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 Table of Contents  
IRACON 2017 - POSTER PRESENTATIONS
Year : 2017  |  Volume : 12  |  Issue : 5  |  Page : 16-132

Poster Presentations


Date of Web Publication24-Nov-2017

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How to cite this article:
. Poster Presentations. Indian J Rheumatol 2017;12, Suppl S2:16-132

How to cite this URL:
. Poster Presentations. Indian J Rheumatol [serial online] 2017 [cited 2019 May 22];12, Suppl S2:16-132. Available from: http://www.indianjrheumatol.com/text.asp?2017/12/5/16/219140


  PB025 Rheumatoid Arthritis Top


Effect of common polymorphisms in the methotrexate pharmacokinetic pathway on efficacy/adverse effects and methotrexate polyglutamate levels in RA

Amit Sandhu, Archana Bhatnagar, Varun Dhir; Postgraduate Institute of Medical Education and Research, Chandigarh, India

Purpose: This study investigated the impact of seven common polymorphisms in genes involved in methotrexate (MTX) pharmacokinetics on response, adverse effects and methotrexate polyglutamate (MTXPG) levels in rheumatoid arthritis.

Methods: This study enrolled 117 RA patients who were treated prospectively with MTX for 24 weeks. Patients were categorized on the EULAR criteria into responders (good and moderate) and non-responders. Adverse effects were ascertained using a questionnaire. The following polymorphisms were ascertained using hydrolysis probes - rs1045642 (ABCB1 3435C>T), rs1128503 (ABCB1 1236C>T), rs10106 (FPGS 1994A>G), rs1544105 (FPGS G>A), rs11545078 (GGH 452C>T), rs3758149 (GGH -401C>T) and rs1051266 (RFC1 80G>A). RBC MTXPG1-5 levels were determined using HPLC at 4,8,16 and 24 weeks.

Results: There was a significant association of the GGH 452C>T CC genotype (OR 9.5, 95% CI 1.2 to 76.0) with response to MTX. On logistic regression, higher DAS28 (3) at baseline and GGH 452CC genotype were significantly associated with response [Table 1]; the accuracy of the model was 75%. The FPGS 1994A>G GG genotype was associated with a significantly lower risk of adverse effects to MTX (Odds Ratio 0.3 (95% CI 0.1 to 0.6)). On logistic regression, FPGS 1994GG genotype and lower BMI were significant predictors for adverse effects with an accuracy of 66%. The other polymorphisms were not associated with response or adverse effects. None of the polymorphisms were associated with change in MTXPG levels.



Conclusion: GGH 452 CC genotype was found to be associated with response to MTX and FPGS 1994A>G GG with a lower risk of adverse effects; however, not by change in MTXPG levels.

Image file PB025.PNG


  PB026 Rheumatoid Arthritis Top


MicroRNA-132, miR-146a, miR-155 as potential biomarkers of methotrexate response in patients with rheumatoid arthritis

Ankita Singh, Pradeepta Sekhar Patro, Amita Aggarwal; SGPGIMS, Lucknow, Uttar Pradesh, India

Introduction: MiRNAs regulate signaling pathways of inflammation and thus can serve as biomarkers. Patients with Rheumatoid arthritis (RA) have high expression of miR-155, miR-146a and miR-132 in their peripheral blood cells. As one third of patients do not respond to methotrexate (MTX), the first line therapy in RA, we studied if levels of these miRNAs can predict response to MTX.

Methods: Patients with RA (ACR 2010 criteria), DMARD naïve with active disease (DAS28 >3.2) were enrolled. Blood sample was collected. All patients were treated with MTX by gradually increasing dose to a maximum of 25 mg/week or the maximal tolerated dose. No corticosteroids were given. At 4 months disease activity was measured by DAS28 and EULAR response was assessed. miR-132, miR-146a and miR-155 were measured using real time qPCR in the whole blood RNA. Let7a was used as a housekeeping gene.

Results: 80 patients (83.8% females; median age with IQR 40 (17) years); median (IQR) duration of disease 2 (2.06) years; median (IQR) DAS28 4.53 (0.96) were enrolled. Among 80 patients 59 were classified as good responders while 14 were moderate- responders and 7 were non-responders at 4 months. The baseline miRNA levels did not correlate with the baseline DAS28 score or any other clinical parameters of RA patients. All the three miRNA’s were lower in responders as compared to non-responders but it was significant only for miR-155 (p=0.016) with median value -7.59 (1.10) for responders (n=73) and -6.58 (2.0) for non-responders (n=7). ROC curve analysis showed that miR-155 with cutoff value of -7.3(dtCt) is predictor of nonresponse to MTX treatment with a sensitivity of 67.1% and a specificity of 71.4% (AUC: 0.777, p=0.016).

Conclusion: MicroRNA-155 may serve as a good biomarker of response to MTX.


  PB027 Rheumatoid Arthritis Top


Decline in CD8+IFNγ+ subset occurs on treatment with methotrexate in rheumatoid arthritis

Varun Dhir, Amit Sandhu, Prabhdeep Kaur, Archana Bhatnagar1, Veena Dhawan; Postgraduate Institute of Medical Education and Research, 1Panjab Univesity, Chandigarh, India

Background: CD8 T cells comprise 40 % of all T cells in the synovial compartment. They can be divided by cytokine production into CD8+IFNγ+, CD8+IL4+ and CD8+IL17+. The effect of methotrexate on these circulating CD8 subsets has not been well described.

Methods: Patients who were 18 to 65 years of age and having active rheumatoid arthritis were treated with methotrexate for 24 weeks. Disease activity was measured using the disease activity score and response to treatment assessed by EULAR criteria. At 0 (baseline) and 24 weeks (post methotrexate), PBMCs were isolated using density gradient centrifugation and stimulated with PMA /Ionomycin (with Brefeldin) for 5.5 hours. Surface staining was done using anti CD3/ anti CD8 and intracellular cytokine staining with anti Interferon g, IL17 and IL4. 30,000 events were acquired and frequencies of CD3+CD8+INFγ+ cells, CD3+CD8+IL17+ and CD3+CD8+IL4+ were determined. Cytokine bead array was used to determine levels of IFNγ, IL12, IL10, IL4 and IL17 in plasma at 0 and 24 weeks. Cell frequencies and cytokine levels at baseline and 24 weeks were described by using median (IQR=interquartile range, 25th- 75th percentile) and compared using non-parametric paired test (Wilcoxon signed rank).

Results: This study included 67 patients (F:M=4:1) with rheumatoid arthritis, 57 (85%) being RF positive and 20 receiving prednisolone at baseline. They were treated with methotrexate for 24 weeks, with mean dose at completion of study being 22.9+/-3.0 mg per week. CD8IFNg+ cells declined from 37.2 (IQR 19.4-60.2) to 22.7% (IQR 8.5-49.7), p=0.04 and there was marginal increase in CD8-IL17 cells from 0.3 (IQR 0.1-0.6) to 0.4 (IQR 0.2-1.2), p=0.006. In non-responders, there was a significant increase in CD8-IL17 (p=0.01) that was not seen in responders. There was a significant decline in the circulating. There was a significant decline in the circulating levels of IL-12 and IL17 and increase in IL4 with treatment [Table 1].



Conclusions: Methotrexate leads to changes in circulating CD8 subsets, predominantly decline of the CD8+IFNγ+ subset, that may be explained due to reduction in the polarising cytokine IL12. This may be one of the mechanisms responsible for its effect in RA.


  PB028 Rheumatoid Arthritis Top


Alleviation of rheumatoid arthritis by cerium oxide nanoparticles: In vitro and in vivo preclinical proof of concept

Prince Allawadhi, Amit Khurana1, Divya Vohora, Chandraiah Godugu1; Department of Pharmacology, School of Pharmaceutical Education and Research, Jamia Hamdard, New Delhi, 1Department of Regulatory Toxicology, National Institute of Pharmaceutical Education and Research, Hyderabad, Telangana, India

Background: Arthritis and related disorders, including rheumatoid arthritis (RA) are common, affecting millions of people throughout the world. RA treatment is hampered due to lack of patient compliance therapy and novel treatment interventions to tackle the burgeoning prevalence of RA. CONPs have emerged as potent anti-inflammatory agent which may prove beneficial for the therapy of RA.

Objective: To evaluate the anti-inflammatory effect of cerium oxide nanoparticles (CONPs) in Lipopolysaccharide (LPS) induced inflammation in vitro in Raw 264.7 macrophages and in vivo in Freund’s complete adjuvant induced experimental model of rheumatoid arthritis in rats.

Methods: Nanoparticles were thoroughly characterized by zetasizer, FTIR, SEM and pXRD. Inflammation was induced in Raw macrophage 264.7 cells with LPS (500 ng/mL). The cell viability in presence of CONPs was assessed by MTT assay. Effect of CONPs (25-200 Âμg/mL) on LPS induced nitrosative stress was evaluated by Griess reagent method. In addition, effect of CONPs intervention on cellular reactive oxygen species (ROS) and SOD were assessed by DCFDA and Mitosox staining, respectively. Mitochondrial membrane potential (MMP) was assessed by JC1 staining. For in vivo study, rheumatoid arthritis will be induced by Freund's complete adjuvant (0.1 mL/rat) by subplantar administration in region of right hind paws through intradermal (ID) route at day first only followed by treatment with cerium oxide nanoparticles at three doses (0.5, 1 and 2 mg/Kg). The in vivo assessment of protective effect of CONPs will be carried out by assessment of all relevant parameters (scoring, X-ray, biochemistry, ELISA, RT-PCR).

Results: CONPs have shown dose dependant reduction in the cellular nitrite secretion as assessed by Griess reagent method. The results of DCFDA and Mitosox clearly indicate remarkable protection of macrophages from LPS induced oxidative stress. In addition, the results of JC1 staining show protective effect of CONPs against LPS induced MMP changes. Moreover we did not observe significant cell death of macrophages even up to 1 mg/mL of CONPs indicating remarkable safety profile of CONPs. The in vivo study is currently under progress.

Conclusion: Our study suggests that CONPs may be used for effective therapy of RA and may overcome the associated complications.


  PB029 Rheumatoid Arthritis Top


Differential expression of CD8+T cells of rheumatoid arthritis patient after bacterial infection

Archana Tripathy, Shweta Khanna, Prasanta Padhan1, Bhawna Gupta1#; School of Biotechnology, Kalinga Institute of Industrial Technology, 1Department of Rheumatology, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India

Background: RA is a progressive autoimmune disease with systemic complications and early deaths. Though the pathogenesis of RA is not yet fully elucidated, it is known to be induced by environmental factors on a genetically susceptible background. The human microbiota is considered a major player in health-related processes, either by direct influence or through interaction with other health determinants, including genetics, diet, life¬style, medical practices and hygiene. Alterations in gut microbiota can induce the activation of effector T cells over iTregs and, consequently, trigger the development of autoimmune/inflammatory diseases.

Objective: These studies identified specific gut commensals that are able to induce immune response of CD8+T cells which can contribute to abnormal activation of pro inflammatory cytokines and enhance the acute inflammation in rheumatoid arthritis.

Methods: Seventy RA patients and seventy healthy donors participated in this study. Clinical variations like disease duration, number of actively inflamed joints, CRP, RF, Anti-CCP and ESR were recorded for each patient and DAS 28 scores were calculated with the help of the clinician. Different cytokines level was checked after stimulation of CD8+T cells with gut microbiota by flow cytometry. We analyzed the expression of bacterial TLR in transcript level by real-time PCR and protein level by flow cytometry in CD8+T cells of RA patients.

Results: Significant increased of different inflammatory cytokines in both transcript level and protein level in patients with RA compared to healthy donors after stimulation with bacteria (p<0.005). We got a strong positive correlation between bacteria TLR expression and DAS 28 score (rs= 0.96). The ROC curve analysis confirmed the significance of TLR4 expression in RA patients (Area=0.77, p<0.01). Expression of co-stimulatory receptors was observed in CD8+T Cells of RA patients upon bacterial infection.

Conclusion: In summary, our data suggest an increased expression of cytokines and cytolytic enzymes in CD8+T cells due to bacterial infection, which play a major role in inflammation of RA patients.


  PB030 Rheumatoid Arthritis Top


Evaluation of rheumatoid arthritis pain scale: A patient reported assessments questionnaire in the self assessment of disease activity

H Singh, M Gaikwad, S Arora, J Singh, R Mathur, M Yadav; Department of Medicine and Rheumatology, Pt B.D. Sharma PGIMS, Rohtak, Haryana, India

Introduction: Rheumatoid Arthritis is a prototypic chronic inflammatory arthritis in which complex nature of pain is difficult to assess by routine disease activity scale (DAS28,CDAI). RAPS (Rheumatoid Arthritis Pain Scale) captures multidimensionality of pain in a comprehensive manner encompassing 4 subscales: sensory discriminative, physiologic, affective and cognitive subscale with 24 questions and score ranging from 0-144 (each question scoring from 0 (never) to 6 (always). So it could be a potential tool for self assessment by patient.

Objective: To study the correlation of Rheumatoid arthritis pain scale (RAPS) with disease activity score DAS28 and CDAI.

Methods: One hundred patients of Rheumatoid Arthritis reporting to rheumatology clinic were subjected to RAPS questionnaire and disease activity assessment using DAS28 and CDAI pain at baseline and followed after 3 months. Scores were recorded on a standard case record sheet and results were analysed using SPSS version 16 at baseline and at 3 months.

Observations: Mean Age of the study group was 43.28 years. Mean RAPS score of the study group at baseline and at 3 months were 62.91± 21.99 and 34.4±16.32 respectively, Mean DAS28 score of the study group at baseline and at 3 months were 5.59±1.22 and 4.23±1.11 respectively;and Mean CDAI score at baseline and at 3 months was 25.24±11.22 and 14.85±7.51 respectively. RAPS was found to be correlated significantly (using Pearson's correlation coefficient) with DAS28 at baseline (Ϊ_ =0.811) and at 3 months (Ϊ_ =0.827); and with CDAI at baseline (Ϊ_= 0.770) and at 3months (Ϊ_= 0.792).

Conclusion: RAPS had a strong correlation with disease activity parameters DAS28 and CDAI and could be a good utility tool for the self assessment by the patient himself which may help in day to day busy clinic practice.


  PB031 Rheumatoid Arthritis Top


Utility of implementing squeeze test of forefeet into the conventional DAS-28 disease activity score in patients of rheumatoid arthritis

H Singh, M Mangla, A Kalra, J Singh, R Mathur, N Kumar; Department of Medicine and Rheumatology, Pt B.D.S. PGIMS, Rohtak, Haryana, India

Background: Presently available disease activity assessment tools (DAS28 and CDAI) are effective but don’t involve forefeet while estimating disease activity. The feet are frequently affected in both early and established RA, and foot impairment is an important cause of disability during the course of the disease. A clinically simple solution may be implementation of the squeeze test of forefeet into disease activity scores such as the DAS28-squeeze disease state and upgrading DAS28 disease state categorizations one state above if the results of the squeeze test are positive. There is paucity of such a study in the Indian subcontinent.

Objective: To compare the results of DAS28-squeeze test to DAS28 and CDAI disease activity scores in Rheumatoid Arthritis.

Methods: A total of 100 patients of Rheumatoid Arthritis (RA) as per ACR criteria (1987) were assessed for disease activity using DAS28 squeeze, DAS28 and CDAI scores. DAS28-squeeze test was assessed by including the results of midtarsal tenderness. Spearman’s correlation coefficients were computed to correlate DAS28 squeeze, DAS28 and CDAI scores. Statistical significance of the level of agreement of the different scales was evaluated using kappa statistics. Cronbach’s alpha was computed to measure the reliability of DAS28 squeeze, DAS28 and CDAI scores.

Results: Mean age of the study group was 43.9 years. Mean DAS28 squeeze score of the study group was 3.92, Mean DAS28 score was 5.58 and mean CDAI score was 27.96. DAS28 squeeze was found to be correlated significantly (all p<0.001) with DAS28 and CDAI. Cronbach’s alpha was highest for the DAS28 squeeze. DAS28 had a good agreement with both the complex indices.

Conclusion: DAS28 squeeze had a strong positive correlation to DAS28 and CDAI for assessment of disease activity and can be an effective assessment tool for calculating disease activity in RA patients as it involves the forefeet.


  PB032 Rheumatoid Arthritis Top


Assessment of platelet indices correlation with disease activity in rheumatoid arthritis

H Singh, A Kumar, R Jagota, N Marwah, R Mathur, B Kiran; Department of Medicine and Rheumatology, Pt B.D.S PGIMS, Rohtak, Haryana, India

Background: Rheumatoid arthritis (RA) is a chronic systemic inflammatory disorder requiring regular disease activity assessment by disease activity scores. Various new disease activity markers (both biochemical and haematological) are being studied. Amongst hematological parameters platelet indices estimation appears to be most convenient.

Objective: To study the effect of platelet indices (count and volume) with disease activity using DAS28 and CDAI score in Rheumatoid Arthritis.

Methods: A total of 100 patients of Rheumatoid Arthritis (RA) as per ACR criteria (1987) reporting to the Out Patient Department of Rheumatology Clinic of Pt. B.D. Sharma P.G.I.M.S., Rohtak were enrolled in the study. All subjects were assessed for disease activity (using DAS28 and CDAI) along with platelet indices(count/volume) estimation. A repeat assessment for both parameter was done at 3 rd month of drug therapy.

Results: Mean platelet count at baseline was 4.10 + 0.70 lakhs/mm and at 3 months (M3), it was 3.42 + 0.37 lakhs/mm. The decrease in platelet count was statistically significant (p value<0.001) at follow up. Mean platelet volume (MPV) at baseline was 7.41 + 0.79 fl and at M3,it was7.88 + 0.70 fl, it was a statistically significant increase in MPV (p value<0.001) at follow up. Assessment of disease activity made using DAS28 and CDAI showed--. Mean DAS28 score was 6.74 + 0.82 at baseline and 2.96 + 0.65 at M3; and Mean CDAI score was 39.88 + 11.57 at baseline and 6.27 + 3.27 at M3. The decrease in both these scores was statistically significant (p value<0.001) at follow up. Platelet count was significantly correlated (using Pearson's coefficient) with both DAS28 and CDAI with a statistically significant (p value<0.05). Mean platelet volume was not found statistically significantly correlated with both DAS28 and CDAI.

Conclusion: Amongst platelet indices, platelet count was found to be strongly correlated with disease activity in Rheumatoid Arthritis and so may be used as haematological lab tool for assessing disease activity due to its easy availability.


  PB033 Rheumatoid Arthritis Top


A Comparison of routine assessment of patient index data 3 with disease activity score 28 and clinical disease activity index for monitoring disease activity of rheumatoid arthritis patients atttending rheumatology OPD of medical college Kolkata

Amit Kumar Das, Rathindra Nath Sarkar, Ritasman Baisya, Chandan Kumar Das, Urmimala Bhattacharjee; Department of Medicine, Medical College, Kolkata, West Bengal, India

Background: Rheumatoid arthritis (RA) is a chronic inflammatory disease marked by a symmetric peripheral polyarthritis. Disease activity is measured by different activity indices for monitoring treatment outcome - DAS28, CDAI etc. RAPID 3 is a new scoring system which is easy to perform and requires less time.

Objective: To monitor disease activity in RA patients with DAS28, CDAI, and RAPID3 at initial visit and at follow up visit on 2 and 4 months, and to study the correlation and agreement of RAPID 3 with DAS28 and CDAI in terms of measuring disease activity/severity and for monitoring response to treatment at 2 and 4 month follow up.

Methods: 105 adult literate persons (≥16 yrs.) having rheumatoid arthritis according to The 2010 ACR EULAR revised classification criteria attending OPD of Medical college Kolkata were recruited to be part of this study. They were evaluated for disease activity by DAS28, CDAI, and RAPID3 scores. Response to treatment at 2 month and 4 month follow up was measured by these scores. Achievement of treatment target was defined as conversion from high/moderate disease activity at initial visit to low activity/remission at follow up visit.

Result: The mean DAS28 (0-10), CDAI (0-76), RAPID 3 (0-30) were 4.73, 16.72, 10.72 respectively. There was substantial agreement between DAS28 and RAPID3 severity categories (kappa = 0.959, p. v <0.0001) and also between CDAI and RAPID3.

Conclusion: RAPID 3 score can be calculated in less than 10 seconds making it attractive for use in usual clinical care, particularly in busy clinical settings. Hence, it can be used to guide treatment decisions in RA and can prove to be of considerable value when compared to qualitative RA care.


  PB034 Rheumatoid Arthritis Top


Understanding molecular mechanisms of rheumatoid arthritis

Bhawna Gupta; School of Biotechnology, Kalinga Institute of Industrial Technology, Bhubaneswar, Odisha, India

Aberrant immune responses characterize autoimmune disorders wherein lymphocytes are recognized as key players. Role of CD8+ T cells in these diseases has been less defined however we found that these cells are activated in patients with autoimmune rheumatoid arthritis (RA) with increased expression of cytolytic granules and inflammatory mediators thereby modulating immune responses contributing to disease severity. Though unconventional expression of different Toll Like Receptor (TLRs) on CD8+ T cells has been proposed but their expression and role in T cell activation and differentiation in RA still remains obscure. We recently found an increased expression of TLR4 on peripheral CD8+ T cells of RA patients skewing CD8+T cells towards activated and inflammatory phenotype thereby playing a significant role in pathogenesis and progression of RA. In continuation we could observe that interaction of CD8+ T cells with live pathogenic bacteria can trigger T cell activation however a probiotic bacterial colony can reduce the T cell activity thereby subsiding expression of proinflammatory milieu. We also observed an activation induced differential T cell metabolic transcriptome of RA patients. We also focus on understanding the role of mitochondria in pathogenesis and progression of RA. Interestingly we could observe an altered mitochondrial membrane potential, phenotype and function in peripheral blood monocytes of RA patients in comparison to the control cohort. The mitochondrial transcriptome analysis revealed a differential transcript expression in RA patients. The mitochondrial proteome analysis revealed differential expression of many functionally relevant proteins in our case control cohort. The next generation sequencing analysis revealed a differential mutation burden in mtDNA in Indian population that may impact the functional capacity of different mitochondrial genes. These observations have advanced our understanding of the immunological basis of the diseases and thus bring us to a conclusion that while considering therapeutic interventions we may now have to take into account the effect on CD8+ T cells and on the metabolic machinery. We may need to perturb effector CD8+ T cell functions as well as mitochondrial refurbication for a better therapy.


  PB035 Rheumatoid Arthritis Top


Factors affecting methotrexate non-compliance in systemic autoimmune rheumatic diseases a study by nurse counselors and allied health professionals

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

Background: Methotrexate (MTX) is universally regarded as the 'anchor drug' in the management of rheumatoid arthritis (RA). It is usually administered orally or subcutaneously in once weekly doses. Non-compliance with MTX in patients with SARDs among the common causes of poor disease control.

Objectives: To assess the degree of non-compliance to MTX treatment and to identify the reasons for the same.

Methods: This prospective study included 271 patients with RA. They were asked about the compliance to the advised MTX dose during their follow-up visit to the clinic. Those who were noncompliant were then enquired about the reasons for noncompliance. The results were recorded in a predesigned form. The results were analysed correlating noncompliance with demographics, MTX adverse effects, MTX non response, personal-family issues and patients' understanding regarding the drug.

Results: 1. 222 (81.9%) patients were fully compliant with the prescribed MTX dose. 2.49 (18.08) were not compliant, to MTX. The noncompliance varied from occasional to complete default. The factors that were identified for non-compliance included: a) 22 (44.8%) patients had MTX intolerance that led 9 (40.9%) patients to completely discontinued the medicine and 13 (59.09%) decreased the MTX dose. b) 1(2.04%) patients discontinued medicines as they had achieved remission and believed that they were 'cured'. c) 4 (8.16%) patients were not aware of the importance of regular long-term treatment with regular follow-up in the rheumatology clinic. d) 7 (25.9 %) patients developed adverse effects of MTX (oral ulcer, transaminitis) that required dose modification/temporary discontinuation. e) 5 (18.5 %) patients were lost to follow-up. f) 4 (14.8%) patients reported non-availability of MTX in the place of their residence. g) 4 (8.16 %) patients simply forget to take the medicines. h) 2 (4.08%) patients were advised to discontinue treatment by other caregivers (non-rheumatologist) who also advised that the patient shifts over to ' much safer alternative systems of medicine'.

Conclusion: Most frequently reported reasons for non-compliance to MTX were intolerance. The patients need more intensive and focused counselling about the management of intolerance and long term treatment. Correct information and positive attitude towards medication could help in increasing the compliance.


  PB036 Rheumatoid Arthritis Top


Functional characterization of a pharmacologically relevant kinase with implication for rheumatoid arthritis

Anuj Kumar Pandey, Jyotsna Singh, Uma Kumar1, Maumita Kanjilal1, Chandra Shekhar Yadav2, B K Thelma; Department of Genetics, University of Delhi South Campus, Departments of 1Rheumatology and 2Orthopaedics, All India Institute of Medical Sciences, New Delhi, India

Chronic joint inflammation and progressive destruction of cartilage and bone ultimately leading to severe pain and loss of function are characteristics of Rheumatoid arthritis (RA), which afflicts around ~1% of the population globally. Conversely, osteoarthritis (OA) is a non-inflammatory degenerative arthritis with primary defect in cartilage rather than in synovium which leads to joint damage and thus serves as a good control for studies in RA. Different cell types in the synovium secrete various cytokines and metabolites which influence cell type specific signalling pathways. Of these, synovial fibroblasts (SFs) are the major effector cell type that are primarily responsible for cartilage invasion and degradation of the synovial membrane in RA and TNFα further augments action of SFs in disease condition. Despite the advent of anti-cytokine therapies that decrease inflammation, there is no cure, and the disease pathogenesis remains enigmatic. Functional characterization of a few pharmacologically relevant gene(s) using patient derived SFs may provide significant insights into RA pathogenesis and consequently to develop new therapeutic targets. Blood, synovial fluid and synovial tissue from RA (n=3) and OA (n=3) patients undergoing total knee replacements at AIIMS, New Delhi were collected with ethical clearance and used for RNA and western blot studies. Synovial fibroblasts (RASF) derived from synovial tissue samples were checked for homogeneity by FACS using surface markers and used for characterization of candidate genes by expression based studies. Real Time PCR experiments confirmed the expression of several pharmacologically relevant genes including ERK, COX-2, PEG2, TNFα, IL-1ÃΫ, MMPs and proinflammatory markers such as IL-6, IL-8 in blood of RA, OA and healthy controls and in RASF. Western blots confirmed protein expression in RASFs and in synovial fluid. Our results confirming the expression of a pharmacologically important kinase and a few downstream and/or interacting genes in RASF, which may help delineate the mechanistic underpinnings of RA will be presented.


  PB037 Rheumatoid Arthritis Top


Unravelling mitochondrial dysfunction in rheumatoid arthritis

Shweta Khanna, Archana Tripathy, Prasanta Padhan1, Bhawna Gupta#; School of Biotechnology, Kalinga Institute of Industrial Technology, 1Department of Rheumatology, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India

Background: Rheumatoid arthritis (RA) is a chronic, inflammatory, autoimmune disease associated with systemic, extra-articular and articular effects, causing permanent disability, early morbidity; making the patient compromised with a worldwide prevalence of 0.8%, commonly effecting women with a rate of 0.7% in India. With improved and developing therapeutics, this disease needs special focus for improved diagnosis and better treatment. The hyperactivity of immune cells is responsible for pathogenesis and progression of the disease. This study unravels the changes in mitochondria of RA patients which may be a potential reason for abnormal functioning of immune cells against self-antigens and occurrence of the disease.

Objectives: In this study we examine the following aspects of mitochondrial functions in the peripheral blood mononuclear cells (PBMCs) of patients and their paired control samples: 1) Change in mitochondrial membrane potential (MMP); 2) mitochondrial mass; 3) mitochondrial superoxide and 4) ATP levels.

Methods: Patients satisfying the 2010 ACR/EULAR classification criteria for RA diagnosis were enrolled in this study. PBMCs of RA patients and controls were collected by differential gradient centrifugation. MMP, mass and superoxide levels were measured using respective commercially available dye using flow cytometry. ATP levels were measured by lysing equal number of cells from patients and controls using ATP measurement kit.

Results: In our case control cohort, we found a significant decrease in MMP (p<0.005) in PBMCs of RA patients where the change in mitochondrial mass was insignificant. The mitochondrial superoxide levels were found to be significantly low (p<0.05) in PBMCs of RA patients with significantly low (p<0.005) total cellular ATP as compared to controls.

Conclusions: Our results indicate reduced potential and mitochondrial superoxides with decreased total cellular ATP. Results depict dysfunction in basic mitochondrial activities which may be a reason for abrupt functioning of immune cells, leading to autoimmunity in RA patients.


  PB038 Rheumatoid Arthritis Top


Efficacy of counselling in busting the dietary myths and misconceptions among patients with rheumatoid arthritis

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

Background: RA is a major health problem with a prevalence of approximately 0.75% in India. Patients with RA are at higher risk of malnutrition due to the chronic nature of the disease as well as the prevailing myths and misconceptions related to dietary restrictions. Most of these patients are malnourished, anemic with poor muscle mass. The scientific evidence favours a normal balanced diet in RA with no proof that any dietary restrictions help ameliorate the disease.

Objectives: To determine the various dietary myths and misconceptions among patients with RA and to study effects of counselling to bust such myths and misconceptions.

Methods: Prospective cross-sectional study a total of 200 adult patients with RA were included. Diet-related counselling was conducted at the time of their scheduled clinic visit. They were told about the importance and the advantages of normal healthy balanced diet. The futility of trying the various kinds of unproven and harmful dietary restrictions were emphasized. The effect of dietary counselling was recorded during the follow-up interview and assessed for the outcome of advice given at the previous session. The results were statically analysed.

