOH manifestations = 25 (51.02%). The BOH group had 43/59 pregnancy losses (72.88%) (mean 1.79 per patient). Six patients were pregnant at initial presentation. Six patients (25%) had successful pregnancy outcomes.

Conclusion: Fifty percent of APS patients had BOH. BOH was the main manifestation in primary APS (80%). Two-thirds of the patients have first-trimester losses in both primary and secondary APS. Second-trimester loss was more common in primary APS as against third-trimester losses in secondary APS.






    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6], [Table 7], [Table 8], [Table 9]



 

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