Results: 15% patients were aware that dietary restrictions have no role and 85% of the patients had a belief that in the treatment of RA, dietary restrictions play a central role with particular emphasis on restricting protein containing food items and certain common vegetables. These included tomatoes, citrus items, rice, pulses (specially urad dal i.e. split black gram) and yoghurt. The patients had been forcefully instructed against ingesting these items by Ayurvedic physicians and Homoeopaths (35%), family members and friends (30%), non-rheumatologist medical colleagues (20%), their own belief (10%), and from popular TV shows (5%). The result of persist counselling and creating awareness only 20% patients had left their self imposed dietry restrictions.

Conclusion: The sources of such misinformations were multiple. These myths are so deeply rooted in society that a single counselling session may not be sufficient to bring about behavioural changes. It is evident that there is a great need for continuous health education. This aspect requires further study.


  PB039 Spondyloarthropathy Top


Nuclear magnetic resonance based serum metabolomics revealed distinctive metabolic signatures of reactive arthritis compared to rheumatoid arthritis

Dinesh Kumar, Durgesh Dubey, Smriti Chaurasia, Sandeep Chaudhary, Rajeev Singh, Anupam Guleria, Ramnath Misra; Department of Clinical Immunology, SGPGIMS, 1Centre of Biomedical Research, SGPGIMS, Lucknow, Uttar Pradesh, India

Background: Reactive arthritis (ReA) and Undifferentiated Spondyloarthropathy (uSpA) have distinct pathogenetic pathways of joint involvement as compared to Rheumatoid arthritis (RA). We hypothesize that, consequently, the metabolic pathways would be different in these disparate entities. Hypothesis free metabolomic analysis will lead us to focus on the pathways to pursue further.

Objective: To compare the metabolomes in the sera of patients with ReA/uSpA and RA.

Methodology: Metabolic profiles of sera collected from 52 patients with ReA/uSpA (median age= 29 years, sex M/F ratio=44:8) and 29 patients with RA (median age= 40 years, sex M/F ratio=2:27) were obtained using high-resolution 1D 1H CPMG. The metabolic profiles were compared using orthogonal partial least square discriminatory analysis (OPLS-DA) and the discriminatory metabolites were identified based on variable importance in projection (VIP) score >1.0 and further evaluated for statistical significance (p-value <0.05) and diagnostic potential using receiver operating curve characteristics (ROC) analysis.

Results and Discussion: An exquisite separation between ReA and RA groups in 2D score plot of OPLS-DA clearly revealed that the metabolic profiles of ReA patients are distinctively different compared to RA patients [Figure 1]. Compared to patients of RA, the sera of ReA patients were characterized by (a) elevated levels of glucose, lactate, acetate, creatinine, 3-HydroxyButyrate and various amino acids (such as valine, leucine, arginine, glutamate, glutamine, and phenylalanine), whereas (b) the serum levels of lipid and membrane metabolites (including low/very-low density lipoproteins, PUFA, choline/GPC and acetone) were decreased. These metabolic alterations clearly hinted that ReA patients exhibit altered membrane and possibly glycolytic/glycogenesis metabolism as comapred to RA. The area under ROC curve (AUC) analysis revealed that four serum metabolites (valine, leucine, arginine and phenylalanine) exhibit high AUC values (>0.9) suggesting their potential as biomarkers. These pathway appear to be in good agreement with the disease pathophysiology and are avenues for further exploration. Conclusions: Metabolomes of ReA/uSpA are discrete from that of RA. Study of these metabolic alterations may provided new insights into the pathophysiology of ReA and RA.




  PB040 Spondyloarthropathy Top


Tenascin-C, a TLR4 endogenous ligand levels are increased in ankylosing spondylitis

Latika Gupta, Shruti Bhattacharya, Amita Aggarwal; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Objectives: Monocytes of patients with Ankylosing Spondylitis (AS) over-express Toll-like receptor (TLR) 4 on their monocytes. Tenascin-C (TNC) is an extracellular-matrix-glycoprotein and acts as an endogenous TLR4 ligand. Thus we studied the serum and synovial fluid levels of TNC in adults with AS.

Methods: TNC was measured in serum of 36 AS patients (ASAS 2009 criteria) and 10 healthy controls by ELISA. 22 patients were followed-up after 3 months of standard of care treatment. Five paired serum-synovial fluid samples were also analyzed. Disease activity was assessed by BASDAI, ASDAS, tender and swollen joint count; enthesitis score, ESR and CRP. All values are in median (IQR).

Results: Median age was 30 (20-35) years and disease duration 5.5 (1.3-10) years. 31 were male and 5 female. 25 cases had peripheral arthritis (69.5%). Median BASDAI was 5.3 (3.3-6.7). HLA B27 was positive in 34 (94.5%) cases. Median serum Tenascin C levels were higher in AS [554.6 ng/ml] as compared to healthy controls [32.88 ng/ml, p<0.00001]. Serum Tenascin C levels correlated with ASDAS ESR [r=0.367, p=0.028] and ESR [r=0.39, p=0.035]; but not with other disease activity measures. In patients with disease duration less than 5 years (early disease) serum levels had better correlation with ESR [r=0.59, p=0.009] and CRP [r=0.479, p=0.044]. On ROC analysis for active (PhGA≥6) vs. inactive (PhGA≤4) disease, Tenascin C (AUC=0.60) performed as well as CRP (AUC=0.65) and ESR (AUC=0.73). The synovial fluid levels [11.61 (5.99-176.9) ng/ml] were lower than in the serum [627.4 (488.5-779.1) ng/ml, p=0.008]. Tenascin C levels fell with treatment [630.8 ng/ml to 376.4 ng/ml p=0.0006] in treatment responders (n=11) but not in non- responders (n=11) [562.3 to 445.6, p=0.33].

Conclusion: Serum TNC levels are significantly raised in AS and can serve as marker of inflammation in early disease. Treatment reduces the TNC levels, probably due to control of disease activity.


  PB041 Spondyloarthropathy Top


Distinctive metabolic signatures of axial and peripheral ankylosing spondylitis revealed by 1H NMR based serum metabolomics

Latika Gupta, Dinesh Kumar1, Anupam Guleria1, Atul Rawat1, Amita Aggarwal*; Department of Clinical Immunology, SGPGIMS, 1Centre of Biomedical Research, Lucknow, Uttar Pradesh, India

Background: Ankylosing spondylitis (AS) is characterized by predominant involvement of the sacroiliac joints and the spine although half the patients have peripheral arthritis as well. The treatments useful in peripheral arthritis like SSZ and MTX have limited role in axial disease, this suggests that these two phenotypes may have different pathogenesis.

Objective: To explore potential of metabolite profiling in serum of AS patients as a biomarker for phenotype estimation, diagnosis and response to therapy.

Methods: Serum of 80 AS patients (ASAS 2010 criteria) and 86 healthy controls (HC) was subjected to metabolite profiling using H1NMR. Seventeen were followed-up after 3 months of standard treatment. Nine paired serum-synovial fluid samples were also analyzed. The serum metabolic profiles were obtained at 800 MHZ NMR spectrometer and compared using multivariate partial least-squares discriminant analysis [PLS-DA, Figure 1A]. The discriminatory metabolites were identified based on variable importance in projection (VIP) statistics [Figure 1B] and further evaluated for statistical significance [p-value <0.05, Figure 1C].



Results: Median age was 31 years and disease duration 6 years. Seventy were male and 6 had non radiographic AS. Apart from differences in AS vs. HC, PLS-DA plots revealed distinctive metabolomics signatures between axial and peripheral disease [Figure 1A]. Sera of peripheral AS patients were characterized by increased serum levels of N-acetyl-glycoproteins, lactate, glutamate, proline, arginine, phenylalanine and branched chain amino acids (i.e. leucine, isoleucine), whereas the serum levels of lipid/membrane metabolites including low/very-low density lipoproteins (LDL/VLDL), PUFA, choline and Glycerophosphocholine and several other amino acids (including alanine, glutamine and histidine) were found to be decreased significantly compared with axial AS patients. In treatment responders, metabolite profile changed significantly post therapy. Metabolite signatures are similar between HLA B27 positive and negative disease. There was no correlation with level of disease activity.

Conclusion: These metabolic alterations suggested that the peripheral AS patients exhibit activated glutaminolysis, glycolysis and membrane metabolism, irrespective of disease activity, suggesting different pathogenesis of axial and peripheral disease subsets. These markers may have the potential to make serum metabolic profiling as a tool for diagnosis, prediction of peripheral disease, and treatment response.


  PB042 Spondyloarthropathy Top


NMR based serum and synovial fluid metabolomics reveal similar metabolomic profile in patients with reactive arthritis and undifferentiated spondyloarthropathy

Durgesh Dubey, Sandeep Kumar1, Sakir Ahmed1, Abhra Chaudhary, Smriti Chaurasia1, Anupam Guleria1, Dinesh Kumar, Ramnath Misra1; Centre of Biomedical Research, SGPGIMS, 1Department of Clinical Immunology, SGPGIMS, Lucknow, Uttar Pradesh, India

Background: Reactive arthritis (ReA) and undifferentiated spondyloarthropathy (uSpA) are seronegative spondyloarthropathies with similar clinical presentations. A clear history of urogenital or gastrointestinal infections defines ReA. In absence of such history, it is classified as uSpA. Both have strong HLA class I associations, similar cytokine profiles and similar T-cell subset distributions.

Objective: To investigate if the metabolic profiles of ReA in sera and synovial fluid are different from those of USpA.

Methods: Metabolic profiles in sera and synovial fluid samples were measured using 1D1H NMR spectroscopy and were further quantified using CHENOMX © software. Metabolic profiles of ReA and USpA patients were compared using Partial Least Square-Discriminatory Analysis(PLS-DA) and Q2 parameter was used to assess the group separation (Q2<0 implies a model that performs worse than chance).

Results: The study involved 19 ReA and 13 uSpA patients with median age 26 (21-33.75) years. Of 32 patients: 7(22%) were females, three (12%) had monophasic illness, 16 (64%) polyphasic, and six (24%) persistent arthritis (follow-up data was not available for 7). 27% had monoarticular, 59% oligoarticular and 14% polyarticular involvement. Six had inflammatory backpain, three had oral ulcers, one dactylitis, but none had mucocutaneous manifestations. Two had asymptomatic sacroiliitis on radiographs. Compared to normal controls (n=18, median age=29 years, male:female=17:1), the sera of ReA/uSpA patients were characterized by elevated levels of malonate, mannose, N-nitrosodimethylamine, and pyruvate, whereas 3-hydroxyisovalerate was decreased (T-test p-value <0.001). PLS-DA of sera versus synovial fluid showed demarcation with higher pyruvate and acetoacetate and lower aspartate, methanol, ethanol, and methylsuccinate in synovial fuild compared to sera (t-test p<0.01). PLS-DA of sera of ReA versus uSpA showed little difference [Figure 1] while that of synovial fluid had Q2<0 for all models. Similarly, both sera and synovial fluid showed poor discrimination (Q2<0 in all models) between HLA-B27 positive and negative groups [Figure 1].

Conclusion: ReA and uSpA have indistinguishable metabolomics profiles in sera and synovial fluid possibly reflecting similar metabolo-inflammatory pathways involved.


  PB043 Spondyloarthropathy Top


Dysregulated cytokine and chemokine profiles in synovial fluid of patient with ReA/uSpA

Sandeep Kumar, Ratandeep Mukherjee, Smriti Chaurasia, Sakir Ahmed, Rajeev Singh, Amita Aggarwal, B Ravindran, Ramnath Misra; Department of Clinical Immunology, SGPGIMS, Lucknow, Uttar Pradesh, 1Institute of Life Sciences, Bhubaneswar, Odisha, India

Objectives: The role of cytokines, chemokine and growth factors in synovitis remain unclear in patients with ReA/uSpA. Here we have assessed a panel of T cell, macrophage, and stromal cell related cytokines, chemokines and growth factors in the serum and synovial fluid of patients with ReA/uSpA.

Methods: Paired serum and synovial fluid (SF) were collected from the 38 patients with ReA/uSpA. Patients with ReA were defined by presence of acute onset of oligoarthritis following diarrhea within 4 weeks and patients who fulfilled ESSG were defined as patients with uSpA. Multiplex cytokine/chemokine bead assay was performed to analyse 27 cytokine/chemokine/growth factors in serum and synovial fluid samples by using Milliplex MAP Human Cytokine/ Chemokine Panel 1 Pre-mixed 27 Plex kit.

Results: The macrophage, T cell and endothelial cell related cytokines and chemokines were differentially expressed in patient than HC. Chemokines IL-8, MCP-1, MIP-1α, MIP-1β, VEGF were significantly higher in patient serum than HC whereas IP-10, IL-10, IL12p70 and TNF were higher in HC serum. In patient's SF cytokines; IL-6, IL-1Rα, IL-15, IL-17, IL-10 and IFN-γ, growth factors; G-CSF, chemokines; IL-8, IP-10, MCP-1, and MIP-1β were highly expressed than serum. Angiogenic and stromal cell factors; VEGF and eotaxin were higher in serum than SF. Further, in HLA-B27 positive group IL-6 was higher in serum and IL-8, MCP-1 and MIP-1α were higher in SF as compared to HLA-B27 negative patients.

Conclusion: The deregulated cytokine, growth factors and chemokines may involve in promoting pro-inflammatory environment in synovium.


  PB044 Spondyloarthropathy Top


ROS generation and NOS gene expression in patients with Ankylosing spondylitis: A case, control study

Ayindrila Saha, Sanchaita Misra, Debanjali Dasgupta, Sumantro Mondal, Alakendu Ghosh; School of Digestive and Liver Disease, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, India

Background: Ankylosing spondylitis (AS), a form of spondyloarthropathy, causes arthritis of the spine, sacroiliac joints and even the peripheral joints. Cardiovascular complications in AS include impaired endothelial function due to systemic inflammation, associated with decrease in both synthesis and nitric oxide (NO) bioavailability, increased oxidative stress. This NO, biochemically is regulated at different levels: 1) eNOS gene expression, 2) eNOS enzymatic activity and 3) degradation of NO. Previously in our study, endothelial dysfunction had been established in AS patient group through significant decrease in ED-FMD compared to controls.

Objectives: The aim of this study is to measure oxidative stress marker and expression of nitric oxide synthase (eNOS and iNOS) genes by real time PCR and eNOS, iNOS proteins by western blot in AS patients with endothelial dysfunction.

Methods: 30 AS patients fulfilling modified New York criteria (mean age 29 ± 6.204, 26 male and 4 females) and 27 healthy controls (mean age 28.3 ± 4.087, 21 male and 6 females) have been recruited for the study. BASDAI are evaluated. The serum level of nitric oxide is determined by colorimetric assay. Reactive oxygen species (ROS) is measured by flow cytometry. eNOS and iNOS gene expression is estimated by real time PCR. eNOS, iNOS protein levels are detected by Western blot analysis.

Results: Nitric oxide concentration is lower in AS patients than control group (p-value = 0.02). Baseline ROS level is much increased in patients as compared to controls (p-value = 0.0039). There is an increasing trend in expression of iNOS gene in patient group compared to control. But the difference is not significant may be due to the small sample number.

Conclusion: Elevated oxidative stress and alteration in gene expression is observed in this study. Chronic inflammatory burden may play a role behind elevated oxidative stress, manifested in endothelial dysfunction. The superoxide formed may react with nitric oxide to form peroxynitrite which requires further study.


  PB045 Spondyloarthropathy Top


Evaluation of endothelial dysfunction by flow mediated vasodilatation of brachial artery and intima-media thickness of common carotid artery in axial spondyloarthritis

Shubhabrata Das, Rathindranath Sarkar1, Urmimala Bhattacharjee1, Ritasman Baisya1, Kaushik Basu1; University of Alberta, Edmonton, Canada, 1Medical College, Kolkata, West Bengal, India

Background: Axial Spondyloarthritis including Ankylosing Spondylitis patients have an increased risk of premature atherosclerosis and cardiovascular disease. Intima-media thickness (CIMT) of common carotid artery is a popular marker of subclinical atherosclerosis. Here we have studied if there is endothelial dysfunction and subclinical atherosclerosis in early spondyloarthritis (<5years disease duration).

Objective: To evaluate endothelial dysfunction by ultrasonographic flow mediated vasodilation (FMD) of brachial artery and intima media thickness of common carotid artery (CIMT) in patients of early spondyloarthritis.

Methods: 50 patients without conventional cardiovascular risk factors meeting ASAS 2009 criteria for axial spondylosrthritis were included as study group and 50 age and sex matched controls were included as control group. Ultrasonography guided flow mediated vasodilation of brachial artery and intima media thickness of common carotid artery were evaluated in both these groups and compared. Disease activity and fasting lipid profile were also studied and compared with ultrasonographic parameters in study group.

Results: Flow mediated vasodilation (FMD%) was significantly impaired in study group compared to control group [4.94±1.35% vs 8.71±1.58% (p<0.0001)]. Similarly CIMT was significantly deranged in the study population [0.52±0.04mm VS 0.44±0.11mm (p<0.0001)]. 4 out of the 50 patients had carotid plaque (8%) whereas none of the individuals in the control group had similar finding. Significant correlation was found between FMD% and disease duration, BASDAI, ESR, CRP, CIMT, LDL cholesterol, HDL cholesterol.

Conclusion: Axial spondyloarthritis has increased risk of atherosclerosis evident by impaired FMD% and CIMT. Disease activity (ESR, CRP, BASDAI) in the study group is increased compared to control group and these parameters of disease activity correlating with novel ultrasonographic risk parameters (FMD %, CIMT) of atherosclerosis.


  PB046 Spondyloarthropathy Top


HLA-B*27Allele subtype prevalence and disease association of ankylosing spondylitis among South Indian Population

Vikram Haridas1,2, Praveenkumar Shetty*3, M Nirmal Kumar4, K C Vasanthakumar1, Kiran Haridas1, Vitthal Khode5, Anil Bargale3; 1Arthritis Super Speciality Center, Hubli, Karnataka, Departments of 2Medicine and 5Physiology, SDM College of Medical Sciences and Hospital, 3Central Research Laboratory, Department of Biochemistry, SDM College of Medical Sciences and Hospital, Dharwad, Karnataka, 4Jeevan Stem Cell Foundation, Chennai, Tamil Nadu, India

Objectives: Ankylosing Spondylitis (AS) is a chronic inflammatory arthritis mainly affecting articular and extraarticular structures. AS is characterized by other clinical manifestations of axial arthritis involving sacroiliac joints and spine. Genetic factors play a key role in AS susceptibility. AS-associated subtypes of HLA-B27 and other HLA-B alleles vary in different ethnic populations. There are no reports of HLA B genotype association to South Indian AS patients. In the current study we have analysed the HLA-genotype association with 105 AS patients and 100 respective controls, we have also verified whether any specific clinical manifestation of AS has any pattern of HLA-B subtype association.

Methods: The patients with AS were diagnosed following ASAS criteria. Prior to enrolling the patients, the written informed consent was collected, genomic DNA was extracted from peripheral blood cells. The genotyping of HLA-B27 was performed in Applied Biotechnologies 3130/ 3500 sequencer using SeCore HLA B class I typing high resolution kit from Invitrogen.

Results: HLA B27 allele frequency in AS patients (74%) is significantly higher than healthy controls (3%). Most of the earlier studies associated AS with HLA B27 antigen. Current data illustrates that only 21% of AS patients presented HLA B27 antigen rather having 74% HLA B27 allele. HLA B27:05 and HLA B27:04 are the predominant subtypes.

Conclusion: Early onset of AS manifestations is seen in HLA B27 phenotypes than non HLA B27 phenotypes. HLA B27 associated AS patients presented more severe axial manifestations like bilateral sacroiliitis and erosion than non HLA types.


  PB047 Spondyloarthropathy Top


Increased Serum levels of Nαacetyllysine in Patients with Ankylosing spondylitis revealed by 1H NMR based serum metabolomics

Dinesh Kumar, Latika Gupta1, Anupam Guleria, Atul Rawat, Amita Aggarwal1*; Centre of Biomedical Research, SGPGIMS, 1Department of Clinical Immunology, SGPGIMS, Lucknow, Uttar Pradesh, India

Background: The introduction of tumor necrosis factor (TNF-a) blocking therapy has revolutionized the management of ankylosing spondylitis (AS). A recent study has revealed the hyperacetylation status in AS patients (1) and has been related to elevated circulatory levels of histone-deacetylase enzymes (HDAC) in these patients (2). The disturbed balance of acetylation/deacetylation can be assessed through profiling Nαacetyllysine (NAAL) level in the sera of AS patients.

Objective: To compare the serum NAAL level of AS patients with normal controls. Further, to evaluate if the serum NAAL levels differ (a) in axial and peripheral AS patients and (b) in AS patients with positive and negative HLA B27.

Methods: The serum metabolic profiles of eighty AS patients (median age=31years, sex(M/F) ratio=71:9, axial/peripheral ratio=37:43 and HLA-B27 (positive/negative) ratio=67:13) meeting the modified New York criteria for AS (3) were measured using one dimensional 1H 1D CPMG-NMR spectra recorded at 800 MHz NMR spectrometer and compared to 86 normal control (NC) samples (median age=32 years, sex(M/F) ratio=72:14). The NMR signals of Na-acetyllysine were identified as broad signals at 1.38 ppm and 2.98 ppm using software CHENMOX and quantified using software AMIX (from Bruker Biospin, Germany). The quantitative levels of NAAL were compared using student t-test, whereas diagnostic sensitivity and specificity of the test was evaluated based on the area under the Receiver operating characteristic (AUROC).

Results: Compared to NC, the NAAL level was significantly higher (with p-value<0.05 and AUROC=0.884) in the sera of AS patients and it might be related to disturbed balance of acetylation/deacetylation of histone proteins involved in transcriptional/epigenetic regulation of TNF-A. [Figures 1]A and [Figures 1]B. However, there was not any significant difference in the serum levels of NAAL between (a) axial and peripheral AS patients [Figure 1C] and (b) AS patients with positive and negative HLA B27 factor [Figure 1D].



Conclusion: The NAAL level were significantly increased in the AS patients suggesting that it might serve as a new marker for early prediction of AS, treatment response and disease progression.

References

  1. Toussirot E, Abbas W, Khan KA, Tissot M, Jeudy A, Baud L, et al. Imbalance between HAT and HDAC activities in the PBMCs of patients with ankylosing spondylitis or rheumatoid arthritis and influence of HDAC inhibitors on TNF alpha production. PLoS One 2013;8:e70939.
  2. Jiang Y, Wang L. Role of histone deacetylase 3 in ankylosing spondylitis via negative feedback loop with microRNA-130a and enhancement of tumor necrosis factor-1α expression in peripheral blood mononuclear cells. Mol Med Rep 2016;13:35-40.
  3. Rudwaleit M, van der Heijde D, Landew ´e R, Listing J, Akkoc N, Brandt J, et al. The development of Assessment of SpondyloArthritis international Society classification criteria for axial spondyloarthritis (part II): validation and final selection. Ann Rheum Dis 2009;68:777–83.



  PB048 Spondyloarthropathy Top


A study of peripheral spondyloarthritis and its association with different HLA B allel

Jeet Patel, Ved Chaturvedi, Lalit Duggal, Neeraj Jain, Monika Jain, Bhandari; Sir Ganga Ram Hospital, New Delhi, India

Background: Spondyloarthritis (SpA) is a heterogeneous group of disorders with less isolated peripheral joints involvement. Study of different HLA B alleles in Indian SpA is helpful as positivity of HLA B27 is low in this group.

Objective: To find any specific HLA B allele which might be helpful in diagnosis of isolated peripheral Spondyloarthritis patients where HLA B27 might not be helpful and to study their association with patient characteristics.

Methods: All patients were recruited at tertiary care centre in India (N-20). ASAS classification criteria for peripheral SpA, classification criteria for reactive arthritis (Braun J et al) and CASPAR criteria for psoriatic arthritis were used to classify patient into subgroups. Colonoscopy/biopsy proven IBD patients with joint involvement were classified into IBD related arthritis. In all above subgroups, axial involvement was taken as exclusion criteria. Social and demographic data, disease characteristics, laboratory investigations, radiological investigations and disease activity indices (ASDAS ESR/CRP, DAPSA) were recorded. HLA B genotyping was done using SSP tray kit.

Result: Out of 20, 60% were male and 40% female. Average age was 42.0. Average duration of illness, age of onset and age of diagnosis were 1.94, 40.15, 41.05 years with average delay in diagnosis of 1.10 year. Most common joint involvement was ankle (80%), followed by knee (75%), wrist/small joints of hands (45%) and elbows (35%). Enthesitis and Dactylitis were present in 20% and 15% respectively. GIT symptoms were present in 40% while 2 patients were of IBD. 20% had eye symptoms while only 1 had proven uveitis. Average CRP and ESR were 82.35 and 46.55 respectively. Most common peripheral SpA was Reactive arthritis (80%), followed by SpA (30%), psoriatic arthritis (15%) and IBD related peripheral arthritis (10%). HLA B27 was positive in only 30% of patients. Other positive HLA B alleles were HLA B 40 (30%), HLA B52 (25%), HLA B44 (20%) and HLA B51 (15%). Other positive alleles (<10%) were HLA B8/13/15/35/37/38/41/58/50. Glucocorticoid was used in 60% and NSAIDS use was in 80% of patients. Sulfasalazine, methotrexate and infliximab were used in 40%, 15%, and 20% respectively [Table 1].



Conclusion: Peripheral SpAs are different subgroup with prominent ankle/knee joint involvement, significant use of steroids and low.


  PB049 Spondyloarthropathy Top


A comparison of clinical and laboratory profile of non-radiographic axial spondyloarthritis and Ankylosing spondylitis

Uday Prabhakar, Saumyaleen Roy, Deepa Kumari, Anup Singh, Manaswi Chaubey; Department of Medicine, IMS, BHU, Varanasi, India

Background: Ever since the publication of Assessment of SpondyloArthritis international Society (ASAS) criteria for Axial spondyloarthritis in 2009, there has been much debate whether nrAxSpa (non radiographic axial SpA) can be taken as an early marker for Ankylosing Spondylitis (AS) or a different disease entity presenting with symptoms similar to AS but without radiographic manifestations. Only a handful studies from India have aimed to delineate the differences between the two groups.

Objective: To investigate the differences in clinical presentation and disease activity in nrAxSpa and AS using the 2009 ASAS criteria.

Methods: The study was conducted in Division of Rheumatology, S.S.Hospital, BHU between June 2016 and June 2017.A total of 114 patients were included in the study, with the entry criteria being chronic inflammatory low back pain for ≥3 months and age of onset <45 years. Patients were then classified into AS if they had radiographic sacroiliitis; and as nrAxSpa if there was presence of either sacroiliitis on MRI and ≥1 Spa features OR HLAB27+ and ≥2 SpA features. Patients with past/present Biological therapy were excluded from the study.Both the groups were compared for various variables.

Results: 50 out of 114 patients had AS, 50 had nrAxSpa; 12 had lumbar spondylosis and 2 had undifferentiated SpA. On comparing the two groups, patients with AS had a longer disease duration and an elevated baseline CRP and ESR. No statistically significant difference was found between other spA variables including current age, Age at onset, HLAB27, family history, enthesitis, uveitis, dactylitis, psoriasis, peripheral arthritis and BASDAI (Bath Ankylosing Spondylitis Disease activity index).

Conclusion: Barring an elevated levels of markers of inflammation (CRP/ESR) and a longer disease duration in AS, most other features do not vary significantly between the two groups. Thus, the two groups may represent same disease in different phases. The limitation of this study was a lack of follow up to monitor the progression of radiographic disease in nrAxSpa group.


  PB050 Lupus APS Sjogren's Syndrome Top


Metagenomics of the saliva microbiome in primary Sjogren's syndrome reveals a distinct set of microbes associated with the disease

Disha Sharma1,4,$, Pulukool Sandhya2,$,*, Shamsudheen Karuthedath Vellarikkal3,4, Ajit Kumar Surin2, Rijith Jayarajan3, Ankit Verma3, Anoop Kumar3, Rowmika Ravi3, Debashish Danda2, Sridhar Sivasubbu3,4*, Vinod Scaria1,4; 1GN Ramachandran Knowledge Center for Genome Informatics, CSIR Institute of Genomics and Integrative Biology, 3CSIR Institute of Genomics and Integrative Biology, 4Academy of Scientific and Innovative Research, CSIR-IGIB South Campus, Delhi, 2Department of Clinical Immunology and Rheumatology, Christian Medical College Hospital, Vellore, Tamil Nadu, India

Objective: Primary Sjogren's syndrome (pSS) is an autoimmune disease characterized by sicca symptoms resulting from salivary and lacrimal gland dysfunction. Salivary gland dysfunction could alter the salivary microenvironment resulting in dysbiosis. We attempted to understand the salivary microbiome in patients with pSS using a metagenome sequencing approach.

Methods: Saliva of adult patients suffering from pSS and matched healthy controls was collected in sterile tubes with DNA preserving buffers. All pSS patients fulfilled the 2016 American College of Rheumatology or the European League Against Rheumatism classification criteria. Those with caries,periodontitis,gingivitis, oral ulceration, oral candidiasis,diabetes mellitus, inflammatory bowel disease, liver, renal or peptic ulcer disease, use of antibiotics or dental procedure in preceding 3 months, use of paan, alcohol, tobacco, chewing gums, carbonated drinks were excluded. 16S rRNA sequencing was performed as per standard protocols using barcoded primers. Analysis was done independently using two computational pipelines QIIME and LotuS. Operational Taxonomic Units (OTUs) were assigned. A consensus of the two methods was taken.

Results: We performed 16s rRNA sequencing for saliva from 39 pSS and 35 control individuals. The data was demultiplexed and independently analysed on the two pipelines and organisms which were significantly differential between the two sets were considered. Actinomycetaceae, Bifidobacterium, Lactobacillus and Veillonella were consistently elevated in pSS while Leptotrichia was found to be consistently and significantly reduced in pSS (p value <0.05 and fold changes >2).

Conclusion: Our analysis revealed a distinct set of organisms was differential in cases in comparison with controls.


  PB051 Lupus APS Sjogren's Syndrome Top


Urinary VCAM1 as a disease activity indicator in lupus nephritis

Shivraj Padiyar, John Mathew, Debashish Danda, Samuel Hansdak1, Vijayakumar2; Departments of Rheumatology, 1Medicine and 2Nephrology, Christian Medical College, Vellore, Tamil Nadu, India

Objectives: To study the utility of urinary levels of VCAM-1 in lupus nephritis.

Methodology: It was a diagnostic case control study. The patients presenting to Medicine, Nephrology and Rheumatology OPD were recruited into this study. Patients were divided into 2 groups, SLE without active nephritis and SLE with active nephritis based on the renal SLEDAI. Urinary VCAM1 was tested in all patients using an early morning spot urine sample. Renal biopsy was done in those patients with active nephritis. VCAM1 levels were compared with the renal SLEDAI, renal biopsy disease activity and standard of care markers. The results were analyzed using SPSS software version 16. A 2x2 analysis for the diagnostic test was done. The validity and predictive value statistics was presented with 95 percent confidence interval. As VCAM1 provides levels, the best cut off was identified using ROC analysis. The risk factor analysis for the nutritional intake was done using multivariate regression analysis.

Results: Out of 83 patients, 74 patients were taken up for final analysis. Urinary VCAM 1 levels had significant correlation (p=0.01) with disease activity based on renal SLEDAI [Figure 1]. However, the correlation between the biopsy findings and VCAM levels was not statistically significant [Figure 2] The sensitivity and specificity of urinary VCAM 1 is 65.22% and 75% respectively. The cut off value of urinary VCAM 1 is 23.8 pg/mg of creatinine.




  PB052 Lupus APS Sjogren's Syndrome Top


Salivary beta 2 microglobulin as a diagnostic marker in Sjogren's syndrome

Kavya Devi Nunna, Phani Kumar Devarasetti, Rajendra Vara Prasad Irlapati, Liza Rajasekhar; Department of Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background: Beta 2 microglobulin (β2 MG) is a part of HLA (human leucocyte antigen) and is particularly enriched on lymphomononuclear cells. Sjogren's syndrome (SS) is characterized by infiltration of these cells in salivary and lacrimal glands resulting in xerosis. Elevated β2MG in saliva thus reflect local lymphomonocytic infiltration and helps in classifying SS.

Objectives: To evaluate the sensitivity and specificity of salivary β2MG in SS. To test the association of β2MG with each parameter of 2012 SICCA criteria.

Methods: Patients satisfying 2012 SICCA criteria1 were included in this cross-sectional prospective study. All participants underwent Labial Salivary Gland (LSG) biopsy, ocular staining score (OSS) and autoantibody assays [anti-Ro, anti-La, and IgM RF by ELISA and ANA by indirect immunofluorescence (IIF)]. β2MG was estimated using ELISA in unstimulated whole saliva. Disease activity was assessed by EULAR Sjogren's syndrome Disease Activity Index (ESSDAI). Receiver operator curve was constructed to estimate the diagnostic utility of β2MG using 2012 SICCA criteria as gold standard. Pearson correlation was done to correlate β2MG with LSG focus score (FS), OSS, autoantibodies and ESSDAI.

Results: Fifty nine SS patients and 20 age and sex matched healthy controls were included. Mean (SD) age at disease onset was 34.8 (10.2) years. Fifty six were females. The common clinical manifestations were polyarthralgias/polyarthritis (80%) followed by oral and ocular sicca (74%). Prevalence of autoantibodies in our cohort was ANA 71%, anti-Ro/La 59% and IgM RF 73 %. OSS ≥ 3 and schirmer's test was positive in 79% and 30% respectively. LSG biopsy with FS ≥1 was seen in 92% of cases. Salivary β2MG was significantly higher in cases (9936.74 ng/ml) compared to healthy controls (4748.59 ng/ml) [p<0.05]. At a cut off of 5375 ng/ml, salivary β2MG has a 72% sensitivity and 70% specificity. There was no correlation between salivary β2MG and FS, OSS, autoantibodies and ESSDAI.

Conclusion: Salivary β2MG as a sole diagnostic biomarker has a low sensitivity and specificity compared to 2012 ACR criteria. OSS ≥3 is more sensitive than schirmer's for ocular sicca.

References

  1. Shiboski SC, Shiboski CH, Criswell L, Baer A, Challacombe S, Lanfranchi H, et al. American College of Rheumatology classification criteria for Sjögren's syndrome: A data-driven, expert consensus approach in the Sjögren's International Collaborative Clinical Alliance Cohort. Arthritis Care Res (Hoboken) 2012;64:475-87.



  PB053 Lupus APS Sjogren's Syndrome Top


IL17/IFNγ double positive TH17 cells leading to glucocorticoid resistance in lupus nephritis

Akhilesh Jaiswal, Mohit Rai, Naraya Prasad, Vikas Agarwal, Mantabya Singh; SGPGIMS, Lucknow, Uttar Pradesh, India

Background: Th17 cells and cytokine IL-17 are involved in many autoimmune diseases. Recently; IL-17/IFN-g double-positive Th17 cell were found to be allied with inflammatory diseases. P-glycoprotein (P-gp) +ve Th17 cells are refractory to steroid. P-gp on lymphocyte effluxes out steroid and prevents its action. We conducted this study with hypothesis that P-gp positive IL-17/IFN-g double+ve Th17 are one of key pharmacokinetic and pharmacodynamic modulator liable for steroid resistance in lupus nephritis (LN). We planned to study the frequency of P-gp expressing pathogenic Th17 cells in steroid responsive and non-responsive steroid patients.

Methods: We analysed the frequency of pathogenic IL-17/IFN-g double-positive Th17 lymphocytes and P-gp expression on their surface by flowcytometry in responsive (n=52; mean age 34.06±10.84) and non-responsive (n=25; mean age 37.29±13.73) patients. We also included 10 age and sex matched healthy controls. All patients were biopsy proven LN.

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

Conclusions: Higher frequency of IL-17/IFN-g double-positive Th17 cell with P-gp expression may be associated immunological and pharmacological factor for steroid resistance in LN.


  PB054 Lupus APS Sjogren's Syndrome Top


Grading of intensity of fluorescence in anti-nuclear antibody test: Does the intensity reflects antibody concentration? Six years of observation from a single tertiary care centre

Therese Mary Dhason, Sowndhariya Annamalai, Euphrasia latha Jairaj, Saranya Chinnadurai, Vignesh Manthram, Kumudha Manoharan, Radhabai Chakrapani, Easwari Raju, Sivasankari Kuppusamy, Santhi Thankaraj; Institute of Rheumatology, Madras Medical College, Chennai

Background: The gold standard method to detect anti-nuclear antibodies is Indirect Immunofluorescence. Interpretation of the test results are based on the titre of serum, intensity of fluorescence and the pattern of fluorescence.

Objective: To find out whether any relationship exists between the intensity of fluorescence and the anti-nuclear antibody concentration. In this study, the samples showing homogeneous pattern were tested for the anti-dsDNA antibody concentration by ELISA test.



Methods: A prospective observational study between June 2016 to June 2017 at a tertiary care centre. The study included a total of 500 patients with systemic lupus erythematosus and 100 healthy controls. ANA was done by indirect immunofluorescence and anti dsDNA by ELISA.

Results: 221 patients had positivity for anti dsDNA out of which 200 had homogenous pattern (p<0.00001).

Conclusion: The mean antibody concentration of the various grades of intensity of fluorescence was almost similar. As the intensity does not reflect the antibody concentration, further studies are needed to elucidate on the significance of grading of fluorescence intensity.


  PB055 Lupus APS Sjogren's Syndrome Top


A study of haamatological manifestations of systemic lupus erythematosus with special reference to disease activity

T Karthikeyan, P Dihingia, S M Baruah, T K Das, C Dutta; Assam Medical College, Dibrugarh

Background: Our understanding of Systemic Lupus Erythematosus (SLE) has been evolved very significantly over the period of last few decades. The hematological abnormalities though more commonly seen are not properly evaluated or estimated and are not given enough representation. In some of these cases presenting with Anemia, Thrombocytopenia, Pancytopenia or Thrombotic episodes, especially so in young females, the diagnosis may be delayed or initially missed if the index of suspicion is low or if there is improper and inadequate follow up. Thus keeping this in mind this study as be carried out.

Objectives: To study Haematological manifestations of SLE. To study its correlation with the disease severity.

Methods: This study is an Observational Cross Sectional Study done in Assam Medical College for a period of one year. All cases who fulfilled the ACR criteria 1997 who came to Rheumatology opd and those who admitted in Medicine ward taken up for the study.

Results: Totally 106 patients were taken up for the study. The Male Female ratio in this study was 1:11. The most common constitutional symptom was Fatigue which present among 57 (53.7%). Raynaud 's phenomenon was present among 15 (14.15%) patients. Anemia was present in 99 patients (93%) of the study group with most common type being Anemia of chronic disease. Neutropenia was present among 8 (7.5%), Thrombocytopenia among 8 (7.55%) in the study group. Severity of anemia and Serum Ferritin Level were in correlation with the Disease severity (SLEDAI).

Conclusion: Haematological Manifestations is one of the most common manifestation presents very early in the disease process and in many cases as the only presentation. This Study demonstrated similarities in most of the investigations, reveling at the same time much Higher incidence of Haematological manifestations.


  PB056 Lupus APS Sjogren's Syndrome Top


Endothelial nitric oxide synthase gene 27bp VNTR polymorphism is associated with SLE but does not influence plasma nitric oxide levels

Shiva Krishna Katkam, Liza Rajasekhar1, Vijay Kumar Kutala; Departments of Clinical Pharmacology and Therapeutics and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background: The increased urinary nitrite/nitrate levels in MRL-lpr/lprmice prone to systemic autoimmunity is reduced by oral administration of NG-monomethyl-L-arginine (NO synthesis blocker), and this prevents the development of glomerulonephritis and reduces the intensity of inflammatory arthritis suggesting a role for nitric oxide in the pathogenesis of lupus. Endothelial nitric oxide synthase (eNOS) is constitutively expressed by glomerular endothelium. eNOS-derived NO is critical in limiting inflammatory reactions. eNOS gene expression is posttranscriptionally regulated by 27bp VNTR repeat region in intron 4 (intron4VNTR) of eNOSby formation of small interference RNA (sirRNA) spliced during eNOS pre-mRNA processing. This intron4 VNTR polymorphism influences plasma concentrations of nitric oxide by regulating the mRNA levels. In vitro studies have revealed that the variant allele 4b carries five copies and produce higher quantities of sirRNA, thereby leading to lower levels of NOS3 mRNA compared to cells containing four copies.

Methods: Patients satisfying the ACR 1982 SLE classification criteria were included in the study. Genotype of intron4 VNTR was determined by using standard PCR primers 5'-AGG CCC TAT GGT AGT GCC TTT-3 (forward) and 5-TCT CTT AGT GCT GTG GTC AC-3 (reverse). Plasma nitrate levels were estimated with simple spectrophotometric acidic Griess reaction method.

Results: Total of 478 subjects (218 SLE cases and 260 controls) were included. The genotype and allele frequencies of eNOS intron4 27bp VNTR polymorphism differed significantly between SLE patients and controls (genotype, OR: 1.8, CI %95:1.22-2.67, P=0.0038, allele OR: 1.57, CI %95:1.11-2.20, P=0.0094 respectively). Genotype and allele frequency distribution in patients with and without lupus nephritis did not reveal any significant difference. Plasma nitrite levels were significantly higher in cases (n=70, 38.5±22.85 μM/L) compared to healthy controls (n=82, 29.11±17.29 μM/L) (P=0.004). Plasma NO levels were similar across different eNOS intron4 27bp VNTR genotypes (bb vs ab+aa) in SLE cases [Figure 1].



Conclusion: Our results provide evidence for association between the 27bp VNTR of eNOS gene and the risk of SLE. Elevated plasma nitrate levels in SLE are not influenced by this polymorphism. Altered expression of iNOS may contribute to elevated plasma NO in SLE.


  PB057 Vasculitis Top


High expression of pro-inflammatory cytokine genes, CCL2 and IL-1Î2 as well as decreased expression of genes involved in angiogenesis in PBMCs of patients with Takayasu arteritis upon TLR4 activation

Jayakanthan Kabeerdoss, Ruchika Goe, M Hindhumathi, Debashish Danda; Department of Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Introduction: Toll-like receptors (TLR) 1 to 4 are highly expressed in aorta. Activation of TLR4 causes transmural arteritis mimicking Takayasu arteritis (TA) in Human temporal artery-SCID chimera model. In this study, we examined the expression of genes involved in angiogenesis and cytokine genes in peripheral blood mononuclear cells (PBMCs) of patients with Takayasu arteritis (TA) following stimulation with lipopolysaccharide (LPS), a TLR4 ligand.

Methods: Thirteen patients with TA and 14 healthy controls were recruited. In the screening cohort, PBMCs of patients with TA (n=6) and healthy controls (n=6) were stimulated with 100 ng/ml of lipopolysaccharide (LPS), and cultured for 4 hours in RPMI medium and incubated at 37ï‚°C with CO2. After incubation, mRNA expression of select angiogenesis genes in PBMCs was quantitated by RT2 ProfilerTM PCR Array Human Angiogenesis (PAHS-024ZC-12; Qiagen India) kit in step-one plus real time PCR system. mRNA expression of genes found to be significant in PCR array were validated in PBMCs of additional TA patients (n=7) and healthy controls (n=7) after stimulating with 100 ng/ml of LPS. Quantification during validation was performed by real time PCR using specific primers and SYBR green chemistry.

Results: Among TA, 9 were females [median age 25.5 (18-58) years], with 7 being treatment naïve. PCR array results showed increased expression of proinflammatory molecules IL-1ß, CCL2, inhibitors of angiogenesis namely thrombospondin and TIMP1 in TA. However, there was down regulation of genes involved in angiogenesis, namely ANGPLT4, ANGPT2, VEGFB, TIE1 and NOS3 in TA as compared to healthy controls upon TLR4 activation. Expression of most significant genes i.e. IL1b, CCL2, and VEGFB was quantified in validation cohort, which confirmed the findings of PCR array. The expression of IL-1ß, and CCL2 were increased, while VEGFB was decreased in TA patients in validation cohort.

Conclusion: Activation of TLR4 leads to increased expression of pro-inflammatory cytokine genes and decreased expression of pro-angiogenic genes in PBMCs of TA.


  PB058 Vasculitis Top


Distinctive metabolic signatures of systemic lupus erythematosus and Takayasu arteritis revealed by 1H NMR based serum metabolomics

A Jain1, D Kumar1, U Kumar1, S Kumar1, D P Misra1, D Dubey1, R Kumari1, A Guleria1, R Misra1, R Goel1, D Danda1, P A Bacon2; Centre of Biomedical Research, Department of Clinical Immunology, SGPGIMS, Lucknow, Uttar Pradesh, 1Christian Medical College, Vellore, Tami Nadu, 2Department of Rheumatology, University of Birmingham, Birmingham, UK

Background: Pathogenesis of SLE and TA, autoimmune diseases predominantly affecting young female, is still not completely understood. Recently, we have shown that the serum metabolic profiles in both SLE and TA are distinctively different compared to age and sex matched normal controls. Serum metabolomics may give us some insight into the mechanism underlying pathophysiology of these inflammatory diseases.

Objective: We sought to identify if the serum metabolic perturbations associated with SLE (non-renal) patients are specific and different compared to TA patients.

Methods: The serum samples were collected from 32 SLE patients satisfying ACR 1987 classification criteria and 46 TA patients fulfilling ACR criteria. The serum metabolic profiles of SLE and TA patients were obtained at 800 MHZ NMR spectrometer and compared using multivariate partial least-squares discriminant analysis (PLS-DA). The metabolites responsible for the separation of groups in score plot were identified based on variable importance in projection (VIP) statistics. The discriminatory metabolites were further evaluated for statistical significance based on p-value <0.05. Results: The median age in TA and SLE groups was 27 years and 33 years respectively. Female to male ratio was 3.5:1 in the TA group and was 31:1 in the SLE group. An exquisite separation in 3D score plot of PLS-DA clearly revealed that the metabolic disturbances associated with SLE are specific and different to TA [Figure 1A]. Sera of SLE patients were characterized by increased serum levels of glucose, glutamine, histidine, tyrosine, valine, isoleucine, arginine and ornithine, whereas the serum levels of NAG, lactate, lipid/membrane metabolites including low/very-low density lipoproteins (LDL/VLDL), PUFA, choline and Glycerophosphocholine) and several amino acids (including alanine, aspartate, proline, glutamate, and leucine) were found to be decreased significantly compared with TA [Figure 1B]. The increase glucose and glutamine levels in SLE patients [Figure 1C] clearly suggested that SLE patients have highly dampened glycolysis and glutaminolysis and activated membrane metabolism under conditions of systemic inflammation and oxidative stress whereas hypoxic environment in TA leads to activates glutaminolysis.



Conclusion: Significant metabolic differences are found between SLE and TA patients reflecting the differences in pathophysiology of two diseases.


  PB059 Vasculitis Top


Serum BAFF and April are increased in Asian Indian patients with Takayasu arteritis

Abhishek Zanwar, Avinash Jain, Smirti Chaurasia, Sandeep Chaudhury, Durga Prasanna Misra, Ramnath Misra; Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttra Pradesh, India

Background: Takayasu arteritis (TaK), a large vessel vasculitis, usually affects young female. The pathogenesis is not clear and treatment is based on immunosuppressive drugs with corticosteroids in the active phase of the disease. Anti cytokine therapy, such as anti IL6 receptor and TNF inhibitor have been used with limited success. There are only handfuls of studies to indicate role of B cells. However, there are reports of the presence of anticardiolipin and endothelial antibodies in the sera of patients. Therapies targeting B cells have changed management of autoimmune disorders. We therefore, decided to assess serum level of BAFF and APRIL, in TaK.

Methods: Sera from 57 TaK patients according to ACR criteria were collected at initial and follow-up visit. Level of BAFF and APRIL by ELISA (R and D Systems, Minneapolis, MN, USA) was compared with 17 age matched healthy control. Clinical details of the patients were recorded. Disease activity was measured by Indian Takayasu arteritis disease activity score (ITAS A). Damage was measured by Takayasu Arteritis Damage Score (TADS). Using independent T test, comparison was done between healthy controls vs patients and active Vs inactive TaK.

Results: There were 44 females and 13 male. Mean age of TaK patient was 29.38 ±10.1 years. The mean duration of disease was 4.55 (±4.1) years. Type V was most common angiographic subtype (n=37). Median (IQR) ITAS, ITAS A using ESR, TADS were 0(0-7), 2.5(1-6.75) and 7(5-9) respectively. Patients were on corticosteroid-34, Methotrexate-22, Azathioprine-10, and Mycophenolate-2. Mean BAFF levels measured from 57 TaK patients at initial visit were significantly raised as compared to healthy individuals (205.15 ± 164 pg/ml vs 115.22 ± 77 pg/ml, p=0.031). Serum APRIL levels of 34 TaK patients was also found to be significantly raised as compared to 17 healthy individual (2084 ± 1432 pg/ml Vs 1364 ± 855 pg/ml, p=0.007) [Figure 1]. There was no significant difference in the serum levels of BAFF and APRIL between active and inactive group. There was neither correlation with TADS nor any change in follow up with change in ITAS.



Conclusion: BAFF and APRIL levels are increased in TaK patient compared to healthy control, supporting role of B cell in pathogenesis of TaK.


  PB060 Scleroderma, Myositis and Overlap Top


Serum BAFF in Indian Patients with IIM: Novel clinico-phenotypic associations in children and adults

Latika Gupta, Smriti Chaurasia, Puja Srivastava, Sanjay Dwivedi, Able Lawrence, Ramnath Misra; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background/Purpose: We studied the levels of B-cell survival-factors BAFF and APRIL in serum and their relation with clinico-laboratory associations and response to treatment.

Methods: Clinical data and sera were collected from 75 patients with IIM (Bohan and Peter's criteria 1975) and 25 healthy adults of similar gender and analyzed for BAFF and APRIL by ELISA (R and D system, Minneapolis, MN, USA). Sera from myositis patients were also analyzed for MSAs and MAAs against SRP, Mi2, Jo1, PL 7, PL 12, EJ, OJ, Ro 52, Ku, Pm-Scl 75 and PM-Scl 100, using line immunoblot assay (Euroimmun, Lubeck, Germany).

Results: Of 75 patients, 59(79%) were adults and 42 had DM and 17 PM. Median disease duration was 4 (2-12) months. BAFF levels were higher in IIM [430.3 pg/ml] than healthy controls [115.1 pg/ml; p=0.001], and in children [641.2 pg/ml] than adults [430.3 pg /ml; p=0.026] [Table 1]. In adult IIM, BAFF levels were higher in those negative for MSAs [n=40 and 35, 520.2 vs. 387.8 pg/ml; p=0.012] and ANA [n=23 and 34, 430.3 vs. 341 pg/ml; p=0.025]. The levels of BAFF were highest with those positive for anti-Ro-52 [F=2.05, R2 =0.195; p=0.045]. Among the various MSAs, lowest levels were seen in those with anti-SRP [n=3 and 56, 79.5 vs. 416.9 pg/ml; p=0.043]. BAFF levels were higher in patients with arthritis [n=23 and 31, 483.1 vs. 326.6 pg/ml; p=0.018], weight-loss [n=14 and 45, 688.7 vs. 348.1 pg/ml; p=0.007], and PAH [n=4 and 55, 1001 vs. 387.8 pg/ml; p=0.004]. Lower levels were associated with functional class 4 (n=11) than class 2 or 3 [n=22 and 26, Bartlett's statistic 9.16; p=0.01], lip-edema [n=2 and 57, 58.9 vs. 416.9 pg/ml; p=0.036] and bulbar weakness[p=26 and 33, 345.9 vs. 430.3pg/ml; p=0.103]. In children, levels did not differ between various phenotypes. Serum APRIL levels were higher in IIM [1112 pg/ml] than healthy controls [115.1 pg/ml; p=0.0001] but levels didn't differ amongst the clinico-serologic phenotypes. Median follow-up duration was 145 patient years. 12 patients relapsed while 9 were in drug free remission. BAFF were similar between these groups.



Conclusion: Serum BAFF levels are elevated in IIM, more so in children and anti-Ro positive. MSA positivity and severe disease are associated with lower BAFF levels at baseline, suggesting probable role as a biomarker of disease severity.


  PB061 Scleroderma, Myositis and Overlap Top


Role of dendritic cells in systemic sclerosis

Ram Raj Singh1,3,4,5, Suzanne Kafaja1, Isela Valera1, Rajan Saggar1, Fereidoun Abtin2, Daniel E Furst1, Dinesh Khanna1, Anagha A Divekar1

Departments of Medicine, 1Radiological Sciences, and 5Pathology and Laboratory Medicine, David Geffen School of Medicine at University of California Los Angeles, 3Molecular Toxicology Interdepartmental Program, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, 4Jonsson Comprehensive Cancer Center, David Geffen School of Medicine at University of California Los Angeles, CA, USA.

Background: Fibrosis is a common end-result of tissue injury, including inflammation. Inflammation and fibrosis are hallmarks of systemic sclerosis (SSc). Mechanisms that drive fibrosis remain unclear. Studies in patients and animal models suggest a role of type-2-cytokine-producing T-cells in fibrosis development. Dendritic cells (DC) that play a role in T-cell priming/differentiation accumulate in the skin of SSc patients. However, DC's role in the pathogenesis of fibrosis is unclear. There are different subsets of DCs, including plasmacytoid DC (pDC). Previous studies have suggested a protective role of tissue-resident DCs and of non-pDCs in chronic inflammation and fibrosis, but the role of pDCs is unknown.

Methods: We performed ex vivo analyses of pDCs in the bronchoalveolar lavage (BAL), peripheral blood, and skin and lung tissues from SSc patients, and correlated them with disease parameters. To directly test the role of pDCs, we used the bleomycin model of fibrosis. We depleted pDCs, and monitored its effect on fibrosis.

Results: While pDCs were reduced in the peripheral blood of patients with SSc compared to healthy donors, their numbers were increased in the lung and skin of patients with SSc. The frequencies of pDCs in BAL correlated with HRCT scores of ground-glass opacity and fibrosis, and with CD4+ and IL-4+ T-cells in BAL. Treatment with tyrosine-kinase inhibitor imatinib that has been reported to reduce skin fibrosis and prevent deterioration of SSc-lung disease reduced pDCs in the BAL but not in peripheral blood of patients. In animals, pDCs were more profoundly increased than other immune cells in the lung and lung-draining lymph-nodes, but not in the spleen, of bleomycin-injected mice compared to controls. Depletion of pDCs improved disease scores, and collagen content compared to controls. B-cells, T-cells, and natural killer T-cells were reduced in the lungs, but unaffected/increased in the spleen, of pDC-depleted mice as compared to controls. pDC-depleted mice also had a reduced expression of genes involved in chemotaxis, DC differentiation, inflammation, and fibrosis in the lungs as compared to controls.

Conclusions: Our results identify the increased trafficking to pDCs to the affected organs as a potential therapeutic target in fibrotic diseases.


  PB062 Scleroderma, Myositis and Overlap: XIST Top


Antisense of TSIX: A probable regulator of fibrosis in diffused systemic sclerosis

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

Background: Uncontrolled fibrosis is the major cause of the multiple organ failure in systemic sclerosis (SSc). TGF-B plays the major role in triggering uncontrolled fibrosis in SSc. Recent studies have shown that long non coding RNA (lncRNA) TSIX is up regulated by overexpression of TGF-B in SSc which stabilizes collagen mRNA, thus supporting fibrosis. Reports have shown concomitant elevated expression of TGF-B and TSIX in SSc patients compared to controls. XIST, also termed as 'Antisense of TSIX', is synthesized from the antisense strand of TSIX.

Objective: To study the expression of antisense of collagen stabilizing lncRNA TSIX (XIST) and its relation with the fibrotic marker in Diffused Systemic Sclerosis.

Methods: 10 consecutive diffused cutaneous SSc patients (ACR, 2013), disease duration (5.4± 2.8), age (35.89±9.48) were selected and 10 age-sex matched healthy controls were also included. Blood was collected from all study participants. Serum was isolated and translational expression of TGF-B was evaluated by ELISA. RNA was isolated from PBMCs and serum. cDNAs were prepared, the expression of XIST and TGF-B was measured by real time PCR.

Result: Serum levels of TGF-B and XIST were significantly higher in SSc patients compared to controls (p=0.04, p=0.03 respectively). The expressions of TGF-B and XIST in PBMCs were also found to be significantly higher in patients compared to controls (p=0.04, p=0.04 respectively). TGF-B has positive correlation with Modified Rodnan Skin Score at both translational (r2=0.006, p=0.7) and transcriptional level (r2 =0.06, p=0.4) which validate the role of TGF-B as a contributor of fibrosis. Also there is a positive correlation between TGF-B and XIST at serum (r2=0.9, p=0.001) and PBMC (r2 =0.08, p=0.7) levels.

Conclusion: TSIX, which is upregulated by overexpressed TGF-B, has a role in collagen mRNA stabilization in SSc. Positive correlation between TGF-B and XIST indicates that XIST is upregulated with the progression of fibrosis in SSc. XIST upregulation in SSc patients might be a transcriptional effort to compensate over expression of TSIX thereby maintaining TSIX-XIST homeostasis. The exact mechanism of action of XIST might lead to better understanding of SSc pathogenesis and open newer avenues for designing therapeutic approaches.


  PB063 Scleroderma, Myositis and Overlap Top


Role of biomarkers KL-6, SP-D and CCL-18 in assessing disease severity of interstitial lung disease in systemic sclerosis

Shalini Dubey, Zena Patel1, Girish Kakde2, C Balkrishnan2, Rohini Samant2; Research Laboratories, P. D. Hinduja Hospital and Medical Research Centre, Department of 1Radiology and 2Rheumatology, P. D. Hinduja Hospital and Medical Research Centre, Mumbai, Maharashtra, India

Background: Interstitial lung disease (ILD) is a significant complication and the leading cause of mortality in systemic sclerosis (SSc). Progression of restrictive lung disease is variable and requires prompt recognition with careful monitoring.

Objectives: To elucidate the clinical significance and utility of serum-biomarkers viz KL-6, SP-D and CCL18 in the monitoring of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc). To correlate the serum levels of these markers with the extent of interstitial lung involvement by HRCT and the functional impairment. (FVC% and DLCO).

Methods: Serum KL-6, SP-D and CCL18 concentrations were determined by ELISA in 77 SSc patients, after obtaining informed consent. Lung function parameters were assessed, and the extent of ILD was measured by HRCT. Correlation between the serum level of these markers with ILD extent on HRCT and lung-function parameters such as forced vital capacity and diffusing capacity of the lungs for carbon monoxide were studied using Pearson's™ correlation test. The serum levels of these markers were also correlated with other parameters like Rodnan score, autoantibody profile and disease duration. Impact of treatment on the serum level of the markers was also studied.

Results: Mean serum concentrations of all three-serum markers were observed to be higher in patients with diffuse systemic sclerosis when compared to those with limited form of the disease. Moreover, patients with ILD were observed to have higher mean serum concentration of these markers in comparison with patients without ILD. There was an inverse correlation between KL-6 and FVC%, a similar trend was observed with other two markers but did not reach statistical significance. SP-D had inverse correlation with DLCO, the remaining two markers followed the same trend without statistical significance. SP-D and CCL18 levels had direct correlation with the Rodnan score.

Conclusion: KL-6 and SP-D values were found to correlate with disease severity as measured by FVC% and DLCO respectively in this group of systemic sclerosis patients.


  PB064 Scleroderma, Myositis and Overlap Top


Sudomotor dysfunction in scleroderma

Ashit Syngle1,2, Nidhi Garg3, Sudeep Kaur1, Tanya Syngle4, Devaansh Syngle5; 1Cardio-Rheuma and Healing Touch City Clinic, 4Healing Touch Foundation, Chandigarh, 2Fortis Multi Specialty Hospital, Mohali, 3Chitkara College of Pharmacy, Rajpura, Punjab, 5SGRR Institute of Medical and Health Sciences, Dehra Doon, Uttarakhand, India

Background: Scleroderma is an autoimmune disease characterized by a vasculopathy, diffuse fibrosis of skin and various internal organs, and immune abnormalities. Accumulating evidence indicates that the immune and autonomic nervous systems (ANS) are major contributors to the pathogenesis of Scleroderma. However, the association between the sudomotor dysfunction and inflammation has not yet been investigated in Scleroderma.

Objective: To investigate the relationship between sudomotor function and inflammation in Scleroderma.

Methods: In this study, 15 Scleroderma patients were recruited. Peripheral sympathetic autonomic function assessed by Sudoscan through measurement of electrochemical skin conductance of hands and feet. Sudoscan investigates the sweat gland activity and used as a surrogate to study the damage of sympathetic sudomotor nerves in neuropathy. Disease-specific measures and inflammatory markers (ESR and CRP) were determined. 15 age and sex matched healthy controls were enrolled as control group.

Results: Scleroderma patients had significantly impaired sudomotor function (p<0.01) compared with healthy controls. ESR and CRP levels were significantly (p<0.05) high in scleroderma patients as compared to controls. Significant negative correlation was found between sudoscan and CRP (r=-0.72, p=0.01), ESR (r=-0.60, p=0.02) and age (r=-0.66, p=0.01). Increasing age, CRP and ESR was found to be associated with an impairment of sudomotor function.

Conclusion: Sudomotor dysfunction occurs in Scleroderma. Inflammation plays an important role in the modulation of peripheral sympathetic autonomic function in Scleroderma. This study provides important support to the hypothesis that biomarkers of inflammation (CRP, ESR) predict sudomotor dysfunction in scleroderma.


  PB065 OA, Osteoporosis Crystal Arthropathy Top


Investigating inflammation-mediated cytokines in human osteoarthritis explants cartilage culture

Manju Singh, Subhashis Pal, Naibedya Chattopadhyay, Urmila Dhakad, Ragini Srivastava and Siddharth K Das; Department of Rheumatology, K.G. Medical University, 1Department of Endocrinology, CDRI, Lucknow, Uttar Pradesh, India

Objective: Colchicine has been using in treatment of osteoarthritic and several other diseases but mechanism of action of colchicine is not known till now. We postulated that colchicine block the NLRP3 pathway. Aim of study is to see the effect of colchicine on IL-1β, TNFα±, capase-1 and pro cathepsinB in human osteoarthritic explants.

Methods: a) Articular cartilage was removed from the knee joints using a scalpel. The tissue was placed in F-12 medium containing 100 IU/ml penicillin, 100 μg/ml streptomycin, 2 mMglutamine, and 50 μg/ml gentamycin. Explant discs (5 mm, ~ 50 - 100 mg) were made from the cartilage and placed into 48-well plates containing 500 μl medium. The explants were incubated at 37°C for 7 days. Sixteen explants discs were used per treatment group with colchicine (25 μM). Following 7 days of treatment, the explants were frozen in liquid nitrogen, and the explants and medium were stored at -80°C. b) ELISA of IL-1β, TNF-α Caspase-1, and Pro-cathepsin-B assay: - Total IL-1β, TNFα± Caspase-1, and Pro-cathepsin-B content was measured in 100-200 μl samples of explants medium using the ELISA Kit. c) Quantitative real-time PCR (qPCR): - Quantitative determination of collagen IA, collagen IIA, aggrecan, IL-1α±, IL-1β, TNFα±, IL-6 and chondrogenic genes compared with housekeeping gene glyceraldehyde-3-phosphate dehydrogenase (GAPDH) in chondrocyte. The explants were collected in trizol to extract RNA; cDNA was synthesized. For qPCR, the cDNA was amplified using ABI Step one plus.

Result: Colchicine treated and nontreated osteoarthritic human explants supernatant ELISA of IL-1β, TNFa, caspase-1 and pro cathepsin-B. ELISA results showed level of IL-1, TNFa, caspase-1 and pro cathepsin-B levels are higher osteoarthritic explants but levels are reduced when explants are treated by colchicines. Gene expression results of inflammatory (IL-1β, TNFα) and anti inflammatory (IL-6, IL-10) cytokines are also altered by treatment with colchicine in case of human osteoarthritis explant.

Conclusion: These results conclude that in human osteoarthritic explants produce IL-1β, TNF-α, caspase-1 and pro cathepsin-B are produced. Whereas production of IL-1β, TNFα, caspase-1 and pro cathepsin-B blocked by colchicine treatment. This study also indicates the singling of IL-1β, TNFα, IL-6, IL-10are downregulated by colchicine in case of osteoarthritic explants.


  PB066 Fibromyalgia Top


A study to detetmine the correlation between Vitamin D deficiency with fibromyalgia in a tertiary care hospital in India

Anirban Bhowmik, Shib Shankar Kundu; West Bengal University of Health Sciences, Kolkata, West Bengal, India

Introduction: Fibromyalgia (FM) is a clinical syndrome defined by the presence of generalized pain, fatigue, unrefreshed sleep, multiple somatic symptoms, cognitive dysfunction and depression. American College of Rheumatology (ACR) 2010 diagnostic criteria for FM requires the presence of widespread pain and multiple symptoms. There is an enormous controversy about the role of vitamin D in FM. Data from the Indian subcontinent is lacking.

Aims: 1. To determine the serum 25OH Vit D concentrations among both new and old diagnosed cases of fibromyalgia. 2. to ascertain the correlation between Vit D concentrations with clinical manifestations based diagnostic criteria for FM 3. To asses the difference in Serum 25-OH D concentration, if any, according to different demographic characteristics of the study subjects as well as seasonal variations.

Methods: We have included 63 new and old diagnosed FM patients (based on ACR 2010 criteria)attending RG Kar Medical College, Kolkata, India for one and half year (from 31st jan 2014 to 31st july 2015). Serum 25-(OH) D was assessed by radioimmunoassay. Data was analyzed by appropriate statistical methods using SPSS software version 20.

Result: Study comprised of 63 patients; including 15 (23.8%) males and 48 (76.2%) females. Mean age of patients were 42.22 ± 10.05 years with highest no of patients in the age group 40-49 years. There was statistical significant (p<0.001) negative correlation between serum 25 (OH) D levels and widespread pain index, symptom severity scale score and fibromyalgianess scale score. We have found statistically significant low serum 25 (OH) D levels in Muslim women than Hindu, and in winter months than summer. There was statistically insignificant 25 (OH) D deficiency in the patients working indoor.

Conclusion: To conclude assessment of Vitamin D status should be taken into account in the evaluation of patients with nonspecific musculoskeletal pain or FM and look for the causes of vitamin D deficiency. The reasons for the low vitamin D in Muslim women could be due to dietary differences, cultural habits and concealed dressing. Higher level of 25 (OH) D in summer is attributed to increased UV-B exposure during summer months.


  PB067 Paediatric Rheumatology Top


Evidence for M2 macrophage activation in patients with enthesitis related arthritis category of Juvenile idiopathic arthritis

Shruti Bhattacharya, Priyanka Gaur, Akhilesh Yadav and Amita Aggarwal; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Introduction: Among juvenile idiopathic arthritis (JIA), enthesitis related arthritis (ERA) category includes most children with juvenile onset spondyloarthropathy (SpA). Synovial fluid from patients with SpA causes more M2 polarization of peripheral blood monocytes than SF from patients with RA. Recently an increase in CD163+ macrophages was seen in ileal biopsies from patients with AS. A 6.5 fold higher expression of CD163 has been reported in SFMC gene expression as compared to PBMC of children with ERA. Thus we studied levels of sCD163 as a marker of activation of M2 macrophages in serum and synovial fluid of children with ERA.

Methods: Serum samples from patients with ERA and healthy young adults were assayed for sCD163 using ELISA (R and D systems). In addition SF from children with ERA was also analyzed where available.

Results: Sera from 85 patients and synovial fluid from 32 patients with ERA and serum from 46 young adults were analyzed. The average age of patients at inclusion was 16 + 3.24 years and age at onset was 11.2 + 2.79 years. 79 of them were boys and HLA B27 was positive in 64/80 patients (data not available in 5). The mean serum sCD163 levels were higher in patients as compared to healthy controls (1433.61 + 1256.11 versus 907.35 + 726.56 ng/ml; p<0.001). The synovial fluid levels were much higher than serum levels (4307.38 + 3363.92 ng/ml; p<0.003). Data on disease activity was available in 56 patients. The mean TJC was 3+2.7, SJC was 2.3 +2.2, ESR was 74 + 34 mm and CRP was 6.98 + 5.4 mg/dl. The serum levels of sCD163 did not have any correlation with disease activity parameters.

Conclusion: A higher levels of sCD163 in serum of patients with ERA and even higher levels in paired SF suggest that there is activation of alternatively activated macrophages in ERA. Lack of correlation with activity may suggest that they have immune regulatory role in patients with ERA.


  PB068 Infection Related Rheumatic Disease Top


Increased infiltration by T cells, macrophages and neo-angiogenesis may differentiate chronic tuberculous arthritis from chronic inflammatory arthritides

Deepak Tripathi, Vinita Agarwal, Sajal Ajmani, Vikas Agarwal; Departments of Clinical Immunology and 1Pathology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Histopathological studies of synovitis show a variety of features, some with clearly defined, disease-specific features and others with non-specific findings. Till date, there are no studies comparing histological and immunohistochemical features of synovitis due to Tuberculous arthritis (TA) and other chronic inflammatory arthritides.

Objective: To find out differentiating features of inflammation on histology and immunohistochemistry (IHC) in chronic synovitis of the knee due to varied etiologies.

Methods: Arthroscopic synovial biopsies were obtained from inflamed knee joints of 46 patients with the chronic inflammatory synovitis. Histological analysis was performed with Rooney's scoring system analyzing different parameters of inflammation: synovial hyperplasia, percentage fibrosis, number of blood vessels, focal aggregates of lymphocytes, percentage of diffuse infiltrate of lymphocytes and percentage of perivascular lymphocytes. IHC analysis was performed to detect subtypes of T cells, B cells, plasma cells, macrophages and endothelial cells numbers in the inflamed synovium.

Results: There were 46 patients, mean age 40.58 years [range, male 34]. There were 18 patients with Tubercular arthritis (TA), 3 with Osteoarthritis (OA), 6 with Rheumatoid arthritis (RA) and 19 with seronegative spondyloarthropathy (SpAs). On histological analysis, percentage of perivascular lymphocytes was significantly higher in TA as compared to OA; however, it was similar to SpAs and RA. On IHC, CD3+ and CD4+ T cell and CD34+ endothelial cell numbers were higher in the synovium of patients with TA (p<0.01) as compared to rest of the groups. Macrophages were significantly increased in TA synovium as compared to RA, OA, but not SpAs. The number of IL-1ß and TNF-A expressing cells were significantly high in the synovium of TA as compared to RA, SpAs and OA. Using ROC analysis, T cells (CD3, CD4, CD8), CD34, IL-1ß and total infiltrating cells yielded the curve with the highest AUCs in favor of TA.

Conclusion: Increased T cell, macrophages and endothelial cells on IHC may help in differentiating chronic TA from other chronic inflammatory arthritides.


  PB069 Miscellaneous Rheumatic and Inflammatory Disease Top


Mantoux test is inadequate to define all subjects with latent Tubercular Infection

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

Background: Latent TB infection (LTBI), defined as ' a state of persistent immune response to  Mycobacterium tuberculosis Scientific Name Search thout clinically-manifested disease', inflicts a third of the world's population and nearly 10% of LTBI positive persons develop TB within 2-5 years. The tuberculin skin test (TST) and, more recently, interferon gamma release assays (IGRAs) are most commonly used for detection of LTBI. However, in a high TB burden setting such as India, both the assays have been found to grossly underestimate the true prevalence of LTBI, since almost equal number of new TB cases emerged from the test-positive and test-negative groups during the follow-up (Sharma et al, PloS One, 2017, e0169539).

Objectives: This study was aimed at exploring whether an in vitro CD3+ T cell response to PPD can complement the in vivo TST response for the determination of true prevalence of LTBI in healthy Indians.

Methods: In this ongoing study, 80 apparently healthy workers (age 19-61 years) at SGPGIMS have been recruited. Their demographic data, including BCG vaccination status and duration of contact with TB patients, was recorded. TST was performed with 5 TU of PPD. Blood T cell (CD3+) responses to PHA (a mitogen, used as positive control) and PPD were determined by flow cytometry in terms of expression of the proliferation-induced nuclear protein Ki67.

Results: 48% of the study subjects showed positivity for TST (skin induration ≥10 mm) and the size of reaction could be correlated with age. There was no association between BCG vaccination status and TST positivity. 72% of the TST positive and 62% of TST negative persons showed positivity for CD3+ T cell response to PPD. Positivity for either assay was found to be 82%.

Conclusion: By combining TST with CD3+ T cell responses, the positivity for PPD was enhanced from 48% (TST alone) to 82%. Therefore, both the assays could be considered as complementary. It remains to be seen whether these assays, either singly or jointly, show a correlation with the emergence of TB in our study population during the follow-up.


  PB070 Miscellaneous Rheumatic and Inflammatory Disease Top


Inflammation and hyperplasia in patients with rheumatoid arthritis and periodontitis: A long noncoding RNA perspective

Sudipta Chatterjee, Dipanjan Bhattachrjee, Sanchaita Misra, Alakendu Ghosh; Department of Rheumatology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

Background: Synovial hyperplasia resulting from hyper-proliferation of synovial fibroblasts outlines the basic pathogenesis in Rheumatoid Arthritis (RA). Periodontitis (PD) has similar pathogenesis in gingival tissue and is reported to have associations with RA. Cell cycle regulation is controlled by numerous factors, Long Non-Coding RNAs (lncR) being one of them. Tumor suppressor lncR (MEG3), pro-metastatic lncR (MALAT1) and transcription regulating lncR (NEAT1) have been implicated in different forms of cancer.

Objective: To determine and compare the systemic expression of regulatory RNAs (lncRs) involved in controlling the cellular proliferations in patients with RA and Periodontitis.

Methods: 30 consecutive RA patients (as per ACR 2010), 15 with PD and 15 without PD were selected from Rheumatology OPD. 15 patients having generalized PD (as per British Society of Periodontology 2011) were selected from Dentistry OPD. None had antimicrobial or steroid therapy in last 2 months. 15 age-sex matched healthy controls were also included. Serum levels of anti-CCP, TNFA±, IL1β, IL8 were measured by ELISA and RF, CRP by Nephelometry. Gingival clevicular fluids (GCF) were collected using paper points and inflammatory cytokines were similarly analysed. DAS28 and Periodontal scorings were done following respective guidelines. RNA was extracted from plasma and isolated PBMCs. cDNAs were constructed. Expression levels of lncRsMEG3, MALAT1 and NEAT1 were measured by Real Time PCR.

Results: DAS28 scores, TNFA±, IL1β, IL8 levels (in serum and GCF) were significantly higher in RA with PD patients compared to the other groups. The expression levels of MEG3, MALAT1 and NEAT1in PBMCs were significantly higher in patients with RA and PD compared to the other groups (p<0.05). Similar expression patterns were seen in plasma as well. The mutual expressions of the lncRs could be compared as: MALAT1>>> NEAT1> MEG3, in all groups.

Conclusion: Higher serum and GCF levels of inflammatory cytokines in RA with PD patients compared to RA without PD indicated the contribution of periodontitis in systemic inflammation. Increased expression of MALAT1 in RA with PD patients might trigger the synovial hyperplasia which could not be ameliorated by MEG3 owing to its comparative low expression. Increased expression of NEAT1 might regulate inflammatory cytokine transcription could not be ameliorated by MEG3 owing to its comparative low expression. Increased expression of NEAT1 might regulate inflammatory cytokine transcription.


  PB071 Miscellaneous and Inflammatory Disease Top


Skewing of T Helper axis towards Th1 and Th17 cells in sarcoid arthritis compared to non-articular sarcoidosis

Avinash Jain, Harshit Singh, Saurabh Chaturvedi, Sanat Phatak, Sajal Ajmani, Alok Nath, Durga P Misra, Vikas Agarwal; SGPGIMS, Lucknow, Uttar Pradesh, India

Introduction: Sarcoidosis is a disease with diverse manifestations and an unclear pathogenesis. An insight into the T cell signature may help us understand the disease better. Objective of the study was to assess T cell subsets in sarcoidosis patients with or without articular involvement.

Methods: Diagnosis of sarcoidosis was based on Non-caseating granulomas negative for AFB, fungal elements or foreign bodies in lymph nodes, liver, skin or exocrine glands and/or Oligo- or polyarthritis with hilar adenopathy, erythema nodosum, uveitis or facial palsy with negative Mantoux test. All patients were treatment naive. They were divided into two groups - Group A comprised of patients with articular involvement and Group B patients had no articular involvement. T cell immunophenotype was done in peripheral blood by flow cytometry with appropriate gating using CD4, CD8, IFN gamma, IL-2, IL-17, and FOXP3 for CD4, CD8, Th1, Th2, Th17 and Treg cells respectively. A record of the clinical details including the management of these patients was made.

Results: 23 patients with sarcoid arthritis were compared with 12 patients of sarcoidosis without articular involvement [8 with pulmonary involvement, 2 renal, one gastrointestinal and one neurosarcoidosis] with mean age 41.2 ± 10.5 years and 39 ± 9.23 years respectively. Male: Female ratio was 9:14 and 3:1 in group A and B respectively. Mean total leucocyte count was comparable in both groups (6971.6 ± 3288.6 cells/mm3 in group B vs 7852 ± 2049.1 cells mm3 in group A, p = 0.33). Percentage of Th1, Th2, CD8+ T cells were significantly high in sarcoid arthritis whereas Treg cells were higher in the non-articular [Table 1]. Th17 cells were lower in group B but did not reach statistical significance [p 0.11]. Th1/Th2 were low and Th1/Treg and Th17/Treg ratio were high in sarcoid arthritis [p<0.05].



Conclusion: T helper cell axis was skewed towards Th1 and Th17 in sarcoid arthritis when compared to patients without articular involvement. Thus this difference in T cell subsets may explain to an extent the diversity in the disease manifestations and may have implications on management. It will be worthwhile to study the T cell repertoire in other tissue or body fluids


  PB072 Miscellaneous Rheumatic and Inflammatory Disease Top


IgG4 related disease

Prijesh Janardanan, Remesh Bhasi1; Departments of General Medicine and 1Rheumatology, Aster MIMS, Calicut, Kerala, India

Background: IgG4 RD is a cluster of disorders, which has been increasingly recognized over the past few years with certain common characteristics. Apart from the multi organ involvement IgG4 RD also has lymphocytic infiltrate rich in plasma cells with storiform pattern as a common attribute. In addition response to glucocorticoid therapy is also a recognized feature for the diagnosis of IgG4 RD.

Objectives: To compare the clinical, pathological and biochemical characteristics of 5 patients with IgG4 RD Methods â€" 7 cases admitted for evaluation of symptoms pertaining to various systems, presenting with varied presentations were investigated and found to have IgG4 RD.

Results: The study subjects in the case series belonged to multiple age groups from age of 27 years to 72 years. M:F ratio was 4:3. Generalized lymphadenopathy was the most common presentation which prompted the further investigation in lines of IgG4 RD with 3 patients having the same. While one patient presented with autoimmune pancreatitis, 2 subjects had retroperitoneal fibrosis as the presenting feature. The diagnosis of IgG4RD was a coincidental finding in one patient who was evaluated for fatigue and found to have kidney disease. All the patients had elevated IgG4 levels. 2 patients had rheumatoid factor positive from before and was being treated accordingly. Antinuclear antibody and anti neutrophil cytoplasmic antibody was positive in one of the patients. 3 of the patients had biopsy consistent with IgG4RD.

Conclusion: IgG4 RD still remains an elusive disease and often under diagnosed. There was an average time gap of 2 years for the patient to be diagnosed with IgG4RD. Serum IgG4 levels and biopsy proved vital for the identification. All the patients responded well to steroids and are under constant follow up.


  PB073 Miscellaneous Rheumatic and Inflammatory Disease Top


Factors affecting methotrexate non-compliance in systemic autoimmune rheumatic diseases a study by nurse counselors and allied health professionals

S S Baghel, R Thakran, C Messi, N Yadav, S Kapoor, S R Garg, Vivekanand, Zaheer Q, A N Malaviya1; Departments of Immunologyand 1Rheumatology, ISIC Superspeciality Hospital, New Delhi, India

Background: Methotrexate (MTX) is universally regarded as the anchor drug in the management of rheumatoid arthritis (RA). It is usually administered orally or subcutaneously in once weekly doses. Non-compliance with MTX in patients with SARDs among the common causes of poor disease control.

Objectives: To assess the degree of non-compliance to MTX treatment and to identify the reasons for the same.

Patients and Methods: This prospective study included 271 patients with RA. They were asked about the compliance to the advised MTX dose during their follow-up visit to the clinic. Those who were noncompliant were then enquired about the reasons for noncompliance. The results were recorded in a predesigned form. The results were analysed correlating noncompliance with demographics, MTX adverse effects, MTX nonresponse, personal-family issues and patients understanding regarding the drug.

Results: 1. 222 (81.9%) patients were fully compliant with the prescribed MTX dose. 2.49 (18.08) were not compliant, to MTX. The noncompliance varied from occasional to complete default. The factors that were identified for non-compliance included: a) 22 (44.8%) patients had MTX intolerance that led 9 (40.9%) patients to completely discontinued the medicine and 13 (59.09%) decreased the MTX dose. b) 1(2.04%) patients discontinued medicines as they had achieved remission and believed that they were cured. c) 4 (8.16%) patients were not aware of the importance of regular long-term treatment with regular follow-up in the rheumatology clinic. d) 7 (25.9 %) patients developed adverse effects of MTX (oral ulcer, transaminitis) that required dose modification/temporary discontinuation. e) 5 (18.5 %) patients were lost to follow-up. f) 4 (14.8%) patients reported non-availability of MTX in the place of their residence. g) 4 (8.16 %) patients simply forget to take the medicines. h) 2 (4.08%) patients were advised to discontinue treatment by other caregivers (non-rheumatologist) who also advised that the patient shifts over to much safer alternative systems of medicine.

Conclusion: Most frequently reported reasons for non-compliance to MTX were intolerance. The patients need more intensive and focused counselling about the management of intolerance and long term treatment. Correct information and positive attitude towards medication could help in increasing the compliance.


  PC074 Rheumatoid Arthritis Top


Effect of Common Polymorphisms in the Methotrexate Pharmacokinetic Pathway on efficacy/ adverse effects and Methotrexate Polyglutamate Levels in RA

Amit Sandhu, Varun Dhir, Archana Bhatnagar1, Shabeer Ahmed; PGIMER, 1Punjab University, Chandigarh, India

Purpose: This study investigated the impact of seven common polymorphisms in genes involved in methotrexate (MTX) pharmacokinetics on response, adverse effects and methotrexate polyglutamate (MTXPG) levels in rheumatoid arthritis.

Methods: This study enrolled 117 RA patients who were treated prospectively with MTX for 24 weeks. Patients were categorized on the EULAR criteria into responders (good and moderate) and non-responders. Adverse effects were ascertained using a questionnaire. The following polymorphhims were ascertained using hyrolysis probes - rs1045642 (ABCB1 3435C>T), rs1128503 (ABCB1 1236C>T), rs10106 (FPGS 1994A>G), rs1544105 (FPGS G>A), rs11545078 (GGH 452C>T), rs3758149 (GGH -401C>T) and rs1051266 (RFC1 80G>A). RBC MTXPG1-5 levels were determined using HPLC at 4,8,16 and 24 weeks.

Results: There was a significant association of the GGH 452C>T CC genotype (OR 9.5, 95% CI 1.2 to 76.0) with response to MTX. On logistic regression, higher DAS28 (3) at baseline and GGH 452CC genotype were significantly associated with response. The accuracy of the model was 75%. The FPGS 1994A>G GG genotype was associated with a significantly lower risk of adverse effects to MTX (Odds Ratio 0.3 (95% CI 0.1 to 0.6)). On logistic regression, FPGS 1994GG genotype and lower BMI were significant predictors for adverse effects with an accuracy of 66%. The other polymorphisms were not associated with response or adverse effects. None of the polymorphisms were associated with change in MTXPG levels.

Conclusion: GGH 452 CC genotype was found to be associated with response to MTX and FPGS 1994A>G GG with a lower risk of adverse effects; however, not by change in MTXPG levels.


  PC075 Rheumatoid Arthritis Top


Efficacy and B-Cell depletion with very low dose rituximab (biosimilar) in sero-positive DMARD resistant rheumatoid arthritis: A 24 week study from Western India

Nibha Jain, Sapan Pandya, Puja Srivastava; Department of Rheumatology, VS Hospital, Ahmedabad, Gujarat, India

Background: Very Low dose Rituximab (RTX) (100 mg) has been used in various autoimmune diseases like ITP, AIHA, etc. As cost of treatment is an important issue in our country, we decided to study Efficacy of this low dose RTX in patients with RA.

Objectives: 1) To study clinical efficacy of very low dose RTX in DMARD refractory RA. 2) To study B cell depletion after very low dose RTX infusion.

Methods: In this prospective open labelled study, biologic naïve, conventional DMARD refractory, sero-positive RA patients (fulfilling ACR 2010 criteria) with high disease activity (DAS 28 ESR), were included after informed consent. Arm I: Received Four 100mg RTX biosimilar infusion at one week interval (Total 400 mg) + baseline combination DMARDs. Arm II: Received Single 100 mg RTX biosimilar infusion + baseline combination DMARDs. • Efficacy was assessed using standard outcome measures for RA (including RAPID3) at week 12 (Arm I and II) and 24 (Arm I). • B cell depletion (CD19 levels in peripheral blood by flow cytometry) was studied at baseline, week 1 and week 12. The results were compared to other studies.

Results: • Total of 14 patients were enrolled in arm I and 9 patients in arm II. Baseline demographic profile, disease activity measures and EULAR response is shown in [Table 1]. • All patients achieved complete B-cell depletion (CD19=0) at week 1 after just single dose of 100mg RTX. • On comparing with other Indian and International studies using both high and low doses of RTX, our results were comparable.



Conclusions: Very Low dose RTX is efficacious in conventional DMARD refractory RA patients, as our results were comparable to other studies with low and conventional doses of RTX. Single dose (100mg) is as good as 400mg upto week 12. Complete B cell depletion can be achieved even with 100 mg RTX as early as week 1.


  PC076 Rheumatoid Arthritis Top


Disability and family burden in rheumatoid arthritis

Laxman Thakur, Anu Daber, Uma Kumar1; Departments of Medicineand 1Rheumatology, AIIMS, New Delhi, India

Background/Purpose: Rheumatoid arthritis is associated with marked physical disability. In addition, it has an impact on patients’ mental and social well-being, and also causes a major financial burden. An often ignored facet is the study of burden on family and caregivers of RA patients, which had been analysed as a part of this study. Limited data are available from India on these aspects.

Methods: 200 Patients with Rheumatoid Arthritis (RA) satisfying the ACR/EULAR criteria (2010) and 100 patients of Diabetes Mellitus (DM) satisfying ADA criteria with disease duration of at least 10 years as controls were recruited in this cross-sectional observational study over a period of 2 years. Caregivers were defined as those more than 18 years of age, staying with patient for atleast one year and not suffering from any long term disease for the purpose of this study. Approval from the institutional ethical committee was taken and data collected using a predesigned proforma after taking a well informed written consent from patients. Besides demographic data, disability was assessed by the administration of Indian modification of Health Assessment questionnaire (HAQ) and WHO Disability Assessment Schedule (WHODAS). Family burden was assessed by interviewing caregiver of the patient satisfying the inclusion criteria using Family Burden Assessment Schedule (FBAS).

Result: The mean age of 100 patients (87 women) of RA was 49.75(+8.63) years and 50 patients (19 women) of DM was 51.67(+7.17) years respectively. The mean duration of disease was 12.72 (+6.49) years for RA patients and 13 (+3.21) years for DM patients. The average monthly expense in RA group was Rs. 1988 (+845) while that in DM group was Rs. 1801 (+66). The mean Indian HAQ score in RA patients was 0.81(+0.49). The mean WHODAS score was 25% (+13.20%) and 13% (+10.23%) in RA and DM patients respectively, this difference being statistically significant (p< 0.0001). The mean FBAS score of RA and DM patients was 4.46 (+2.45) and 4.0 (+1.92) respectively, however, this difference could not gain statistical significance (p=0.2026).

Conclusion: The disability in RA patients was significantly higher than DM patients. Family burden was greater in RA patients but not statistically significant. Thus RA causes more disability but comparable family burden when compared to DM. An important take away message derived from this study highlights that despite RA causing greater disability and family burden as compared to prevalent non â€"communicable diseases such as DM, it fails to find its place among the non-communicable disease burden without being given it due importance.


  PC077 Rheumatoid Arthritis Top


To study 10-year risk of vaccine preventable infections in patients with rheumatoid arthritis

Ashok Kumar, Mehul Lapsiwala, Anunay Agarwal; Department of Rheumatology, Fortis Flt. Lt. Rajan, New Delhi, India

Background: ACR recommends 5 vaccines in patients with RA. The risk of vaccine-preventable infections in our RA patients is unknown.

Objective: To estimate the 10-year risk of vaccine preventable infections in patients with RA.

Methods: Medical records of RA patients registering with our rheumatology clinic between 1st January 2011 to 31st July 2015 were retrieved. History of episodes of infection since the diagnosis of RA was noted until their loss to follow up, death or end of study (30th June 2017). Details of all infection episodes and their potential predisposing factors (diabetes, alcoholism, ILD, maintenance steroid therapy), were retrieved. Details of disease modifying anti-rheumatic drugs (DMARDs: conventional and biological) received by patients and hospitalization, if any, were noted. Any treatment modification post-infection was also documented.

Results: Records of 1720 RA patients (20,542 patient-years) were studied. Majority (80%) were females. Median disease duration as on 30thJune, 2017 was 9 (IQR: 7, 17) years. At the end of available follow up, 68% had remission/low disease activity of RA and 52% had deformities. Predisposing factors for infection were present in 24% patients. DMARDs included methotrexate (81%), hydroxychloroquine (78%), sulphasalazine (26%), leflunomide (21%), etanercept (3%), rituximab (1.8%), adalimumab (1.4%), infliximab (1%), azathioprine (0.7%), cyclophosphamide (0.5%), cyclosporine/tacrolimus (0.2%), mycophenolate (0.1%). A total of 369 episodes of infection occurred in 201 (11.6%) patients. Common infections recorded were upper respiratory tract infection [URTI] (4.9%), herpes zoster (3.8%), bacterial skin infection (3.4%), gastroenteritis (2.1%), superficial fungal infection (2.1%), urinary tract infection (1.9%), tuberculosis (1.1%), pneumonia (0.9%). Other less common infections: subcutaneous tissue infection (n=6).


  PC078 Rheumatoid Arthritis Top


A rituximab dosing protocol based on peripheral B cell depletion achieves good disease control at significantly lower dose in rheumatoid arthritis

Glindow Antony, MariaymVarsha, Joseph, Kaveri Nalianda, Sreelakshmi Sreenath, Padmanabha Shenoy; Centre for Arthritis and Rheumatism Excellence, Kochi, Kerala, India

Aim: To assess the efficacy and cost effectiveness of Rituximab treatment protocol based on B cell depletion.

Methods: Seropositive (RF and/or CCP) RA patients with inadequate response (DAS28>3.2) to combo-DMARDs were treated with 500 mg of Rituximab. If the patient has not achieved B cell depletion (CD 19<0.001%) at 2 weeks another 500 mg Rituximab was given. If the patient has achieved B cell depletion and is not showing clinical response at 3 weeks patient was observed till 8 weeks and another 500 mg given only if the patient has not shown EULAR moderate response. Once the patient relapses as evidenced by increase in DAS > 1.2 during 2 subsequent visit and CD 19 >0.01 % patient was retreated.

Results: 102 patients were treated with 114 cycles of Rituximab and were followed for a mean of 14±4.82months. 97 patients (83.3 %) required only single infusion of 500 mg Rituximab and rest were given 2 infusion of 500 mg. With a mean dose of 588.79±222.93 mg, DAS28 decreased from a mean of 6.25+/-1.10 to 3.64+/-1.18 at 8 weeks. With this protocol 45 infusions (44.11%) induced EULAR good response and 50 infusions (49.01%) inducedEULAR moderate response. Only 7 (6.86 %) infusions failed to achieve any response. 25 patients achieved remission and another 18 were in low disease activity. Mean duration of response was 12.19 ±4.18 months. Upon relapse during follow up, 10 patients required repeat infusions. All these patients achieved EULAR good response with 500 mg dose.

Conclusion: By using this B cell depletion based protocol of Rituximab most of the patients could achieve reasonable control of disease activity with 30% of the recommended dose.


  PC079 Rheumatoid Arthritis Top


RADAI5 in the assessment of disease activity of rheumatoid arthritis in a real life setting!

Vignesh Mantharam, T N Tamilselvan, M Saravanan, Mythili Seetharaman, Saranya Chinnadurai, A Aravindan; Institute of Rheumatology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India

Background: Standard disease assessment scores in assessing disease activity in Rheumatoid arthritis (RA) is time consuming in a busy outpatient schedule. Utility of self-reported RADAI5 has been validated and found to correlate with the current disease assessment measures.

Objective: 1. To assess the utility of RADAI5 in the assessment of disease activity in RA. 2. To assess the impact of comorbidities and ACR functional status over RADAI5

Methods: 75 patients with RA as per ACR/EULAR classification criteria 2010 were included in the study. Demographic profile, comorbidities, ACR functional class, DAS28ESR, DAS28CRP, CDAI and SDAI were assessed and these patients were administered RADAI5. In question5, a maximum score of 10 was given for early morning stiffness present throughout the day. But in active RA, EMS lasts for 2 hours only. While we tested RADAI5 in 20 patients, 15 patients needed guidance to answer Q5. Hence we decided to add another NRS with a maximum score of 10 given for an EMS lasting for 2 hours or more (as already utilized in BASDAI-Q6) to assess modified RADAI5 (mRADAI5). Statistical analysis was done using SPSS software.

Results: 75 patients (F:M=14:1) with a mean age(42.7±10.9 years) and a median disease duration(4 years (1,30)) were assessed. 18 patients needed assistance to complete Q5 of RADAI5; 4 patients required help to complete the modifiedQ5.


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Clinical experience of tofacitinib in rheumatoid arthritis: Real-world data from 2 centres in South India

U R K Rao, Vijay K R Rao1, C Satyavati, Sripurna Deepti, J Shivanand, Datta Kumar, Maryam Younis, Archana Rani, A Shashikala, Amit Thorat2; Sri Deepti Rheumatology Centre, Hyderabad, Telangana, 1Manipal Hospital, Bengaluru, Karnataka, 2Pfizer Ltd, Mumbai, Maharashtra, India

Background: Tofacitinib is an oral Janus kinase inhibitor for the treatment of Rheumatoid Arthritis (RA). It has demonstrated efficacy and manageable safety in multiple phase I-III trials and long term extension studies extending over 8 years. Real world data with Tofacitinib is limited and published Indian data is unavailable.

Objectives: Evaluation of efficacy and safety of Tofacitinib in patients with RA not responding to conventional synthetic disease modifying anti rheumatic drugs (csDMARDs) or biologics (bDMARDs) in routine clinical practice.

Methods: Retrospective data from 2 centers in South India was studied where Tofacitinib was given 5 mg twice daily orally to patients with RA who did not respond to csDMARDs/bDMARDs. Efficacy and safety data was obtained using standardized formats at baseline, 1month and 3months.

Results: Data of 35 patients treated with Tofacitinib from Oct 2016 to June 2017 was analyzed. Mean age was 54 years (26-76), most were females (32/35), average duration of RA 11.5 years (2-30), 34/35 were seropositive (RF/ACCP) and 32/35 had erosive arthritis. All the patients were inadequate responders (IR) to Methotrexate (average dose 15-20 mg/wk) or to combination csDMARDs. Thirteen (37%) patients were bDMARDs IR- Etanercept 3, Tocilizumab 4, Abatacept 3, Golimumab 2 and Rituximab 1. Seven patients experienced bDMARDs (all Etanercept) were not IR but chose Tofacitinib for convenient oral medication. All patients received Tofacitinib along with csDMARDs and were evaluated before and after treatment $$[Table 1] and [Graph]. Mild and reversible adverse events noted were herpes simplex (1), headache (1), feet skin pigmentation (1), gingivitis (1), neck pain (1), pharyngitis (1), alopecia (1) and hypertriglyceridemia (1). One patient with recurrent upper respiratory infection stopped Tofacitinib after 3 months and one patient with severe thrombocytopenia (<20,000) stopped after 2 months. Two patients were lost to follow up after receiving therapy for 1 month who felt better.



Conclusion: Patients with severe RA showed good EULAR response as early as one month and continued to show improvement by the end of 3 months. No new safety signals were identified. Present real-world data is reassuring but it needs more experience among the rheumatologists over longer treatment duration.


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Comparison of clinical efficacy of split versus conventional weekly oral dose methotrexate in patients of rheumatoid arthritis: A case control study

H. Singh, B Kiran, N Kumar, M Yadav, A Mittal, R Mathur; Department of Medicine and Rheumatology, Pt BDS PGIMS, Rohtak, Haryana, India

Background: Methotrexate (MTX) is referred as the anchor drug for DMARD therapy in RA. With high MTX enteral doses, bioavailability decreases. Therefore, it is beneficial to spilt MTX into smaller doses and thus increase its concentration systemically, increasing efficacy without worsening adverse effects.

Objective: To compare changes in the disease activity when using weekly split dose in comparison to use of weekly conventional dose of MTX.

Methods: A total of 60 patients of Rheumatoid Arthritis (RA) as per 1987 ACR criteria having evidence of active disease were enrolled in the study. All subjects were divided randomly in two groups of 30 each. Group І were given the split dose of MTX and Group II was given conventional dose of MTX. MTX was started as an initial dosage of 10 mg /week for first 2 weeks followed by an incremental dose of 5 mg every 2 weeks. The dose of MTX in group I was split in equal divided doses separated by 12 hours apart who were receiving MTX at a dose of ≥15 mg/week. Disease activity using DAS-28 and CDAI in each of the subjects of either group was evaluated monthly for 3 months and data were analysed statistically by using Mixed Method Anova at the end of the study.

Results: All subjects included in both groups completed the study. The mean age (in years) of group I was 49.44 ± 11.67 and of group II 42.42 ± 11.82 respectively. DAS-28 in group I was 6.76 ± .74; 5.24 ± .74; 3.98 ± .7; 3.00 ± .75 and in group II 6.62 ± .85; 5.39 ± .9; 4.58 ± .81; 3.55 ± .93 at 0, 4, 8, 12 weeks respectively. CDAI in group I was 41.48 ± 11.14; 24.36 ± 8.13; 12.34 ± 5.73; 6.42 ± 4.4 and in group II 37.64 ± 11.12; 24.54 ± 9.4; 16.38 ±6.81; 9.62 ±6.1 at 0, 4, 8, 12 weeks respectively. Group I showed statistically significant improvement as compared to group II in disease activity score (p=.048).

Conclusion: Split oral dose MTX is observed to be clinically more efficacious than conventional oral dose in patients of RA.


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Descriptive study of Asian Indian patients with rheumatoid vasculitis in retrospect: A single, tertiary care centre experience

John Mathew, Arvind Ganapati, Ruchika Goel, Sandhya Pulikool, Ashish Jacob Mathew, Mahasampath Gowri1, Debashish Danda; Departments of Clinical Immunology and Rheumatology and 1Biostatistics, Christian Medical College, Vellore, Tamil Nadu, India

Background: Rheumatoid vasculitis (RV) is a disease with high morbidity and mortality reported in previous studies. We describe the Indian perspective on RV patients.

Objectives: To ascertain the percentage of patients attaining a BVAS remission with 3 months of immunosuppressive therapy and identify the factors predicting remission/persistently active disease, with treatment.

Methods: A retrospective chart based review of electronic medical records of 8984 patients of Rheumatoid Arthritis (RA) from January 2007-August 2016 was done for based on the Scott and Bacon criteria for RV.

Results: 63 patients of RV were identified, giving a period prevalence of 0.7%. Mean age was 50.7ï'±11.5 years and the median duration of RA was 6 years. Peripheral Nervous System involvement was the commonest manifestation of RV in 52/63 (82.5%) patients followed by skin involvement in 34/63 (53.9%). 52 (82.5%) patients had biopsy evidence of vasculitis. 26/51 (50.9%) patients were started on mycophenolate mofetil (MMF), 13/51 (25.5%) patients on cyclophosphamide (CYC), 8/51 (15.7%) patients on azathioprine, 4/51 (7.8%) patients on Methotrexate as immunosuppressive (IS) inducing agent along with mean dose of 46.6ï'±13.7(0.86ï'±0.23 mg/kg/day) prednisolone equivalent. Rituximab and IVIg was used in 2 patients each. At 3 months after initiation of immunosuppression 26/50(52%) patients under follow-up had attained remission and 39/47(82.9%) patients attained remission at 6 months on follow-up. Mean time to achieve remission was 151.1ï'±86.3 days. All IS agents were equally effective in inducing remission. After a mean follow-up of 1124.3ï'±912.8 days, 47/54 (87.1%) patients were in remission with 7 (11.2%) deaths noted in the cohort at their respective last visit over a period of 195.3 patient years. 4/50 (8%) patients had relapse of vasculitic symptoms. The 2 and 5 year survival rate was 96.2% and 83.9% respectively. The 2 and 5 year mortality rate was 3.8% and 16.1% respectively.

Conclusions: Our cohort of Asian Indian RV patients are comparitively younger with lesser RA duration, less of ever-smokers, higher PNS involvement with better survival/mortality rates compared to published literature. All IS agents showed equal rates of BVAS remission and BVAS reduction at 3 and 6 months of treatment.


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Frequency of amyloidosis in a cohort of Egyptian rheumatoid arthritis patients without proteinuria and disease duration more than five years

Hassan Bassiouni, Ahmed El-Atrebi1, Kaled Zaky, Hoda Abou-Gabal2; Department of Rheumatology, Al Azhar University, 1National Institute of Locomotory System, 3Department Clinical Pathology, Ain Shams Univ, Cairo, Egypt

Background: Amyloidosis is a serious complication of Rheumatoid Arthritis (RA) that should be detected as early as possible to avoid its morbidity and mortality.

Objective: To detect amyloidosis in a cohort of patients with RA without proteinuria and a disease duration more than 5 years.

Methods: RA patients seen between October 2013 and August 2014 were evaluated for the study. Those with five years disease duration were included in the study and those who had proteinuria, serum creatinine >1.5 mg/dl or disease onset before the age of 16 years, were excluded ending with thirty patients (23 women,7 men) who were eligible. Clinical and laboratory findings, imaging study results, and treatment were recorded for each patient. Abdominal fat aspiration biopsy (AFAB) was performed on all eligible patients. Amyloid deposits were identified by polarized light microscopy after Congo red staining. Informed consent was obtained from all patients. Clinical disease activity was scored according to DAS. Serum amyloid A protein (SAA), CRP and RF were measured by ELISA.

Results: AFAB showed amyloidosis in 4 (13.3%) patients out of thirty. The amyloid deposits were (1+) in 1 patient and (2+) in 3 patients. RA duration was longer in amyloid rather than the non-amyloid patients (12.50 years versus 6.15years) with a highly significant correlation (P< 0.001). Extra-articular manifestations were present in 50% of the amyloid patients and in 15.3% of the non-amyloid patients demonstrating a significant value in Amyloid patients (P< 0.01). DAS 28 score was higher in amyloid patients compared to the non-amyloid (P< 0.001). No association was found between amyloid and non-amyloid patients regarding age, sex, or deformities. Serum amyloid A was significantly higher in amyloid patients (P< 0.001) in contradiction to hemoglobin level which was found to be significantly lower in amyloid patients (P< 0.001). Conclusion: The prevalence of subclinical amyloidosis by AFAB was (13.3%).

Conclusion: AFAB sensitive for detecting subclinical amyloidosis. A simple screening tool such as AFAB should be used, particularly in patients with risk factors. Subclinical amyloidosis requires close monitoring to ensure the early detection and treatment of symptomatic amyloidosis.


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Clinical response to tocilizumab in Indian patients of seropositive rheumatoid arthritis: A prospective observational study

Abhishek Kumar, D S Bhakuni, K Shanmuganandan, V Vasdev, A Hegde, A T Atal; Army Hospital Research and Referral, Delhi, India

Background: IL-6 receptor antagonist TCZ is newer addition to the list of biological DMARDs being used for treatment of RA in India. There is not enough data available in literature at present on its efficacy and safety in Indian patients. Objectives: (a) To study the clinical response to IL-6R antagonist therapy with Tocilizumab (TCZ) in patients of rheumatoid arthritis as measured by DAS28. (b) To study the safety profile of TCZ in Indian patients.

Methods: This was a prospective observational study carried out over a period of 18 months. Seropositive RA patients with inadequate response to at least two conventional DMARDs were selected for the study. Baseline patient data, including patient demographics, clinical and laboratory parameters to assess disease activity was recorded. Disease activity was measured by DAS28 scores and functional capacity was assessed by Indian HAQ-DI. Patients were administered Inj Tocilizumab 8mg/kg body weight on baseline and at 04 weeks interval for total 06 doses. Paired t-test (for continuous variables), was used to analyze the change or difference in DAS-28 score and HAQ-DI scores at various time points between baseline to 6 months.

Results: Total 34 patients participated in the study. At 24 weeks there was statistically significant reduction in DAS28CRP from baseline value of 5.3 to 1.8 (∆ 3.5, p value <0.005). There was significant improvement in disability index after the first dose of TCZ as indicated by reduction in HAQ-DI from baseline value of 1.6 to 0.39 (∆ 1.21, p value <0.001) at 24 weeks.

Conclusions: Tocilizumab therapy at 8mg/kg body weight dose results in rapid decline in disease activity as well as levels of acute phase reactants and improvement in quality of life index.


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Assessment of traditional risk factors and subclinical atherosclerosis in patients with rheumatoid arthritis

Geeta Kumari, Rajnish Avasthi, Amitesh Aggarwal, Rafat S Ahmed, Vijay Gandhi, Shuchi Bhatt, Vinita Batra; Department of Medicine, University College of Medical Sciences, Delhi University, Delhi, India

Background: Cardiovascular (CV) diseases have become the leading cause of mortality in patients with rheumatoid arthritis (RA). Accelerated atherosclerosis is one of the most important mechanisms implicated in increased CV mortality. Carotid intima media thickness (CIMT) and flow mediated dilation (FMD) of brachial artery are well established markers of subclinical atherosclerosis in patients with Rheumatoid Arthritis (RA). The role of coronary artery calcium score (CACS) is still being worked, which is class IIa recommendation in asymptomatic patients.

Objectives: Assessment of traditional risk factors and subclinical atherosclerosis in patients with RA.

Methods: Twenty-five patients diagnosed with RA were evaluated for the presence of traditional risk factors for CV diseases. Endothelial dysfunction was assessed by FMD of brachial artery, and also ultrasonographic measurement of CIMT was done. Coronary artery calcium score was calculated using MDCT.

Results: The mean age of our study population was 40±8.0 years with majority (92%) of female patients. Dyslipidemia and obesity were most prevalent among the cardiovascular risk factors. Low HDL was found in 84% of RA patients. Obesity was found in 76% patients, whereas only 17.3% of patients were obese with respect to BMI >25kg/m2 as criteria for obesity. The mean FMD (%) in patients with RA was 5.88 ± 2.1%, with abnormal FMD (<6%) in 15/25 (60%) patients. The mean CIMT in patients with RA was 0.65±0.09 mm, with abnormal CIMT in 18/25 (72%) patients (considering upper limit of normal CIMT as 0.57 mm). MDCT detected calcium in 5/25 (20%) of patients, all of which were in low risk category. There was no patient with CACS >10.

Conclusion: CIMT and FMD seem to be promising investigations for the assessment of subclinical atherosclerosis in young to middle aged RA patients with a low CV risk. The debate on role of CACS in risk stratification of RA patients in particular and those with low to intermediate risk in general continues with various inconclusive treatment guidelines so far.


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Correlation of health assessment questionnaire (Indian version) with disease activity in rheumatoid arthritis

H Singh, N Kumar, J Singh, B Kiran, M Mangala, R Mathur; Department of Medicine and Rheumatology, Pt BDS PGIMS, Rohtak, Haryana, India

Background: Rheumatoid arthritis (RA) is a chronic inflammatory disease likely to be affecting activity of daily life requiring chronic DMARD therapy. Disease Activity Score using 28 joint count (DAS28) and Clinical Disease Activity Index (CDAI) are commonly used to assess the disease activity and therapeutic responses in RA. However patient related Health Assessment Questionnaire could be an alternative to the traditional disease activity score. Indian Health Assessment Questionnaire is available and can used to assess disease activity.

Objective: To assess the correlation (if any) of disease activity and therapeutic responses using DAS28 and CDAI and Health Assessment Questionnaire (Indian version).

Methods: 50 patients of RA as per 1987 ACR criteria were assessed for disease activity at base line (M0) and at three months (M3) follow up using DAS 28 and CDAI and Health Assessment Questionnaire (Indian Version). The correlation between the Health Assessment Questionnaire (Indian Version) and the traditionally used scores DAS 28 and CDAI were assessed for disease activity and therapeutic responses.

Results: Mean age (year) for the study group was 44.8 with male:female ratio 1.3:6. The mean value of disease activity score at M0 and M3; 58 and 4.54 using DAS28; and 27.96 and 17.67 using CDAI respectively. The mean value of Health Assessment Questionnaire (Indian Version) score at M0 and M3 was reduced from 1.92 to 1.24 correlating to therapeutic response and disease activity scores. The spearman’s correlation coefficient between Health Assessment Questionnaire (Indian Version) score and DAS28 was 0.981 and 0.974 at M0 and M3 (P < 0.001) while it was 0.976 and 0.946 (P < 0.001) at M0 and M3 between Health Assessment Questionnaire (Indian Version) score and CDAI.

Conclusion: Health Assessment Questionnaire (Indian Version) has a strong correlation to DAS28 and CDAI for the assessment of disease activity in RA.


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Comorbidities in rheumatoid arthritis in comparison with psoriatic arthritis â€" study from a tertiary care center

V A Sowndhariya, T N Tamilselvam, J Euphrasia Latha, S Balameena, M Saravanan, V Sivakumar, R Anuja, M Rajavel; Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: The term ‘Inflammatory arthropathies’ is misleading as inflammation is not limited to the joints but also involves heart and blood vessels.

Objectives: This article is concerned with comparison of comorbidities between Rheumatoid arthritis (RA) and Psoriatic arthritis (PsA).

Methods: This is a single centre, cross sectional study comparing cardiovascular (CV) and other comorbidities between psoriatic and rheumatoid arthritis. The study included consecutive patients with rheumatoid arthritis (1987 ACR criteria) and psoriatic arthritis (CASPAR criteria) between January 2015 to may 2017. Demographic characteristics and accompanying diseases were recorded.

Results: One hundred and seventy nine patients with rheumatoid arthritis (15 males and 164 females, mean age 45.4 years) and sixty two patients with psoriatic arthritis (44 males and 18 females, mean age 39.7 years) were included. Comorbidities were found in 64.2% and 64.5% of patients with rheumatoid arthritis and psoriatic arthritis respectively. There was no significant difference in the cardiovascular comorbidities like diabetes mellitus, hypertension, metabolic syndrome, dyslipidemia between the two groups after adjusting for age, sex and smoking. Among other comorbidities, hyperuricemia (odds ratio 3.002, 95% CI 1.25-7.20) and transaminitis (odds ratio 4.17, 95% CI 1.50-11.59 p<0.0001) were seen commonly in psoriatic arthritis, and there was no difference in depression between the two groups. There was no significant difference in the mean Charlson comorbidity index between the groups (RA vs PsA 1.66 vs 1.54 p=0.4).

Conclusion: While there is no difference in the cardiovascular comorbidities between rheumatoid and psoriatic arthritis, psoriatic arthritis is typified by the presence of hyperuricemia and transminitis.


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Unrecognised malady of depression in rheumatoid arthritis

A Aravindan, T N Tamilselvam, J Euphrasia Latha, M Vignesh, Mythili Seetharaman, C Saranya; Institute of Rheumatology, MMC, RGGGH, Chennai, Tamil Nadu, India

Background: Depression is rarely acknowledged as a serious co-morbidity in rheumatoid arthritis (RA).

Objective: To find the occurrence of depression in RA patients attending rheumatology opd in a tertiary care institute and correlate with disease variables.

Methods: A cross sectional study was conducted among patients attending our OPD, registered between 2013 and 2017. Inclusion criteria: Patients fulfilling the 2010 ACR/EULAR classification criteria for RA. Exclusion criteria: Pregnant and lactating mothers, children <16 years, chronic disease or treatment, patients with psychiatric illness. After obtaining consent, patients were examined clinically; disease activity (DAS 28 CRP and DAS 28 ESR) was estimated. The screening for depression was done by using validated Tamil version of questionnaire PHQ9, with cut off score of >14. The results were analysed statistically.

Results: The Study population was 180. Males were 29 (15.5%) and a female, 151 (84.4%).The median age was 40 + 10.78. The mean disease duration was 6.96±5.27. The mean PHQ9 score was 17.56±5.66. Among males, 14 patients (48.3%) had depression, while 15 (51.7%) had no depression. Among females, 107 (70.8%) patients had depression, while 44 (29.1%) had no depression. Among 59 non-depressed patients, 24 (40.67%) were in mild/remission category and 35 (59.32 %) had moderate/ high disease activity as per DAS 28 ESR. Among 121 depressed patients, 50 (41.32%) were in mild/remission category and 71 (58.67%) had moderate/high disease activity. The correlation with DAS 28 CRP, revealed 82 (67.76%) depressed patients in mild/remission category and 39 (32.23%) in moderate/ severe disease category. In patients with no depression, 48 (81.35%) were in mild/remission category and 11 (18.64%) were in moderate /severe category. The overall prevalence of depression was 66.85 %. Pearson correlation co-efficient and p value calculated for variables. (Age, education, disease duration, ESR, CRP, RF, anti-CCP).

Conclusion: Depression was common in both categories of mild/remission and moderate/ high disease activity. There was no statistically significant correlation with age, gender, education, disease duration, treatment duration, ESR, CRP, disease activity, RF and anti-CCP. This study shows, screening for depression is mandatory. The significance of screening for depression would go a long way in addressing the needs of the patients, in addition to specific therapies for RA.


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Palindromic rheumatism-early RA or a different phenotype?

S Pandya, R Solanki, T Parikh; Vedanta Institute of Medical Sciences and Sheth VS Municipal Hospital, Ahmedabad, Gujarat, India

Background: Palindromic rheumatism is believed to be a milder variant of RA and an early presentation. We at our center have observed PR remaining so for and not evolving into RA as is believed, most being seropositive for CCP antibody.

Aims: To study demographic, clinical and serologic features of patients of PR cases and compare them to those with established RA presenting during the same period.

Methods: This was a retrospective cross sectional analysis of data collected at our center. All cases presenting with PR (defined as pain, swelling and or redness in or around a joint lasting from hours to days - different joint area each time over 6 weeks) and RA (ACR 2010 criteria) from 9/1/2013 to 27/7/2017 were included in the analysis. Descriptive statistics was used to compare the demographic, clinical and serologic features between the two groups.

Results: Females were 85.8% and 72.1% in RA and PR respectively. In the PR group, 87.5% reported swelling, 40.9% overlying erythema and the pain (mean) VAS was 6.25/10 (10 severe) on presentation. Patients reported a mean of 11.6 attacks per month and mean duration of attack was upto 55.8 hours. Majority were treated with MTX + HCQ (64.7%). At a mean follow up of 10.46 months, none of them evolved into full blown RA [Table 1].



Conclusion: PR cases were younger, weighed more, presented earlier and had lower ESRs than established RA cases. Seropositivity was similar for both RF and CCP in the two groups. On our follow up none of them evolved into RA although mean follow up period is short. PR seems a different phenotype of RA rather than an early presentation of it. Diagnostic criteria need.


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Correlation of disease activity score 28 with anticcp levels in patients with rheumatoid arthritis

R Rana, V Arya, R S Taneja, M Ali, R Ramadoss, K Gupta; PGIMER Dr. RML Hospital, Guru Gobind Singh Indraprastha University, New Delhi, India

Background: Rheumatoid arthritis (RA), is the commonest rheumatological disorder seen in clinical practice. The “gold standard†index used to assess disease activity in RA has been the Disease Activity Score-28 (DAS28). The presence of Anti- Cyclic Citrullinated Peptides (Anti CCP) antibodies are found as highly specific and predictive for rheumatoid arthritis. In order to address any correlation between AntiCCP levels and Disease Activity, we conducted a study in patients with RA.

Objectives: To assess and correlate Disease Activity Score (DAS28) and Anti-CCP levels in patients with Rheumatoid Arthritis.

Methods: This was a cross-sectional observational study including 40 patients with RA fulfilling ACR/EULAR 2010 criteria and with AntiCCP positive status. Patients who had received DMARDs in the preceding 3 months and also who were on glucocorticoid equivalent dose of ≥5 mg prednisolone for ≥1 month were not included in the study. Laboratory parameters including ESR by Westergren’s method, IgM Rheumatoid Factor by ELISA, IgG Anti-CCP antibodies by 2nd Generation ELISA were done for all patients. Assessment of disease activity was done using DAS28 score and its correlation with antiCCP levels was seen by regression analysis using Stata 12 SE (Stata Corp LP, Texas, USA).

Results: The minimum value of AntiCCP levels detected were 5.35 IU/ml and a maximum of 337 IU/ml with a mean of 147.15 ± 116.37 IU/ml were found. Most patients had high disease activity by DAS28. Scores ranged from 2.93 to 7.25, with a mean of 5.26 ± 0.87. Univariate linear regression analysis with DAS28 as the outcome and AntiCCP titers as the predictor variable showed an R-squared= 0.1858 and a p-value of 0.0056 showing a significant correlation between the two [Figure 1].



Conclusions: In patients with rheumatoid arthritis who are positive for antiCCP antibodies, there is a direct, positive and statistically significant correlation between antiCCP titers and disease activity as measured by DAS28 score. If confirmed in larger studies, our results suggest that antiCCP antibody titers could supplement clinical assessment in the assessment of disease activity in patients with rheumatoid arthritis.


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Clinical profile of patients of patients of rheumatoid arthritis treated with conventional DMARDS: Experience from a private rheumatology clinic in Dehradun

Kamal Bhatt; Dehradun

Background: There is a need to collect data on RA patients achieving remission with conventional DMARDs as biologicals are still out of reach for the majority of patients.

Objective: To do a retrospective study of clinical profile of RA patients and find out remission rates in patients treated with conventional DMARDs with a special focus on Methotrexate.

Methods: Records of patients of RA who retrospectively fulfilled 2010 ACR/EULAR criteria1 between February 2009 and May 2017 were analyzed. Remission was defined as per Boolean based definition of 2011 ACR criteria2. Patients on daily or alternate day steroid were excluded from this definition. Student's t-test was used for statistical analysis.

Results: Records of 554 patients were analyzed. Female to male ratio was 5.9:1, mean age was 49 ± 12.1 years, mean disease duration was 60.45 ± 69.64 months, and mean duration of follow up was 32.67 ± 23.03 months. 260 patients (47 %) achieved remission, out of which 174 (31.5 %) had sustained remission while 86 (15.5 %) relapsed. Average disease duration in patients with remission (38.6 ± 48.13 months) was lower than in those without (79.9 ± 79.47 months). Average duration of follow up in patients who reached remission was longer (39.7 ± 22 months) than in those without (26.4 ± 22 months). The mean Methotrexate (MTX) dose was 17.85 ± 7.16 mg and 353 patients (65 %) tolerated ≥17.5 mg dose. The maximum tolerated MTX dose in patients with remission was slightly higher (18.81 ± 6.50 mg) than in those without (17.05 ± 7.55 mg), but statistically significant (p<0.05). The most common reason for drug withdrawal or dose reduction was GI upset in 99 patients (24 %). Fifty-seven patients (10.3 %) were given injectable MTX, out of which 24 (42 %) had better tolerance than oral. The MTX dose at which maximum number of patients reached and maintained remission was 17.5 mg.

Conclusion: About one third of patients achieved sustained remission with conventional DMARDs, especially those with lesser disease duration and regular long term follow up. Higher Methotrexate doses were well tolerated in about two-third of patients and significantly associated with remission.

References

  1. Aletaha D, Neogi T, Silman AJ, Funovits J, Felson DT, Bingham CO 3rd, et al. 2010 rheumatoid arthritis classification criteria: An American College of Rheumatology/European League Against Rheumatism Collaborative Initiative. Ann Rheum Dis 2010;69:1580-8.
  2. Felson DT, Smolen JS, Wells G, Zhang B, van Tuyl LH, Funovits J, et al. American college of Rheumatology/European League Against Rheumatism provisional definition of remission in rheumatoid arthritis for clinical trials. Arthritis Rheum 2011;63:573-86.



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To study the effect of methotrexate and prednisolone on tuberculin skin test in RA patients screened for latent TB: Implications on anti-TNF alpha therapy

Kriti Kishor1, Shefali K Sharma1, Varun Dhir1, Nusrat2, Sanjay Jain1; 1Unit of Clinical Immunology and Rheumatology, Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, 2Department of Pharmacology PGIMER, Chandigarh, India

Background: Tuberculin skin test has been used as an indicator of latent TB. Patients of RA are treated with multiple DMARDS and often steroids. The effect of these drugs on tuberculin tests unclear. This study aims to find the prevalance of positive TST in treatment naive RA patients and those on treatment with methotrexate alone or in combination with steroids. The effect of DMARDS with or without steroids have important implication on TST prior to treatment with biologicals.

Methods: Tuberculin testing was done using Monteaux method. 5 TU of PPD (0.1 ml) was injected intradermally on flexor aspect, left forearm. Results were read by measuring the horizontal induration at 72 hours. >10 mm were taken as positive and <5mm negative.

Results: 200 RA patientsand 60 controls were included in the study. Study population had treatment naïve patients(n=115, 57.5 %), patients on 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 naive population (n=26,22.6%) as compared to healthy controls(n=20, 33.3% statistically insignificant(p=0.101).There were more no of tuberculin positive cases amongst the methotrexate group (38.6% n=22) as compared to those who those receiving steroids and methotrexate both (11.6% n=6); statistically not significant (p=0.065). The median duration of treatment was 3.5 months (IQR 1.25-6) in methotrexate only group and 3 months (IQR 3-5.7) in methotrexate plus steroid group. Tuberculin positivity was not influenced by disease activity, dosage of methotrexate, BCG vaccination, or presence of co-morbid conditions not requiring immunosuppression.

Conclusion: Tuberculin positivity was low in treatment naive RA patients. Treatment with methotrexate or steroids does not improve anergy to tuberculin. Mantoux positivity is not affected by BCG vaccination if the test is done >15 years after receiving vaccine. The mechanisms by which steroids and DMARDS alter response to tuberculin antigen need to be studied elaborately.


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Poor compliance, despite early treatment initiation, poses major challenge to rheumatoid arthritis care in India: Paradigm shift over 5 years

Sanjana Joseph, Devika Mohan, Nissy Bejoy, Sreelakshmi Sreenath, Padmanabha Shenoy; Centre for Arthritis and Rheumatism Excellence, Cochin, Kerala, India

Background: Early diagnosis of Rheumatoid arthritis and early initiation of DMARDs is important in order to improve long-term outcome.

Objective: To analyze whether diagnostic and treatment delays in Rheumatoid Arthritis has changed over 5years in India.

Methods: Adult patients diagnosed with RA according to ACR criteria, visiting the outpatient rheumatology clinic for the first time were included in the study. Data regarding the treatment patterns was collected using a standardized data collection form in 2012 and 2017.Data was compared to analyze the change in trends.

Result: 323 consecutive patients visiting an outpatient rheumatology unit for the first time were interviewed in 2012 and same procedure was repeated after 5years. In RA patients, diagnostic delay decreased from 55months in 2012 to 22 months in 2017 (p<0.005). Likewise, delay in initiation of DMARDS decreased from 66 months to 24 months from symptom onset (p<0.0001). It was found that 91% patients frequently change systems of medicines. The number of patients who consulted modern medicine doctors first after symptom onset increased significantly from 54.18% to 62.54% (p-0.03). Among them, 60% doctors made a wrong diagnosis in 2011 and similar number (50.99%) in 2017 also (p-ns). The number of patients exposed to DMARDs at the time of enrollment into study has significantly increased from 44.58% in 2011 to 84.21% in 2017 (p<0.0001). But among the patients who were initiated on DMARDs, 85% stopped them despite having a good self-reported outcome. The percentage of patients defaulted due to fear of side-effects significantly increased from 49.31% to 60.66% (p-0.02) over 5years.

Conclusion: In a developing nation, despite significant improvement in early diagnosis and initiation of treatment, majority of patients default due to fear of side-effects. Patient education strategies addressing this is the need of the hour.


  PC094 Rheumatoid Arthritis Top


Pulmonary involvemnent in rheumatoid arthritis: A retrospective analysis from a tertiary care, teaching institute in South India

Anupama Mundu, John Mathew, Debashish Danda; Department of Clinical Immunology and Rheumatology Christian Medical College, Vellore, Tamil Nadu, India

Background: Pulmonary involvement in RA includes parenchymal, pleural, airway and vascular diseases. Interstitial lung disease (ILD) has been reported in up to 30% of RA patients.

Objectives: To identify and describe the pattern of lung involvement by imaging in a cohort of in-patients with RA.

Methods: All patients with RA (1997 ACR criteria), admitted under Clinical Immunology and Rheumatology in CMC, Vellore from January 2014 to September 2016, and who had undergone either high resolution computed tomography (HRCT) or CT thorax were included.

Results: Pulmonary abnormalities were observed in 134 out of 286 patients (46.8%) with mean age of 55 ± 11.4 yrs and female:male 105:29. ILD was reported in 37 patients (27.6%) â€" NSIP in 21 (56.7%), UIP in 4 (10.8%), fibrotic NSIP/UIP in 2 (5.4%), early ILD in 7 (18.9%) and unclassified ILD in 3patients (8.1%). Among 20 patients with NSIP in whom rheumatoid factor (RF) was done, 11 (55%) had high titres RF (>100IU). All 4 UIP patients also had high RF positive. Other common findings included mild bronchiectatic changes in 38 patients (28.3%), atelectasis in 24 (17.9%), emphysematous changes in 7 (5.2%), advanced bronchiectasis in 1 patient (0.74%), peribronchial thickening in 15 (11.1%), bronchial thickening in 3 (2.23%) and pleural thickening in 40 patients (29.8%). Lung nodules were reported in 34 patients (25%). Lung haemorrhage in 1 (0.74%) and lung malignancy were found in 1 patient (0.74%).In 37 ILD patients 54% were inititated on mycophenolatemofetil, 18.9% on azathioprine and 29.7% were given Rituximab for indications other than ILD.11 patients(29.7%) were not on any second line agent.

Conclusion: In our cohort, ILD was seen in 27.6% of patients. Most common pattern of ILD was NSIP, which was associated with high titre RF.


  PC095 Rheumatoid Arthritis Top


Novel concept of vascular age in Rheumatoid arthritis

Ashit Syngle1,2, Nidhi Garg3, Sudeep Kaur1, Tanya Syngle4, Vijaita Syngle1; 1Cardio-Rheuma and Healing Touch City Clinic, 4Healing Touch Foundation, Chandigarh, 2Fortis Multi Specialty Hospital, Mohali, 3Chitkara College of Pharmacy, Rajpura, Punjab, India

Background: Cardiovascular disease is the leading cause of death in RA. Vascular age (VA) is an alternate means of representing cardiovascular risk. Systemic inflammation is major driver for excess cardiovascular morbidity and mortality in RA. However, little information exists on relationship between vascular age and disease specific risk factors in Indian RA patients.

Objective: To investigate VA in RA and its relationship with disease specific risk factors.

Methods: 38 RA patients fulfilling the 2010 Rheumatoid Arthritis classification criteria were recruited without CV risk factors and matched to 25 healthy age and sex matched controls. VA was calculated with help of vascular age chart according to Systematic Coronary Risk Evaluation (SCORE). As surrogates for disease activity, DAS28, CRP and ESR levels were determined.

Results: In RA, VA (65.75+1.01) was significantly more than the chronologic age (49.43±9.55 yrs). Compared with healthy controls, RA patients had significantly (p<0.05) increased levels of DAS 28, ESR and CRP. Vascular age was found to be more in RA patients than healthy controls. Significant correlation was found between VA and CRP (r=0.43, p<0.01) and disease duration (r=0.46, p<0.01) in RA.

Conclusion: Vascular age in Indian RA patients is more than the chronological age and correlates with CRP and disease duration. Longer disease duration and high levels of inflammatory activity contribute to a proinflammatory microenvironment that facilitates both the development of vascular dysfunction and promotes atherosclerosis. Early and aggressive treatment of RA would help to prevent CV risk in RA.


  PC096 Rheumatoid Arthritis Top


Disease activity status of patients with rheumatoid arthritis in follow-up in a busy Rheumatology Outpatient Department: A cross sectional study

Q Zaheer, V Aggarwal, S S Baghel, A N Malaviya; ISIC Hospital, New Delhi, India

Introduction: Early and intensive treatment by a combination of conventional synthetic DMARDs (csDMARDs) or biological DMARDs (bDMARDs) for RA seems to be the key to achieving better remission rates. Tight control of the disease activity has shown reduced radiographic progression, physical function, and quality of life. The remission rates all over the world are improving with 'Treat-to-Target' (T-2-T) principle. In India, the use of DMARDs is inexplicably low 1,2.

Aim: To find out the disease activity status of patients with RA follow-up in routine rheumatology OPD clinic.

Methods: 361 consecutive seropositive RA patients were taken from 1st January 2015 to 30th September 2015. Their demographic characteristics, Simplified Disease Activity Index (SDAI) and treatment at the last visit was taken. All the patients were started on tapering low dose glucocorticoids (GC), escalating methotrexate (MTX) and hydroxychloroquine (HCQ). Patients who do not go into remission or continue in low disease activity state after 3 - 6 months were additionally given leflunomide (LFN) or sulfasalazine (SSZ).

Results: No of patients: 361. Mean age at presentation: 52.15 yr. Male:female54:307 (1:5.68) Clinical presentation: Early- 34 (9.4%), Established- 60 (16.6%), Late- 267 (74%) SDAI BASELINE LAST VISIT Remission 43 (11.9%) 281 (77.8%) Low Disease Activity 35 (9.7%) 45 (12.5 %) Moderate Disease Activity 122 (33.8%) 33 (9.1%) High Disease Activity 161 (44.6%) 2 (0.5%) Total no of patients on 2 csDMARDS: 200 (55.4%) Total no of patients on 3 csDMARDS: 161 (44.6%) 4 (0.011%) and 15 (0.41%) patients on bDMARDs currently and earlier, respectively.

Conclusion: It is possible to achieve a reasonably good remission or low disease activity rates with double or triple csDMARDs in the majority of our RA patients. There is a need for better csDMARDs usage in India.

References

  1. Vij AS, Malaviya N, Kumar S. Characteristics of rheumatoid arthritis patients at first presentation to a specialized rheumatology department. Int J Res Med Sci 2015;3:2073-8.
  2. Malaviya AN, Gogia SB. Treatment of rheumatoid arthritis (RA) in India-how and by whom: Results from a speciality clinic-use of low-dose methotrexate (MTX) was inexplicably suboptimal. Clin Rheumatol 2016;35:2163-73.



  PC097 Rheumatoid Arthritis Top


Treat to target approach in rheumatoid arthritis: Are we able to achieve target outcomes in patients with RA in public sector hospitals: A study from western India

Nibha Jain, Sapan Pandya, Puja Srivastava; Department of Rheumatology, VS Hospital, Ahmedabad, Gujarat, India

Background: Achieving low disease activity or remission is the primary end point for patients with Rheumatoid Arthritis (RA), according to treat to target approach. However, with financial constraints and limited use of biological DMARDs, this goal is difficult to achieve at many public hospitals in India and other resource poor countries.

Objective: Assess the EULAR response at 6 months follow up in patients of RA, treated with conventional DMARDs.

Methods: Patients with RA having at least 6 month follow up were included in the study, from Sept 2016 to July 2017, after informed consent. Their clinical data was recorded in pre-specified proforma.

Results: Total of 55 patients were included (F:M ratio: 3:1). Methotrexate was the most common DMARD used, 53% received two drugs, while 41% patients received triple therapy [Table 1]. Overall, 65% of patients achieved EULAR good response on Conventional DMARDS alone, and 20 % achieved remission.



Conclusion: Two-third patients achieved EULAR good response with the use of conventional DMARDs alone which is comparable to other Indian and international studies where biologics have been used.


  PC098 Rheumatoid Arthritis Top


Correlation of disease activity score 28 with neutrophil lymphocyte ratio and platelet lymphocyte ratio in patients with rheumatoid arthritis

K Gupta, R Ramadoss, V Arya, R; Rana PGIMER Dr. RML Hospital, GGSIPU, New Delhi, India

Background: Goal directed Therapy in RA involves repeatedly assesing and controling disease activity for which we require precise and affordable indices that could be calculated easily on OPD basis. Studies have shown that most rheumatologists do not objectively assess disease activity in patients with RA at most visits may be due to the complex formulas involved or the burder of serological investigations required like ESR, CRP. Establishing NLR and PLR as markers of active disease in RA could solve the said problems. A few recent studies have compared disease activity in patients with RA as reflected by the DAS-28 with NLR and PLR with some studies finding a positive correlation between DAS28 with NLR or PLR and other studies failing to find any such correlation. No such study has been conducted in the Indian population.

Objectives: To assess and correlate Disease Activity Score (DAS 28) and Neutrophil â€" Lymphocyte ratio (NLR) and Platelet - Lymphocyte ratio (PLR) in patients with rheumatoid arthritis.

Methods: This was a cross sectional study in New Delhi with 40 cases and 40 controls. Inclusion and exclusion criteria were applied. All patients were evaluated for various demographic and laboratory parameters for calculation of DAS28, HAQ, ESR and a hemogram including a DLC done by manual count. NLR and PLR were calculated for both cases and controls and compared using appropriate statistical methods. Their correlation was also compared with disease activity as assessed by DAS28 in patients with RA.

Results: The mean NLR in cases and controls was 2.78 + 1.44 and 2.11 + 0.59, respectively. The mean value of PLR in cases and controls was 142.86 + 61.71 and 97.66 + 25.97 respectively.

Conclusion: ESR, NLR and PLR were significantly higher in patients with RA when compared with healthy individuals (p value = 0.0003, 0.0079, 0.0001 respectively). However, a correlation of NLR and PLR with disease activity as assessed by DAS28 and HAQ seperately in patients with RA was not found. Hence it was concluded that NLR and PLR are new biomarkers for RA that could be used to differentiate RA from healthy individuals.


  PC099 Rheumatoid Arthritis Top


Study of serum lactate dehydrogenase as a disease severity marker in rheumatoid arthritis

(IJR UG Fellowship- 2017)

Patel Dhruvkumar Mukundkumar, Mukund V. Patel, Reena Sharma; Smt. NHL Municipal Medical College, Ahmedabad, Gujarat, India

Background: Lactate dehydrogenase is released during tissue injury and inflammation. Serum LDH is also raised in certain anaemias, liver disease, acute myocardial infarction and different malignancies. Raised serum LDH level are linked with progressive joint damage in patients with RA. LDH is released from dead and damaged cells into the synovial fluid and spread via the lymphatic from all inflamed joints into blood circulation. Serum LDH level reflect as measure of joint inflammation. So serum LDH level might predict the progression of joint damage.

Objectives: To study S.LDH as a biochemical marker of Rheumatoid arthritis by comparing it with clinical disease activity index (CDAI), DAS28 CRP, Anti CCP antibody and rheumatoid factor titre.

Methods: We have done prospective study of total 49 patients of rheumatoid arthritis as per ACR 2010 criteria from 15 April to 15 July 2017. We have excluded patients having comorbid conditions like megaloblastic anaemia, malignancies, liver failure, cardiac failure, renal failure, severe osteoporosis, hypothyroidism and age below 18 years. After taking consent, detail clinical history and appropriate investigations, CDAI and DAS 28 CRP was calculated. Patients were categorised into different disease activity and S. LDH was measured. This protocol was repeated on every 30 day follow up visit. S.LDH more than 3 times of mean normal value (288 IU) was considered as significantly raised.

Results: Out of total 49 patients 32 (65.31%) were female and 17 (34.69%) were male. Mean age of the patient was 49.35 +- 7.75. As per CDAI Score, 22 (44.90%) patients were having moderate disease with Mean S.LDH of 485.03 IU and 27 (55.10%) patients were having severe disease with mean S.LDH 418.63 IU. As per DAS 28 CRP index 33 (67.35%) patients were having moderate with mean S.LDH 445.37 IU and 16 (32.65%) patients were having severe disease with Mean S.LDH 454.35 IU.

Conclusion: The aim of present study was to establish role of S.LDH as a disease severity marker of RA as it is released from inflamed joint tissue. Our study shows that S.LDH was not raised to significant level in moderate or severe RA so S.LDH is not a useful biochemical marker of RA.


  PC100 Rheumatoid Arthritis Top


A study on adherence to methotrexate therapy in patients with rheumatoid arthritis and to identify the factors influencing the compliance of methotrexate

Mohan Rajavel, Thiruchengode Natesan Tamilselvam, Selvakumar Balameena, Euphrasia Latha, Myilsamy Saravanan, Sowndhariya Velu Annamalai, Sivakumar, Anuja Rajan.; Postgraduate Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Methotrexate (MTX) remains as sheet anchor in the therapy of RA even today in the era of biologics. Better understanding of reasons for non- adherence may aid in developing strategies to increase overall adherence to MTX therapy.

Objective: To determine and identify adherence to MTX therapy in patients with rheumatoid arthritis and to determine the factors that promote either adherence or non- adherence.

Methods: This was a cross sectional observational study, conducted at a tertiary care center out patient's clinic over a period of 10 months from September 2016 to June 2017. A total of hundred patients satisfying 2010 ACR/EULAR classification criteria for rheumatoid arthritis who were started on methotrexate either alone or along with other DMARDs were included in this study and followed regularly. Details such as demography of the patients, duration of MTX intake, current dose of MTX, Disease activity score at the current visit, concomitant drugs taken, number of doses of MTX missed in the previous 8 weeks, side effects due to MTX and factors that promoted either adherence or non-adherence to MTX therapy were recorded.

Results: Mean age was 48 ± 9.9 years. Females were 89 %. Overall, non- adherence to MTX rate was 19 %. Age, gender, level of education, duration of RA, current dose of MTX, concomitant use of other DMARDs, NSAIDs and steroids were not significantly different between adherent and non adherent patients. Patients of lower socioeconomic class had statistically significant non-adherence rate (P value < 0.015). Disease activity score (DAS 28 ESR) and side effects profile were not significantly different between the adherent and non-adherent patients. Adequate counselling by medical personnel contributed 43 % of MTX adherence. Intolerance to MTX and lack of awareness about the need and importance of MTX therapy, contributed 30% and 25 % of MTX non-adherence respectively.

Conclusion: Contrary to the common notion that Methotrexate toxicity leads to non-adherence, this study demonstrates that adequate counselling by trained medical personnel significantly improves compliance and efficacy. All patients on Methotrexate therapy, whether a part of combination immunotherapy or biological therapy need proper education to promote compliance.


  PC101 Rheumatoid Arthritis Top


A snapshot of cost of RA treatment in the private sector

P Prajapati, S Chikani, N Patel, A Parmar, R Shah, R Tank, N Shah, A Shukla, R Sharma, A Digole, S Deewanji, B Desai, P Srivastava, J Usdadiya, B Meghnathi, T Parikh, S Pandya;Vedanta Institute of Medical Sciences and Sheth VS Municipal Hospital, Ahmedabad, Gujarat, India

Background: Surprisingly few studies have been published on direct and indirect costs of RA treatment.

Objective: To study costs of treatment that our patients of RA coming to the private OPDs/corporate clinics incur.

Material and Methods: Ours was a prospective study. A short questionnaire which included demographic data of patients and details of the expense they incurred during a visit to the rheumatology OPDs in private practice chambers or corporate OPDs was sent to all participant investigators (14 in all). Study duration was from July 15th 2017 to Aug 15th 2017.

Results: While the total cost of monthly treatment was more than that done elsewhere in India before [Table 1] (997.05) (1), it was similar to other developing countries like Brazil, (approx. 2000 Rs) Mexico and much cheaper than Sweden and USA (approx. 16K in Rs) (2). As would be expected, significantly more had to be spent by New cases than follow up due to consultation and investigational charges. There were in group differences between the four cities (Surat, Vadodara, Ahmedabad and Rajkot) with travel expenses and consultation charges being more in the larger city Ahmedabad while medicinal charges were more in Surat. (A total of 4 patients were on biologic/biosimilar therapy of which 3 were from Surat and 1 from Vadodara).



Conclusion: The cost of treatment for RA patients per month (including consultation/travel/investigations and drugs) in a private set up is about Rs 2800 which is similar to other developing countries and much cheaper than developed countries.

References

  1. Syngle A, Kaur S, Verma I, Syngle T, Syngle V. Cost-effective analysis of disease-modifying anti-rheumatic drugs in rheumatoid arthritis. Clin Rheumatol 2017;36:1715-20.
  2. Griffiths RI, Bar-Din M, MacLean CH, Sullivan EM, Herbert RJ, Yelin EH, et al. Medical resource use and costs among rheumatoid arthritis patients receiving disease-modifying antirheumatic drug therapy. Arthritis Care Res 2000;13:213-26.



  PC102 Rheumatoid Arthritis Top


Pulmonary manifestations in rheumatoid arthritis

Satish Sharma, Jyotsna Oak; Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Mumbai, Maharashtra, India

Introduction: Rheumatoid Arthritis (RA) is a chronic systemic disease characterized by articular involvement, extra articular manifestations and the presence of rheumatoid factor. Pulmonary involvement in RA is a common extra articular manifestation of RA leading to significant morbidity and mortality.

Objectives: To study pulmonary manifestations in Rheumatoid Arthritis and their classification based on type, site and clinical outcomes.

Methods: We undertook the study to determine the prevalence and spectrum of pulmonary abnormalities in patients with RA, 114 patients of RA who met with ACR criteria durning period February 2016 to June 2017 of which 44 patients had pulmonary symptoms were subjected to clinical examination of chest X-ray, Pulmonary Function Tests and High Resolution Computerized Tomography HRCT.

Result: 44 out of 114 patients of RA (29.9%) had respiratory symptoms with exertional dyspnea (29.6 %), Cough with expectoration (41.3 %) and Fine Respiratory Rales (36.6 %). The X ray chest showed bilateral lower zone haziness (36.59 %) and prominent pulmonary vasculature (15 %). Amongst 38 patients who were subjected to pulmonary function test, obstructive pattern was found in 18.42 %, Restrictive pattern in 65.79 % and mixed pattern in 10.53 %. HRCT revealed Ground glass patch (38 %), Sub pleural reticulation(20%) Pleural thickening 20 % and pulmonary vasculature prominence (22%) [Figure 1]. In 43.9 % of cases both RF and Anti-CCP were positive.



Conclusion: It was observed that patients with high titers of Rheumatoid factor and both RF and Anti-CCP positive had more severe pulmonary manifestations. The patient of Rheumatoid Arthritis should be screened for pulmonary involvement, especially those who have serological markers positive.


  PC103 Rheumatoid Arthritis Top


Rheumatoid arthritis: New treatment

N Subramanian; Velammal Medical College Hospital and Research Institute, Madurai, Tamil Nadu, India

Introduction: Biological drugs and biosimilars have revolutionised the treatment of Rheumatoid arthritis with early remission and long term disease free survival. Biosimilars, developed in Asia, are affordable to our population, equally effective and achieve remission in rheumatoid arthritis.

Methods: Patients with rheumatoid arthritis who failed DMARDS were counselled to take Biologics/Biosimilars. All patients should satisfy ACR criteria for diagnosis and were screened for TB, and Hepatitis and Immunoglobulins checked prior to Rituximab. Biologic users in the Rheumatology centre in Velammal Medical College Hospital, Madurai and Chendhur Hospitals, Tirunelveli were reviewed (Jan 2015-May 2017). All had 3, 6 and 12 months review and are under regular review.

Results: Patients with RA and on bio DMARDS have been studied in this retrospective study. There were 38 patients M: F=9:29, 13 patients had CCP negative. Mean DAS 28 CRP score was 6.12, 3 months following biologics: biosimilars DAS 28 went down to 2.74. 3 patients had high disease activity despite drop in DAS by 1.2.We have used different biosimilars and biologics as per patient choice and disease extent. Drugs used in RA- Biosimilars Biosimilar drug Intascept Exemptia Reditux RA Rituxrel Adfrar Mabtas No of patients 11 7 3 1 1 1 Mean duration of follow up was 12 months (3-20 months). Recent follow up showed good disease control with mean DAS 28-2.65, four patients became drug free. One had JIA. - Excellent results. All Ritux patients haven't needed retreatment yet. Two of Exemptia users had secondary failure. Mean Bio free remission period was 5.2 month (0-15 months). 9 patients are on biosimilars still. No specific infections had been reported except one had chicken pox after Rituximab. Those who had Biologics- Actemra had good response but due to costs patient stopped. Enbrel patients are using still under scheme. Ristova had good remission in RA.

Conclusion: This is the first of our Biosimilar experience in south India (Prelimary results are going to be presented in APLAR). Biologics and biosimilars achieve excellent disease control in 3 months and two patients had secondary failure. Drug free remission and Bio drug free remission are very much possible.


  PC104 Rheumatoid Arthritis Top


A study to compare the clinical features and laboratory findings of seropositive and seronegative cases of rheumatoid arthritis

Abhirup Sinha, Rathindra Nath Sarkar, Ritasman Baisya, Akashdip Bhattacharjee; Department of Medicine, Medical College, Kolkata, West Bengal, India

Introduction: Rheumatoid arthritis (RA) is a chronic inflammatory disease of marked by a symmetric, peripheral polyarthritis. RA patients who test positive in stated titres either for Rheumatoid factor (RF)/Anti-CCP antibody are classified as sero-positive and who test negative as sero-negative. This study aims to find out the comparison of clinical features and laboratory findings in both cases of Rheumatoid Arthritis.

Methods: It was cross-sectional study, conducted in the Rheumatology OPD of Medical College, Kolkata during a period of 1 year among 100 adult literate persons - 50 were diagnosed as seropositive RA (either RF and/or Anti-CCP antibody positive), other 50 patients were seronegative RA. Both groups were were evaluated regarding demographic profile, clinical evaluation, disease activity.

Result: Distribution of study population regarding joint deformity, Xray and extra articular manifestation showed significance among seropositive and seronegative patients (p significant). Tender and swollen joint count were not significant but ESR and DAS28 score were found to be clinically significant.

Conclusion: This study highlights that seropositive RA patients have higher disease activity, greater joint deformity, X-ray changes and extra-articular manifestations, it also demonstrates the higher preponderance of serious disease (detected clinically and laboratory findings) in seropositive RA patients in Eastern India.


  PC105 Rheumatoid Arthritis Top


Treat to target approach in rheumatoid arthritis: Are we able to achieve target outcomes in patients with rheumatoid arthritis in public sector hospitals: A study from western India

Nibha Jain, Puja Srivastava, Sapan Pandya; Department of Rheumatology, VS Hospital, Ahmedabad, Gujarat, India

Background: Achieving low disease activity or remission is the primary end point for patients with Rheumatoid Arthritis (RA), according to treat to target approach. However, with financial constraints and limited use of biological DMARDs, this goal is difficult to achieve at many public hospitals in India and other resource poor countries.

Objective: Assess the EULAR response at 6 months follow up in patients of RA, treated with conventional DMARDs.

Methods: Patients with RA having at least 6 month follow up were included in the study, from Sept 2016 to July 2017, after informed consent. Their clinical data was recorded in pre-specified proforma.

Results: Total of 55 patients were included (F:M ratio: 3:1). Methotrexate was the most common DMARD used, 53% received two drugs, while 41% patients received triple therapy [Table 1]. Overall, 65% of patients achieved EULAR good response on Conventional DMARDS alone, and 20 % achieved remission.



Conclusion: Two-third patients achieved EULAR good response with the use of conventional DMARDs alone which is comparable to other Indian and international studies where biologics have been used.


  PC106 Rheumatoid Arthritis Top


Disease activity status of patients with rheumatoid arthritis in follow-up in a busy Rheumatology Outpatient Department: A cross sectional study

Q Zaheer, V Aggarwal, S S Baghel, A N Malaviya; ISIC Hospital, New Delhi, India

Introduction: Early and intensive treatment by a combination of conventional synthetic DMARDs (csDMARDs) or biological DMARDs (bDMARDs) for RA seems to be the key to achieving better remission rates. Tight control of the disease activity has shown reduced radiographic progression, physical function, and quality of life. The remission rates all over the world are improving with ‘Treat-to-Target’ (T-2-T) principle. In India, the use of DMARDs is inexplicably low 1,2.

Aim: To find out the disease activity status of patients with RA follow-up in routine rheumatology OPD clinic.

Methods: 361 consecutive seropositive RA patients were taken from 1st January 2015 to 30th September 2015. Their demographic characteristics, Simplified Disease Activity Index (SDAI) and treatment at the last visit was taken. All the patients were started on tapering low dose glucocorticoids (GC), escalating methotrexate (MTX) and hydroxychloroquine (HCQ). Patients who do not go into remission or continue in low disease activity state after 3 â€" 6 months were additionally given leflunomide (LFN) or sulfasalazine (SSZ).

Results: No of patients: 361. Mean age at presentation: 52.15 yr. Male:female54:307 (1:5.68) Clinical presentation: Early- 34 (9.4%), Established- 60 (16.6%), Late- 267 (74%) SDAI BASELINE LAST VISIT Remission 43 (11.9%) 281 (77.8%) Low Disease Activity 35 (9.7%) 45 (12.5 %) Moderate Disease Activity 122 (33.8%) 33 (9.1%) High Disease Activity 161 (44.6%) 2 (0.5%) Total no of patients on 2 csDMARDS: 200 (55.4%) Total no of patients on 3 csDMARDS: 161 (44.6%) 4 (0.011%) and 15 (0.41%) patients on bDMARDs currently and earlier, respectively.

Conclusion: It is possible to achieve a reasonably good remission or low disease activity rates with double or triple csDMARDs in the majority of our RA patients. There is a need for better csDMARDs usage in India.

References

  1. Vij AS, Malaviya AN, Kumar S. Characteristics of rheumatoid arthritis patients at first presentation to a specialized rheumatology department Int J Res Med Sci 2015;3:2073-8.
  2. Malaviya AN, Gogia SB. Treatment of rheumatoid arthritis (RA) in India-how and by whom: Results from a speciality clinic-use of low-dose methotrexate (MTX) was inexplicably suboptimal. Clin Rheumatol 2016;35:2163-73.



  PC107 Spondyloarthropathy Top


Methotrexate achieves major cDAPSA response, improvement in dactylitis and functional status in psoriatic arthritis

Sravan Kumar Appani, Phani Kumar Devarasetti, Rajendra Vara Prasad Irlapati, Liza Rajasekhar; Nizam's Institute of Medical Sciences, Hyderabad

Background: Very few randomized or open label observational studies are available with methotrexate in Psoriatic arthritis (PsA) with limited efficacy.

Objectives: To study the effectiveness of methotrexate in PsA.

Methods: Patients satisfying CASPAR criteria for PsA were included in this open label, prospective, 9 months follow up study (June 2015 â€"October 2016) after obtaining informed consent and institutional ethical committee approval. Disease activity was assessed by tender (TJC) and swollen joint count (SJC), global assessment, DAS-28ESR, Clinical Disease Activity Index for Psoriatic Arthritis (cDAPSA), Leeds Dactylitis Instrument basic (LDI basic), Leeds Enthesitis Index (LEI), Psoriasis area and severity index (PASI) and HAQ (modified CRD Pune version). Response to therapy was assessed by EULAR DAS28 ESR response, DAPSA response, HAQ response and PASI 75 response. All patients received high dose methotrexate (≥15 mg/week), further dose titration and combination DMARDS (leflunomide or sulfasalazine) were added according to disease activity. NSAIDS were given continuously in acute dactylitis and enthesitis patients. Differences at baseline and follow up visits (3, 6 and 9 months) in disease activity parameters were analysed with Friedman test and Wilcoxon signed Ranks test. Difference in ESR between baseline and subsequent visit were analysed by paired sample test.

Results: Seventy three patients were included in the present study with mean (SD) age 44(9.7) years. The mean (SD) dose of methotrexate used was 17.5(3.8)/week. Seven patients received additional DMARDS (Leflunomide/Sulfasalazine). At end of study period significant improvement (p value <0.05) was noted in TJC, SJC, global activity, DAS-28ESR, cDAPSA, LDI basic, LEI, PASI and HAQ. Major cDAPSA response was achieved in 58.9% patients. EULAR DAS28 moderate and good response achieved in 74% and 6.8% respectively. PASI 75 response and HAQ response were achieved in 67.9% and 65.8% respectively.

Conclusion: High dose methotrexate (>15 mg/week) resulted in significant improvement in the skin, joint, dactylitis and function.


  PC108 Spondyloarthropathy Top


Thalidomide in the treatment of axial spondyloarthropathy: Single centre experience of 70 patients

Urmimala Bhattacharjee, Pallav Biswas, Chandan Kumar Das, Kaushik Basu, Rathindranath Sarkar; Medical College, Kolkata, West Bengal, India

Background: The therapeutic options for spondyloarthropathy were limited until the last decade which experienced the emergence of tumour necrosis factor (TNF) alpha inhibitors as potential therapeutic options in spondyloarthropathy. However in developing countries like India, cost and availability of TNF alpha inhibitors is a serious concern. Thalidomide can be a useful option in this regard as it has been found to reduce the activity of TNF-α.

Objective: In this study we evaluated the efficacy of Thalidomide in axial spondyloarthropathy patients.

Methods: A case series of 70 patients were selected in the age group 20-50 years, having disease duration 5 years or less. All patients were HLA-B27 positive and satisfied the ASAS criteria for axial spondyloarthropathy. All patients had ASDAS-CRP score >1.3 at study entry. All patients received thalidomide 50mg daily at bedtime for first two weeks and then 100mg daily at bedtime for one year. None of the patients received DMARD, steroid during the study period and were continued with the pre-trial dose of NSAIDs. All patients were evaluated at the end of 3,6,9 and 12 months using ASDAS-CRP, total spinal pain, duration of morning stiffness, patient global assessment and BASFAI parameters. Improvement if any was measured by ASAS20 and ASAS40 scores at each visit.

Results: Out of 70 patients, four patients withdrew and 66 completed the study. Thirty-six (54.5%) patients fulfilled ASAS20 improvement criteria by 3 months, 51 (77.2%) patients achieved this goal by 6 months, 58 (87.8%) patients achieved response by 9 months and 63 (95.5%) patients fulfilled ASAS-20 by 12 months. Only 3 patients were non-responders. ASAS40 improvement criteria were fulfilled in 23 (34.8%) patients in 3 months, in 35 (53.1%) patients at 6 months, in 42 (63.6%) patients at 9 months, and 47 (71.2%) patients at 12 months. There was significant reduction of ASDAS-CRP, BASFAI and duration of morning stiffness parameters at the end of 12 months compared to baseline.

Conclusion: Thus in resource poor countries, Thalidomide can be tried as an effective therapeutic option for treatment of axial spondyloarthropathy.


  PC109 Spondyloarthropathy Top


Non radiographic axial spondyloarthritis vs ankylosing spondylitis: Are they same!: A cross sectional study

Sham santhanam; Gleneagles Global Hospital, Chennai, Tamil Nadu, India

Introduction: The advent of MRI led to the new ASAS (Assessment of SpondyloArthritis international Society) criteria for the diagnosis of non radiographic axial spondyloarthritis (nr - SpA) in contrast to the conventional modified New York criteria for diagnosis of ankylosing spondylitis. We wanted to study whether both were different diseases or the same disease at different stages.

Aim: To study the clinical features, inflammatory markers, HLA B27 positivity and disease activity in patients with axial spondyloarthritis (SpA) and to find out any differences between the radiographic (ankylosing spondylitis) SpA and non radiographic axial SpA groups.

Methods: All patients who presented to the rheumatology outpatient department with features of inflammatory back pain were included. Study period: Jan2015 - Dec2016 Detailed history, routine clinical examination, inflammatory markers (ESR, CRP), HLA B27, X ray and MRI of pelvis and spine were done.

Results: One hundred and twenty eight patients with inflammatory back pain were screened and 114 of them fulfilled the ASAS criteria for axial SpA. 62 of them had non radiographic axial SpA (nr - SpA) and 52 had radiographic SpA (Ankylosing spondylitis - AS). In patients with nr - SpA, 27 had pure axial and 35 had both axial and peripheral joint symptoms. Ten had psoriasis, 5 dactylitis, 4 acute anterior uveitis and 2 inflammatory bowel disease. In AS patients, 32 had pure axial and 20 had both axial and peripheral joint symptoms. Thirteen had acute anterior uveitis, 5 psoriasis and 2 dactylitis. The demographic and clinical data (inflammatory markers, HLA B 27 and disease activity measures) of both the groups has been discussed in [Table 1]. On comparison, there was no statistically significant difference in inflammatory marker levels (ESR, CRP) or disease activity (BASDAI, BASFAI) between the groups. Median disease duration was more in ankylosing spondylitis group as expected and the incidence of HLA B27 positivity was slightly higher in AS group (57.69% vs 45.16%).



Conclusion: There was no major difference between the groups except for the increased median disease duration in radiographic SpA (AS) patients.


  PC110 Spondyloarthropathy Top


Factors affecting response to tnf blockers in patients with axial spondyloarthritis-experience from tertiary care centre

V A Sowndhariya, T N Tamilselvam, J Euphrasia Latha, S Balameena, M Saravanan, V Sivakumar, R Anuja, M Rajavel; Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Emergence of anti TNF therapies heralded the revolution in the management of patients with spondyloarthritis. Newer molecules targeting different cytokines are coming into vogue implying that there are a group of patients not responding adequately to anti TNF therapies.

Objectives: The main objective is to identify factors at baseline affecting desired response to anti TNF therapies.

Methods: A prospective observational study conducted at a tertiary care centre between September 2015 to May 2017. Demographic parameters, disease activity and functional index were recorded. Covariates included were age at onset, gender, smoking, disease duration, baseline ASDAS CRP, BASFI, C reactive protein, HLA B27. Multivariate regression analysis was used to identify the factors affecting the major improvement defined as ∆ ASDAS CRP≥2.

Results: A total of 102 patients who received either Etanercept 50mg/week for 12 weeks or Infliximab 3mg/kg infusion for 4 doses without interruption were included. 58.8% (60/102) of patients achieved major improvement in ASDAS CRP. Gender, smoking, body mass index, baseline disease activity and HLA B27 were not found to have significant influence on the outcome. Results with multivariate analysis have been shown in [Table 1].



Conclusion: Patients with younger age at onset, lower scores in functional index and raised inflammatory markers are more likely to respond in a better way to anti TNF therapies.


  PC111 Spondyloarthropathy Top


Efficacy of Secukinumab in patients with psoriatic arthritis: A single centre experience

Rachel Oommen, Dhanya Susan Abraham, Sanjana Joseph, Glindow Antony, Padmanabha Shenoy; Centre for Arthritis and Rheumatism Excellence, Cochin, Kerala, India

Introduction: Secukinumab is the first anti -IL17A therapy available in India to treat Psoriatic arthritis (PsA) and is a valuable addition to the available treatment options for PsA. Data on use of secukinumab in Indian PsA patients is scarce.

Objective: To analyse the efficacy and safety in patients of PsA treated with secukinumab

Methods: The PsA patients who fulfilled CASPAR criteria and showed inadequate response to conventional synthetic and/or biological DMARDs were treated with secukinumab. Secukinumab was given at a dose of 150mg subcutaneously at 0,1,2,3,4 weeks followed by every 4weeks. Patients were assessed based on relevant core outcome (TJC, SJC, ESR, CRP and BASDAI) variables for PsA. Patients were also monitored for adverse events. Data was collected retrospectively from electronic medical records.

Results: 16 patients (9 females and 7 males) with a mean age of 42 years were treated with secukinumab. 6 out of the 16 patients had failed on anti-TNF therapy. Mean duration of treatment being 28 weeks (Range 8 weeks to 72 weeks). Mean BASDAI decreased from 6.2 to 3.85 at 2 months. Most patients started improvement by the second week. At one month all patients achieved ACR20 response and 37.5% patients achieved ACR50 response. One patient developed severe depression at 8 weeks of therapy due to which therapy had to be discontinued. No other serious adverse events were observed.

Conclusion: Although this is a small and retrospective study, secukinumab appears to be very effective and safe in patients with PsA showing inadequate response to Cs DMARDs and anti TNF.


  PC112 Spondyloarthropathy Top


Extreme patient reported outcome in early spondyloarthritis: A surrogate for fibromyalgia? Its impact on tnf-alpha blockers treatment effect?

Bhowmik Meghnathi1,2, Adrien Etcheto1, Pascal Claudepierre3, Maxime Dougados1,2, Anna Molto1,2; 1Department of Rheumatology B, Paris Descartes University, Medicine Faculty; APHP, Cochin Hospital, 2INSERM (U1153): Clinical Epidemiology and Biostatistics PRES Sorbonne Paris-Cite, Paris, 3AP-HP, Groupe Hospitalier Henri-Mondor, Service de Rheumatologie, Universite Paris Est Creteil, Creteil, France

Objective: To describe the prevalence of extreme patient reported outcomes (PRO) in an early axial spondyloarthritis (axSpA) setting, to compare the phenotype of patients with/without extreme PRO and to evaluate the impact of extreme PRO on TNF alpha blockers (TNFb) effectiveness. Methods: This analysis was performed in the DESIR cohort of early axSpA. Extreme PRO were measured at baseline and were defined as a score ≥8 on at least three of first five BASDAI items (i.e. excluding morning stiffness duration). Phenotype of patient's with/without extreme PRO was compared. Impact of extreme PRO on TNFb effectiveness was evaluated by comparing the retention rate of the first TNFb in both groups by survival curves analysis (log-rank and Cox analysis).



Results: Extreme PRO were present in 95 out of the 708 patients (13.4%). Patients with extreme PRO were older (mean (SD) age of 35.4 (8.6) years vs. 33.5 (8.7) years) and more frequently females (65.3% vs. 51.9%), had higher BASDAI (7.1 vs. 4.1), BASG-week (8.0 vs. 4.7) and BASFI (51.1 vs. 27.2) scores, reported more frequently history of depression (25.3% vs. 10.2%) and use of anti-depressive drugs (19.0% vs. 7.2%). A TNFb treatment was more frequently initiated in the extreme PRO group (48.4% vs. 25.5%), while the proportion of patients still on TNFb at 2 years was significantly lower in the extreme PRO group 18.6 % (n = 8) vs. 39.5 % (n = 60). Presence of extreme PRO was independently associated with first TNFb discontinuation (HR 1.8, [95% CI 1.2; 2.9], p=0.01)).

Conclusions: Although presence of extreme PRO in this early axSpA setting was not very frequent, patients with extreme PRO were more likely to receive a TNFb and less likely to maintain the treatment at 2 years. Further studies evaluating the specific impact of FM on TNFb treatment in axSpA should confirm (or not) our findings.


  PC113 Spondyloarthropathy Top


Assessment of subclinical hand joint synovitis in patients with psoriatic arthritis by ultrasound and its relationship with clinical disease activity

Sumantro Mondal, Rudra Prosad Goswami, Debanjali Sinha, Geeta Bali Sircar, Parasar Ghosh, Alakendu Ghosh; Department of Rheumatology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

Background: Articular involvement in Psoriatic arthritis (PsA) can have diverse presentations; oligoarticular involvement is predominant in early disease. Recently, it has been shown that, Ultrasound (US) detected subclinical synovitis can be present in early PsA and a substantial portion of oligoarthritic PsA patients could be reclassified as having polyarthritis.

Objectives: 1. To evaluate sub-clinical synovitis of hand joints in patients with PsA by B-mode and Power Doppler US. 2. Correlation of PsA disease activity with US detected synovitis.

Methods: 27 patients of PsA (disease duration < 2years, no clinical evidence of hand joint involvement) and 30 controls were recruited. In PsA group cutaneous and articular disease activities were assessed by PASI score and DAPSA score respectively. Ultrasonography [grey scale (GS) and power Doppler (PD)] was used to assess synovitis of hand joints. A GS score ≥ 2 and/or a PD score ≥ 1 were used to identify US detected synovitis.

Result: In PsA group, 62.96% patients had sub-clinical synovitis of any of the hand joints. Median numbers of joints involved was 4. In the control group 20% had evidence of sub-clinical synovitis in any of the hand joints as detected by GS and PD score. The difference was statistically significant (P value =0.0013). Among 810 hand joints scanned in PsA group, 7.28% joints showed evidence of sub-clinical synovitis. Wrist joint was the most commonly involved (28.81%), followed by DIP3 (13.56%) and MCP3 (10.17%). Least involvement noted in MCP4, MCP5, PIP1, PIP5 and DIP5 (1.69%). No correlation noted between numbers of joints with subclinical synovitis with DAPSA or PASI score.

Conclusion: Almost two third patients with early PsA had PDUS evidence of sub-clinical synovitis in hand joints, most commonly in wrist joint followed by DIP3 and MCP3. There was no correlation between number of joints with sub-clinical synovitis and disease activity indices in PsA.


  PC114 Spondyloarthropathy Top


Correlation between BASDAI and AS disease activity score combines patient-reported assessment and serologic marker of inflammations scores: A cross-sectional study from eastern India

Ashutosh Mukherjee, Urmimala Bhattacharjee, Kaushik Basu, Rathindranath Sarkar; Kolkata Medical College, Kolkata, West Bengal, India

Background: BASDAI has been the most widely used clinical disease activity measure in axial spondyloarthritis. However BASDAI is patient oriented and measures only part of disease activity. Whereas AS disease activity score (ASDAS) combines patient-reported assessment and serologic marker of inflammations (CRP and ESR).

Objective: To establish possible correlation between BASDAI and ASDAS (CRP and ESR) scores in axial spondyloarthropathy patients.

Methods: Total 70 patients of Axial Spondyloarthritis (according to ASAS Criteria, 2009) irrespective of disease duration and treatment received were selected for the study. Demographic data and the history of disease onset and duration, treatment received including its duration and adherence to treatment, other co-morbidities and medications were documented from each patient along with HLA-B27 status, X-ray SI joints, MRI STIR images of SI joint (for X-ray negative subjects). Routine blood tests including ESR and CRP were performed and BASDAI and ASDAS (CRP and ESR) questionnaires are filled up.

Result: Strong correlation was noted among values of CRP and ESR in the defined population. Strong correlation were also noted in between either of these two biochemical disease activity measures and objective and composite disease activity scoring BASDAI and ASDAS (CRP and ESR) respectively in this study. High correlation was noted between BASDAI scores when compared with ASDAS CRP and ASDAS ESR. Robust correlation noted in between ASDAS CRP and ASDAS ESR. BASDAI showed 45.7% of study population had good control and 54.3% had poor control of disease. Among these, on comparison with ASDAS, mean disease activity were 3.55±0.96 by ASDAS CRP and ASDAS ESR score was 3.54±0.7 suggestive of very high disease activity.

Conclusion: Both BASDAI and ASDAS CRP and ESR were highly validated by strong statistical correlation in regard of assessment of disease activities in Spondyloarthropathies. ASDAS CRP and ASDAS ESR among themselves are more strongly validated. With ASDAS scores, more active diseases are picked up and in this regard it is superior to BASDAI as both patient and caregiver remain more cautious with change of disease status.


  PC115 Spondyloarthropathy Top


Safety and efficacy of rituximab as primary immune-suppressant in ankylosing spondylitis: A prospective one-year follow-up study

Shinjan Patra, Kripasindhu Gantait, Rajdip Chowdhury; Midnapore Medical College, Midnapore, West Bengal, India

Background: Among the various spondyloarthritides ankylosing spondylitis (AS) holds the special value for its unique characteristics and most importantly limited therapeutic options. Non-steroidal Anti- inflammatory Drugs (NSAID's) were the first effective options discovered but Tumour Necrosis Factor (TNF) alpha inhibitors had revolutionized the treatment of AS later. Additionally sulfasalazine and methotrexate can be used in peripheral-predominant AS. Abundance of CD20+ cells in the biopsy specimens of AS has prompted us to use rituximab as a primary immunosuppressant in this study. Various case reports have depicted some kind of efficacy and reduction in the inflammatory response in rituximab treated AS patients but longitudinal long-term follow up studies are very limited.

Objectives: Our aim was to study the safety and efficacy of Rituximab as a primary immunosuppressant's in those patients of AS who were on conventional treatments like NSAID's or sulfasalazine/ methotrexate only, but not received any biologics like TNF-alpha inhibitors before.

Methods: Patients with AS diagnosed by modified New York Criteria were included but patients suffering for more than 10 years and with active tuberculosis were excluded. We gave 2 doses of rituximab (375 mg/m2) 14 days apart pre-medicated with methylprednisolone and followed them periodically up-to 48 weeks and monitor all possible clinical, metrological and laboratory indices to assess the efficacy and also noted any possible adverse events in this time-period.

Results: Total twenty-five patients (22 male and 3 female; 21 axial-predominant and 4 peripheral-predominant) completed the follow-up and all indices including the inflammatory markers have improved significantly like Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) has improved more than 50% from its pre-treatment values in all groups at the end of 48 weeks. No major side-effects were seen in all these patients.

Conclusions: TNF-alpha inhibitors, though efficacious, has its problems of reactivation of latent tuberculosis and infusion-related side-effects. As there is a paucity of literature regarding using rituximab as a primary immune-suppressant ahead of TNF-alpha inhibitors, we are probably the first one to study in such fashion with a year-long follow-up. Using rituximab in this study has showed great promise in terms of efficacy and safety issues in AS.


  PC116 Spondyloarthropathy Top


Non-radiographic axial spondyloarthritis-clinical profile and disease burden 2-year experience from a tertiary care centre in south India

Sivakumar Vengudusamy, T N Tamilselvam, S Balameena, Euphrasia J Latha, V A Sowndhariya, R Rajavel; Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: The 2009 classification criteria for axial spondyloarthritis includes both patients with radiographic evidence of sacroiliitis and patients without radiographically definite sacroiliitis or non-radiographic axial spondyloarthritis. Non-radiographic axial spondyloarthritis may be considered an early stage of axial spondyloarthritis because the condition progresses to radiographic axial spondyloarthritis over several years in a subgroup of patients.

Aim: To provide an overview of clinical characteristics and burden of non-radiographic axial spondyloarthritis and difference between non-radiographic and radiographic axial spondyloarthritis in our population.

Methods: Patients who presented to our OPD with features suggestive of inflammatory back pain were included in the study. Detailed history, physical examination was done. Investigations include base line evaluation (CBC, RFT, LFT), acute phase reactants (ESR, CRP), imaging (skiagram, MRI-SIJ) and disease activity indices like BASDAI, BASFI were noted.

Results: 109 consecutive patients fulfilling ASAS criteria for spondyloarthritis were enrolled in the study out of which 31 patients had non-radiographic axial spondyloarthritis. There is no statistical significance among the variables compared except for short disease duration in non-radiographic spondyloarthritis [Table 1].



Conclusion: Non-radiographic axial spondyloarthritis patients also have similar disease burden when compared to radiographic spondyloarthitis.


  PC117 Spondyloarthropathy Top


Altered dose regime of Infliximab in Ankylosing Spondylitis Can Biosimilar be equally effective?

Syamasis Bandyopadhyay, Susobhan Mondal, Sandip Chandra, Suvasis Ghosh; Apollo Gleneagles Hospital, Kolkata, West Bengal, India

Background: Altered dosage regimen of Infliximab (INX) has shown to be effective in reducing disease activity in Ankylosing Spondylitis (AS). However, this has not been studied with Biosimilar Infliximab (B INX).

Objective: To compare safety and efficacy of INX and BINX, with altered loading regimen in patients newly diagnosed with AS.

Methods: Data of 40 newly diagnosed AS subjects, who received three 5 mg/kg infusions of either INX (n=26) or BINX (n=14) at 0, 8 and 16 weeks, were retrospectively analyzed. Patients voluntarily chose the drug without bias. The endpoint of analysis was change is BASDAI, ESR and CRP from baseline to 16 weeks. Paired T-test was used for statistical significance. Safety was assessed by adverse event (AE) records.

Results: For INX and BINX, most were males, mean age were 32.96 and 31.14 respectively. The results of INX, BINX and their comparison are given in [Table 1],[Table 2],[Table 3], respectively (Attached seperately). No adverse events were recorded.



Conclusion: The preliminary results suggest biosimilar infliximab in an altered regimen is effective but larger studies needed for further evaluation


  PC118 Spondyloarthropathy Top


Biosimilar adalimumab in ankylosing spondylitis â€" comparison of efficacy at 6 months and 1 year

Syamasis Bandyopadhyay, Abhrajit Ray1, R N Sarkar2, Suvasis Dash, Susobhan Mondal, Sandip Chandra; Apollo Gleneagles Hospital, 1Fortis Hospital, 2Medical College Hospital, Kolkata, West Bengal, India

Background: Adalimumab is approved for treatment in ankylosing spondylitis (AS). A biosimilar adalimumab (bADA), a 'fingerprint match' of originator adalimumab in terms of physicochemical and clinical parameters, is approved for use in India.

Objective: This retrospective analysis reports real-life data of bADA in AS patients.

Methods: Medical records of 52 AS patients who received bADA therapy, 40 mg s/c twice a month for at least 24 weeks and up to 48 weeks, were evaluated for standard AS outcome-measurement scores. Paired T-test was used for statistical analysis.

Results: Treatment with bADA for 24 weeks was associated with significant reductions in ESR, CRP, BASDAI, BASFI, and HAQ scores in all patients. Of these, 28 patients continued to receive bADA therapy, resulting in further significant reductions of all outcome scores at week 48 [Table 1]. For rest of the 24 patients who received only 24 weeks of treatment, BASDAI and BASFI scores did not deteriorate despite discontinuation of bADA treatment; however, rise in biomarkers was observed. Conclusions: Real-life safety and efficacy analysis of bADA in AS patients shows continued effectiveness post 24 weeks treatment despite bADA stoppage. Continued bADA use for 48 weeks is associated with improved biomarker and clinical parameter.



Acknowledgement: The authors would like to thank Dr. Hetal Shah, Independent Medical Writing Expert from Ahmedabad, India for her professional support in data analysis and abstract writing.


  PC119 Spondyloarthropathy Top


Low versus high dose adalimumab in ankylosing spondylitis patients

Arug Gogna, Sameer Gulati; VMMC and Safdarjung Hospital, New Delhi, India

Background: Adalimumab is one of the TNF inhibitors used in the treatment of ankylosing spondylitis (AS). It's dose for AS has been extrapolated from that of rheumatoid arthritis and psoriatic arthritis. There is not much information on efficacy of 40 mg adalimumab administered every month.

Methods: We analyzed AS patients treated with Adalimumab retrospectively. Adalimumab was given to AS patients with poor response of either axial symptoms to NSAIDs or peripheral arthritis to sulfasalazine. Patients with poor response to TNF inhibitors other than Adalimumab were also considered. Active infections, hypersensitivity, pregnancy, lactation, tuberculosis, multiple sclerosis, lupus and malignancies were ruled out. Low dose Adalimumab (40 mg every month subcutaneously) was offered to patients with cost constraints and rest were offered conventional high dose (40 mg every other week subcutaneously). Back pain, peripheral joint pain/swelling and global patient assessment were recorded on a scale of 0-10 at baseline and at 12 weeks. Besides, duration of morning stiffness and C reactive protein was also recorded at baseline and at 12 weeks. The ASDAS scores were calculated at baseline and at 12 weeks to assess responders (Delta ASDAS >1.1 and delta ASDAS >2).

Results: Four patients each were treated by low and high doses of Adalimumab. All of them were HLA B27 positive and one patient each had peripheral arthritis along with axial symptoms in both the groups. Delta ASDAS > 1.1 (Baseline ASDAS - ASDAS at 12 weeks) was achieved in all the patients in the high dose group and in 3 patients in low dose group (not significant at p < 0.05). Delta ASDAS > 2 (Baseline ASDAS - ASDAS at 12 weeks) was achieved in 3 patients in high dose group and in 2 patients in low dose group (not significant at p < 0.05).

Conclusion: There was no significant difference between the two treatment doses of Adalimumab. The power of our study is not adequate in view of small statistical sample. Thus, this may be taken as a pilot study to plan further randomized controlled trials with adequate power to validate our findings in order to enable cost saving with once monthly Adalimumab in Ankylosing Spondylitis patients.


  PC120 Spondyloarthropathy Top


To study the efficacy of nonsteroidal anti-inflammatory drugs/coxibs on acute phase reactants in patients with axial spondyloarthritis

S Bhalla, Q Zaheer, S Verma, A N Malaviya; Department of Rheumatology, A & R Clinic™ and Indian Spinal Injuries Centre, New Delhi, Inida

Background: NSAIDs/Coxibs are highly effective in controlling the major symptoms of axSpA (pain and stiffness). Some workers have suggested that they may even have disease-modifying properties including retarding the progression of structural damage in the spine1.However, 2 other studies have shown that there were no changes in objective signs of inflammation as assessed by MRI and CRP in patients treated with NSAIDs2.

Objectives: To study the effect of NSAIDs/Coxibs on acute phase reactants in patients with established axSpA.

Methods: The study included 31 patients with axSpA classified according to the ASAS criteria (2009). They were prescribed only NSAIDs/Coxibs without glucocorticoids or any DMARD. Baseline and follow-up assessment included BASDAI, BASFI, BASMI, ASDAS, ESR and CRP levels. The baseline values of the clinical parameters and the acute-phase reactants were compared with the values at the follow-up visits statistically.

Results: Of the 31 patients, 19 (61.3%) had high acute phase reactants (mean ESR 43 mm/hr, mean CRP 24.3 mg/L) at the baseline. Twelve patients (38.7%) had normal acute phase reactants (ESR < 30 mm/hr, CRP <10mg / dl). Post-treatment patients were followed up at 3, 6 and 9 months. It was observed that in 16/19 (84.2%) the acute-phase reactants reached normal levels [Figure 1]. Among them, 13 patients (81.25%) achieved normal levels of in 3 months, 2 (12.5%) at 6 months, 1 (6.25%) at 9 months respectively. The fall of the levels of the acute-phase reactants was statistically significant (ESR 'p' value 0.042; CRP 'p' value 0.001 respectively).



Conclusion: NSAIDs/Coxibs treatment appear to be effective in reducing the acute-phase reactants in patients with axSpA. These drugs, therefore, may not only be effective in controlling the symptoms but also, could be disease modifying. In a low socioeconomic country like ours where TNF inhibitors are not affordable by the majority, these drugs may be the cornerstone for the treatment of axSpA.

References

  1. Dougados M, Simon P, Braun J, Burgos-Vargas R, Maksymowych WP, Sieper J, et al. ASAS recommendations for collecting, analysing and reporting NSAID intake in clinical trials/epidemiological studies in axial spondyloarthritis. Ann Rheum Dis 2011;70:249-51.
  2. Escalas C, Trijau S, Dougadas M. Evaluation of the treatment effect of NSAIDs/ TNF blockers according to different domains in AS. Rheumatology (Oxford) 2010;49:1317-25.



  PC121 Spondyloarthropathy Top


Treatment response to conventional DMARDs in psoriatic arthritis

Taral Parikh, Sapan Pandya, Rakesh Solanki; Vedanta Hospital, Ahmadabad, Gujrat, India

Background: Psoriatic arthritis (PsA) is a heterogonous disease, so are the treatment options. DMARDs are indicated for multiple domains in PsA, however studies have shown conflicting results regarding their efficacy.

Objective: This study was undertaken to study the efficacy of DMARDs in a cohort of PsA patients.

Methods: 49 patients, from 2012-2017, fulfilling the CASPAR criteria, were analysed. Treatment response was assessed as per the (psoriatic arthritis response criteria) PSARC criteria, at the first follow up visit. Secondary outcomes assessed were improvement in (psoriatic arthritis skin index) PASI, dactylitis and enthesitis.

Results: (n=49) Male 29/female 20 Mean age 42 (±10.85), weight 67.69 (±12.59), duration of psoriasis onset 6.86 (±6.1), duration of PsA 3.26 (±4.88). 32.5%-family history of psoriasis Pattern of PsA: Polyarthritis 14 (28.5%), oligoarthritis 13 (26.5%), polyarticular +axial 10 (20.4%), oligoarticular +axial 10 (20.4%), axial + DIP 2 (4.08%) Baseline PASI 4.94 (±7.23), SJC 4.73 (±4.03), TJC 6.89 (±4.65), clinical enthesitis 6, dactylitis 18, physician global assessment (Ph GA) 3.17(±.0.84), patient global assessment of PsA (Pt GA) 3.50 (±0.83). Mean duration of follow up 2.8 (±1.35) months. Treatment response: 33 (67%) patients were started on NSAIDs, 3 (6%) on prednisolone, 40 (81.5%) on methotrexate, 16 (32.5%) on sulfasalazine, 13 (26.5%) on combination (MTx + Ssz) and 2 (4%) on leflunomide. At first follow up visit 38 (77.5%) were PSARC responders. PASI reduced from 4.94 (±7.23) to 1.8 (±1.87). 19 (38.7%) achieved PASI 75 and 3 (6%) PASI 50. Enthesitis improved in 4 (66%) and dactylitis in 9 (50%) patients.

Conclusion: After 3 months 77.5% patients were PSARC responders, with conventional DMARDs. There was improvement in other domains of PsA as well.


  PC122 Spondyloarthropathy Top


Long-term sustainability of response with infliximab biosimilar (BOW015) in ankylosing spondylitis: Efficacy and safety analysis from two centers in South India

Vijay K R Rao, A N Roy1, M Shamil2; Department of Rheumatology, Manipal Hospital, Bengaluru, 1Department of Rheumatology, Yashoda Hospital, Secunderabad, Telangana, 2Sun Pharma Laboratories Ltd., Mumbai, Maharashtra, India

Background: Recent studies have shown that four doses of infliximab biosimilar (BOW015) is effective in the treatment of ankylosing spondylitis (AS) over a 6 month period. However, the real-world long-term data on the safety and sustainability of response is limited.

Aim: To evaluate the long-term safety and sustainability of response to BOW015 at the end of one year.

Methods: Retrospective data from two centers in BOW015 infused biologicnaive patients diagnosed with AS over one year was analyzed. Data pertaining to patient’s demographics, medical history and concomitant medication were collected. We defined the primary variable - sustainable clinical response as proportion of patients achieving 2 points on a patient's global assessment of disease activity visual analog scale (0-10) at the end of one year.

Results: In the analysis set (N=25) for the primary variable, 21 (84%) patients were males with a mean age of 34.79 ± 11.75 (SD) years, mean body weight of 68.53 ± 9.88 (SD) kgs and 10 (40%) patients were positive for HLA-B27. Twenty three patients received the first four doses of BOW015 as per standard regimen (0, 2, 6 and 14 weeks) and further infusions on demand. In total, 21 (84%) patients received ≤ 5 infusions a one year period Further, 16 (64%) patients were treated with 200 mg, 4 (16%) with 300 mg and 5 (20%) patients were treated with 400 mg of BOW015 respectively. At the end of one year, 19 (76%; P<0.001) patients experienced a sustainable clinical response [Figure 1]. There was an absolute change of 41.05 (95% CI 28.77 - 53.33) and 42.22 (95% 27.57 â€" 56.86) in ESR and CRP values respectively. ASDAS and BASDAI were done in 10 patients in whom an absolute change of 2.55 (95% CI 0.45-4.65) in ASDAS and 1.69 (95% CI 0.11-3.27) in BASDAI were noted [Figure 2]. Overall, mild adverse events were observed in 5 patients: 2 patients had increased ALT, 2 patients had increased AST and one patient developed leukocytosis.



Conclusion: BOW015 was safe, well tolerated and majority of the patients had sustained clinical response with just five doses of infusion at the end of one year.


  PC123 Spondyloarthropathy Top


Retrospective outcome analysis of patients with reactive arthritis and peripheral spondyloarthropathy

Sakir Ahmed, Abhra Chowdhury, Smriti Chourasia, Vikas Agarwal, Able Lawrence, Amita Aggarwal, Ramnath Misra; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Introduction: Reactive arthritis (ReA) is a seronegative spondyloarthropathy (SpA) that is precipitated by urogenital or gastrointestinal infection. Peripheral spondyloarthropathy (pSpA) may be indistinguishable from ReA except known preceding infection. It is stated that two-thirds of ReA resolve within three months while a third develop chronic or recurrent course. However, there is a paucity of data on the long-term outcome. Thus, it is difficult to justify treatment decisions like the use of biologicals in ReA.

Objectives: To determine the long-term clinical outcome of ReA/pSpA.

Methods: Patients in our database meeting Amor criteria for spondyloarthropathy with history of preceding infections were included as ReA, while pSpA were included as per ASAS criteria. Data on this retrospective cohort was updated with telephonic interviews. Follow-up of less than 1 year were excluded. Patients with persistent inflammatory back pain (IBP) were reviewed in the clinic. Radiographs assessed progression to AS (modified New York criteria).

Results: Follow-up data on 85 patients (63 ReA; 22 pSpA) was obtained. Median (IQR) age at presentation was 24.5 (20-33) years. 14 (16.5%) were female. At presentation, 23 (30%) had monoarthritis, 44 (57%) had oligoarthritis, 10 (13%) had polyarthritis (data missing for eight). Enthesitis and dactylitis were documented in 20 and 5 respectively. Keratoderma and balanitis were seen in one each. 40 (80%) out of 50 were positive for HLA-B27. Median (IQR) follow-up was 2 (1-5.25) years. 22 had monophasic illness of which 13 had acute arthritis (<12 weeks). Seven had two episodes, 27 had polycyclic course while 29 had persistent arthritis. 26 had inflammatory back pain (IBP) at any time and eight had persistent symptoms. Out of these, three (3.5%) met criteria for AS (disease duration: 1, 3 and 10 years) while two had unilateral grade 2 sacroiliitis. Three patients without IBP also had sacroiliitis on radiographs. Logistic regression revealed no significant parameter that could predict progression to AS.

Conclusion: This is the first longitudinal study in ReA that has shown, in contrast to the existing literature, that one-third of patients develops persistent arthritis and another third develop recurrent arthritis. 3.5% progressed to AS. As duration of follow-up increases, a greater proportion may progress to AS.


  PC124 Lupus APS Sjogren's Syndrome Top


Lupus enteritis: A retrospective study from a tertiary care hospital

Spoorthy Kothapalli, I R Vara Prasad, L Rajasekhar; Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Introduction: Lupus enteritis is a rare manifestation of Systemic Lupus Erythematosus (SLE). Aim is to study the clinical profile of patients with lupus enteritis admitted in tertiary care hospital.

Methods: In this retrospective study, hospital records of patients admitted with SLE and lupus enteritis between January 2012 and July 2017 were reviewed and analysed. Lupus enteritis was diagnosed by clinical features and abdominal computed tomographic findings. Demographic data, Clinical features, laboratory parameters and treatment details were noted. Disease activity was measured by SLEDAI. Patients with gastrointestinal infection and incomplete data were excluded. Descriptive statistics for the categorical variables were performed by computing frequencies in each category.

Results: Twenty seven patients with lupus enteritis have been identified of which 21 were females. Mean age was 26.6 ± 8.9 years. Eleven patients had lupus enteritis at initial diagnosis of lupus, while three had recurrent episodes. Most common clinical symptom was pain abdomen (25/27) and diarrhea (18/27).Ascites was seen in (16/27).Seventeen patients had Nephritis. Median ESR was 50mm in 1 hour. Hypocomplementemia was noted in (12/18), dsDNA positivity in (20/27). Anticardiolipin antibodies in medium titres were positive in (12/19) cases. Mean SLEDAI was 14.7 ± 7.38. The most common part involved was colon (15/27) followed by ileum (10/27), jejunum (8/27) cases, only one case had duodenal and one had caecal involvement. All patients received high dose of corticosteroids, twelve required pulse methyl prednisolone. Cyclophosphamide was given in (14/21), Rituximab in 1 patient of recurrent enteritis. Two patients died.

Conclusion: Lupus enteritis was observed in patients with high disease activity. Colon is the most commonly involved. Anticardiolipin antibodies were positive in two thirds of the patients tested. Most of them responded favourably to high dose corticosteroids.


  PC125 Lupus APS Sjogren's Syndrome Top


Antibody profile in Indian lupus

Wasim Kazi, Muzzafar Bindroo, Dhiren Raval, Navan Mendiratta, Shruti Bajad, Vinay Singal, Rajiva Gupta; Medanta the Medicity, New Delhi, India

Background: SLE is characterized by the aberrant production of heterogeneous group of antibodies. Even though these antibodies are known for a long duration, still efforts are being taken to know the pathogenic, diagnostic and prognostic significance of these antibodies. Indian data with reference to prevalence of these antibodies is scarce.

Methods: We studied 305 SLE patients, who presented to OPD and IPD of Medanta Hospital, from September 2015 to April 2017. In all patients, autoantibody profile was done using the Blue Diver ANA 25 screen immunodot kit. A titer of >10 was considered to be positive. Anti-phospholipid antibody profile (Lupus anticoagulant, Anticardiolipin IgM, IgG and Anti β GPI IgM and IgG) was done in 245 patients.

Results: 305 patients were studied. The median age of cohort was 36 years and 84.6% of them were female [Table 1].



Conclusion: In our cohort, most common antibodies were anti SS-A Ro 60 (52%), followed by anti SS-A Ro 52 (39%) and anti dsDNA (22%). As opposed to western data, which shows dsDNA is positivity in 70 to 98% patients, our study showed anti dsDNA positive results only in 22% patients. Also SS-A Ro antibodies were present in almost 90% of patients. Amongst patients who were screened for ALPA, lupus anticoagulant was most prevalent (23%).


  PC126 Lupus APS SJogren's Syndrome Top


Across-sectional study of nailfold microvascular changes in Indian patients with RNP+ lupus and mixed connective tissue disease using nailfold videocapillaroscopy

Pramod Prahlad Chebbi, Ruchika Goel, J Ramya, M Gowri, Debashish Danda; Department of Clinical immunology and Rheumatology and Biostatistics, Christian Medical College Vellore, Tamil Nadu, India

Background: Nailfold Capillary (NFC) changes represent degree of microvascular involvement in autoimmune connective tissue diseases. Anti U1-RNP is associated with specific internal organ involvement in SLE. Nailfold capillaroscopy may objectively predict the systemic microvascular abnormalities in SLE patients with positive Anti U1-RNP antibody.

Objective: To study nailfold microvascular changes (NFVC) in SLE patients with RNP+ and compare them with NFVC changes observed in patients with RNP negative SLE and Mixed connective tissue disease (MCTD).

Methods: Nailfold Videocapillaroscopic (NFVC) examination (Optiliamediscope, 200X) was performed in consecutive patients satisfying classification criteria of SLE with or without Anti-U1 RNP positivity. Patients satisfying criteria for MCTD were recruited as disease controls. Individual NFC parameters were analysed by a blinded assessor in accordance with the definitions described in literature. Changes in 3 groups were compared using nonparametric tests. Ordinal logistic or linear regression was used wherever applicable to assess any independent association of NFC changes with disease groups.

Results: Total of 81 patients were studied, of which 28 had SLE with RNP+ (age 30.0±10.37; 26 females), 26 had SLE without RNP positivity (age 29.42 ± 9.20; 25 females) and 27 had MCTD (age 37.0 ± 9.86; 26 females). Capillary density was significantly reduced in MCTD as compared to RNP+ SLE patients (5.33 ± 1.66/mm vs 7.25 ± 1.38/mm, p=0.000), as well as in RNP+ SLE as compared to RNP negative SLE patients (7.25 ± 1.38/mm vs 8.92 ± 1.13/mm, p=0.000). Conversely,patients with RNP+ SLE had more frequent giant capillaries, enlarged capillaries and ramified/branched capillaries as compared to RNP negative SLE (p = 0.047,0.001 and 0.029 respectively). However, there was no statistical difference in number of haemorrhages among these groups. These changes were more severe in patients with MCTD as compared to RNP+SLE. Ordinal logistic regression showed more severe reduction in capillary density in patients with RNP+SLE as compared to RNP negative SLE (OR= 9.5, p=0.007) independent of the presence of Raynaud's, ILD and disease duration.

Conclusion: Presence of anti-U1RNP antibody is independently associated with microvascular abnormalities in SLE as detected by NFVC. Patients with MCTD have more profound abnormalities as compared to RNP+SLE patients.


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Lupus retinopathy: A marker of active systemic lupus erythematosus

Gaurav Seth, K G Chengappa, Pooja Belani, K C Shanoj, Vir Singh Negi; Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

Background: Systemic lupus erythematosus (SLE) can affect virtually any organ in the body and ocular involvement is common seen in almost one- third of the patients. Retinopathy has often been associated with a more severe form of disease and poor prognosis.

Objectives: To study ocular involvement and correlate their association with other clinical manifestations and autoantibody profile in patients with SLE.

Methods: Four hundred and thirty seven patients fulfilling the Systemic Lupus International Collaborating clinics- American college of Rheumatology- 2012 (SLICC-ACR) criteria, attending the outpatient and inpatient department of Clinical Immunology, JIPMER, were enrolled under this cross sectional study. A comprehensive clinical examination, detailed ophthalmological examination, immunological profile (C3, C4, ENA, anti APLA IgG/IgM, Beta2 GP1 IgG/IgM and lupus anticoagulant) were performed for patients. Retinopathy was defined as the presence of any of the following lesions: cotton-wool spots, hemorrhages, vasculitis, papilledema, optic atrophy, or retinal detachment. Retinal lesions were confirmed by an ophthalmologist. SLEDAI was used to calculate disease activity.

Results: The mean age of the study participants was 28.06 ± 9.7 years and a majority of them were women (93.1%). The median duration of disease was 12 months (IQR: 6, 36). Fifty-nine subjects (13.5%) had juvenile onset of disease. Of the 437 patients, forty-five (10.3%) were diagnosed to have SLE associated retinopathy. Autoimmune haemolytic anaemia [31.1% vs 14.5%, p value: 0.004, OR (CI): 2.65 (1.33- 5.29)], serositis [33.3% vs 18.9%, p value: 0.023, OR (CI): 2.14 (1.11-4.10)], lupus nephritis [62.2 vs 40.8%, p value: 0.06, OR (CI): 2.38 (1.26-4.50)] and seizures [28.9% vs 12.8%, p value: 0.004, OR (CI): 2.77 (1.36-5.65)] were significantly higher among the patients with retinopathy. Mean SLEDAI score among patients with retinopathy was higher than those without retinopathy (25.44 vs 18.01), though it failed to reach statistical significance.

Conclusion: This study strongly suggests that SLE associated retinopathy is commonly seen in SLE and is a marker of active disease with a frequent central nervous system and renal involvement. Retinopathy portends poor visual prognosis, it is therefore recommended to screen all patients for retinopathy as a part of disease workup.


  PC128 Lupus APS Sjogren's Syndrome Top


Long term outcome of 273 juvenile onset SLE patients

Sanat Phatak, Puja Srivastava, Bonnie Abujam, Ramnath Misra, Vikas Agarwal, Durga Prasanna Misra, Able Lawrence, Amita Aggarwal.; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Objectives: Long term outcome of juvenile onset SLE (JSLE) has been difficult to assess as they are initially managed by pediatricians and later transitioned to adult services. Our clinic provides care to both pediatric and adult SLE patients thus ensuring continuity.

Methods: Medical records of patients with onset of disease before age of 18 years (JSLE) were used to collect data on demographics, clinical and laboratory features, medications, outcome and complications. Overall outcome and actuarial survival were calculated using SPSS software.

Results: There were 273 patients with JSLE (250 girls) among a total of 1510 SLE patients, with median age at onset of 14 years (1-18 years). Among major organ involvement, 165 had renal disease and 80 had CNS disease. Twenty-five patients were treated elsewhere and 248 patients were followed up with median follow up duration of 3.5 years (0.5-28 years). Ninety-eight and 46 patients have 5 years and 10 years follow up data respectively. At last visit, 183 patients were in complete remission, 28 in partial remission, 18 had active disease and 14 had died. Flares were seen in 120 patients (85 major and 48 minor flares), with 40 patients having more than 1 flare. Median time to first flare was 2 years (0.5-16 years). Infections were seen in 72 patients of which 38 required hospitalization. The common infections were: bacterial pneumonia (n=26), skin infection (n= 23) and tuberculosis (n=17). Ninety-one patients accrued damage and the maximum SLICC damage index was 6. ESRD-free survival was 24.3 years (95% CI 22.3- 26.4 years). Five and 10-year cumulative survivals were 94.3 and 89.2% respectively. CNS disease, leucopenia, raised anti-dsDNA levels and hospitalization due to infection were independent predictors of poor outcome (Death/ESRD) in both univariate and multivariate logistic regression analysis. Fourteen patients died (6 early deaths due to disease activity, 2 with ESRD, 1 TB, 1 PAH with CAPS, 3 with septicemia, 1 diabetic ketoacidosis).

Conclusion: JSLE in India has good outcome but has significant morbidity due to disease flares and infections.


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Comparison of different immunosuppressive treatment in induction therapy of Lupus nephritis (cyclophosphamide versus mycophenolate versus rituximab): A retrospective study in eastern Indian population

Geetabali Sircar, Rudra Prasad Goswami, Sumantra Mondal, Subhankar Haldar, Hiramanik Sit, Alakendu Ghosh, Parasar Ghosh; Department of Rheumatology, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, India

Systemic Lupus Erythematosus multisystem connective tissue disease with high propensity for renal involvement. Evidence suggests that Asians have higher rates of lupus nephritis and more severe nephritis compared to other racial and ethnic groups. We studied the efficacy of induction regimens of high dose and low dose cyclophosphamide, mycophenolate and rituximab in lupus nephritis in Eastern India. It is a retrospective observational study in 222 patients in lupus nephritis of classes III, IV and V. Four groups were compared: high dose cyclophosphamide (NIH regimen), low dose cyclophosphamide (EUROLUPUS), mycophenolate and Rituximab (1g in 2weeks apart). Outcome measures were complete (CR) and partial remission (PR) at the end of 6 months. One hundred and thirteen patients received high dose cyclophosphamide (NIH regimen), 26 patients low dose cyclophosphamide (EUROLUPUS), 61patients with mycophenolate and 22 patients with Rituximab (1g in 2weeks apart). Overall 16.2% (36/222) had no improvement of proteinuria, 18% (40/222) had PR and 65.8% (146/222) had CR. High dose Cyclophosphamide and Rituximab regimens achieved higher rates of CR (90% each) compared to low dose cyclophosphamide (73%) and MMF (72%) (p=0.006). Rituximab was also most effective in relapsing disease (100% CR). Infection was highest with the NIH regimen followed by EUROLUPUS regimen; MMF group had a higher incidence of gastrointestinal side effects. To conclude, high dose cyclophosphamide and rituximab were effective treatment options in moderately severe lupus nephritis in our cohorts of patients with rituximab effective in relapsing disease. Low dose cyclophosphamide and MMF were similar in efficacy.


  PC130 Lupus APS Sjogren's Syndrome Top


24 Hour urinary protein versus random spot protein-creatinine ratio: A comparison in lupus nephritis

Urmimala Bhattacharjee, Ritasman Baisya, Akashdip Bhattacharya, Kaushik Basu; Medical College, Kolkata, West Bengal, India

Background: The accurate assessment of proteinuria is vital for management of lupus nephritis. As 24 h urinary collections are cumbersome and frequently unreliable due to inadequate collection, a reliable and easy measure like the spot urine protein-creatinine ratio (UPCR) would be ideal in clinical practice.

Objective: To compare random spot UPCR with 24 hour urinary protein (which is considered the gold standard) under different ranges of proteinuria and scores of NIH activity index (AI).

Methods: Total 57 biopsy proven lupus nephritis patients between June 2015 and May 2016 were included. Biopsy proven lupus nephritis patients were divided into two groups based on ranges of 24h urinary protein: Group1 (<0.5g/day), Group2 (0.5-1.0g/day), Group3 (1.0-3.0g/day) and Group4 (≥3.0g/day). Also these patients were divided into four groups in the ascending order of activity indices in renal biopsy: Group A (low AI), Group B, Group C and Group D (high AI). Inclusion criteria included eGFR>10ml/min and a time period not exceeding 4 days between the two methods of sample collection for assessing proteinuria. The correlation and limits of agreement between the two methods were calculated.

Results: A good correlation (r=0.8849, p<0.0001) was found between UPCR and 24 h proteinuria. • Good correlation and agreement between the two methods were found at ≥3.0g/day proteinuria (r=0.9870, p=0.0003) as against previously reported studies. • Good agreement is also noted at higher ranges of activity indices contrary to previous studies. • Spot urine P/C ratio of 0.58, 0.852, 1.312 and 1.710 mg/mg reliably predicted 24-h urine protein of 0.5, 1.0, 2.0 and 3.0g/day respectively.

Conclusion: Though there is a good correlation between the two methods; spot UPCR cannot adequately replace the “gold standard†method 24h urinary protein in lupus nephritis, where the absolute values are used to define the activity of the disease.


  PC131 Lupus APS Sjogren's Syndrome Top


Long term outcome of nonbacterial thromboendocarditis in Indian lupus patients

Dhiren Raval, Muzaffar Bindroo, Natasha Negalur, Gayatri Ekbote, Dhaval Tanna, Wasim Kazi, Naval Mendiratta, Shruti bajad, Vinay Singal, Rajiva Gupta; Medanta the Medicity, Gurgaon, Haryana, India

Background: NBTE, though rare, is commonly seen in advanced malignancies and closely followed by SLE. The prevalence of NBTE in SLE is 6-10 % as per western studies. It is the most characteristic cardiac manifestation in SLE patients, which usually remains silent without significant valvular dysfunction or embolic phenomena. Treatment of NBTE consists of anticoagulation and therapy directed to the underlying disease. Whether anticoagulation should be recommended for patients with APLA-associated vegetations who have never had any thromboembolic event has not been studied. We conducted this study to look for long term outcome of NBTE in SLE patients with or without APLA syndrome.

Aims: To study the long-term outcome of NBTE in Indian lupus patients with or without APLA syndrome.

Methods: All SLE inpatients and outpatients attending the department of Rheumatology from September 2015 to June 2017 were enrolled. Those who had NBTE on 2D echo were followed up to look for any complications (thromboembolism or valvulitis).

Results: The total number of patients enrolled in the study was 307, out of which 189 underwent 2D echo.NBTE was found in 29(15.34%) patients. Median follow up was 8 months [Table 1]. Three patients were lost to follow up. Nine patients of SLE with APLA syndrome were subjected to repeat 2D echo out of which 7 patients showed resolution of NBTE, while in APLA negative SLE patients four were subjected to repeat 2Decho and all had the resolution of NBTE.



Conclusion: The prevalence of NBTE in SLE is 15.3%.We found that most of the SLE patients with NBTE with or without APLA syndrome did not have any long- term complications. However, NBTE patients without concomitant APLA syndrome did not have any complications even without anticoagulation treatment. However, long duration of follow up is needed to confirm our results.


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Rituximab in primary Sjogren's syndrome with ild: 1 year outcome from a tertiary care centre

Anuja Rajan, Thiruchengode Natesan Tamilselvam, Selvakumar Balameena, Eupharasia Latha, Myilasamy Saravanan, M Rajavel, C Saranya; Institute of Rheumatollogy, Madras Medical College, Chennai, Tamil Nadu, India

Background: Primary Sjogren's syndrome is commonly associated with various respiratory manifestations the most typical of which is chronic ILD. The most common type of ILD in sjogrens syndrome is NSIP pattern. While corticosteroids are the mainstay of therapy for ILD, the efficacy of other immunosupressants needs to be assessed.

Objective: 1) To evaluate the therapeutic effect of rituximab on ILD in primary sjogrens syndrome. 2) To evaluate the outcome of treatment in patients with primary Sjogren's syndrome with ILD treated with rituximab when compared to cyclophosphamide.

Methods: A prospective observational study was conducted in 23 patients of primary Sjogren's syndrome with ILD who were admitted in Institute of rheumatology. They were categorized into two groups, one group (n=12) received 1 gm of rituximab at 2 weeks intervals at 0, 6 months, followed by azathioprine. The other group (n=11) received 6 monthly pulses of cyclophosphamide followed by azathioprine as maintenance. The outcome of treatment was assessed by HRCT chest using semiquantitative scoring by a radiologist as well as FEV1 and FVC measurements by computerised spirometry at 0, 6 months and 1 yr.

Results: After treatment with rituximab, there was significant improvement in the FEV1 (p <.0001) and FVC (p <.0001) at 1 year when compared to baseline. There was also significant improvement in the CT scores (p <.0001) after 1 year of treatment. When compared to cyclophosphamide, the patients who received rituximab showed significant improvement in the CT scores (p =.024).

Conclusion: Rituximab is effective in improving the radiologic features and lung function in patients with ILD due to Primary Sjogren's syndrome. Though cyclophosphamide is the most common immunosuppressant used in these patients, Rituximab is set to emerge as one of the promising therapies for ILD in primary Sjogren's syndrome in the recent future.


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Sex matters, race/ethnicity matters, and it matters where you live: The case of SLE mortality

Ram Raj Singh, Eric Yen; UCLA, Los Angeles, California, USA

Background/Purpose: In the largest population-based study of secular trends for SLE mortality, age-standardized mortality rate (ASMR) for SLE has decreased from late-1990s after periods of increasing rates in 1970s-1990s, however, it remains high relative to non-SLE-ASMR (Ann Int Med, accepted for publication). Evaluating characteristics associated with mortality could help identify modifiable risk factors that might inform targeted research and public health programs.

Methods: We used US mortality data from 1999-2013 to calculate ASMR for SLE and non-SLE deaths, and SLE:non-SLE ASMR ratio, and trends using ‘joinpoint’ analysis. We performed Poisson-regression to predict SLE mortality with robust estimator of the variance using Huber/White/sandwich estimator and estimated the risk ratio on the aggregated, group-level data (death-count and population-size for each of 192 groups by sex/race/ethnicity/age/region/time categories) using Wald Chi-Square-test. In addition to main effects for each of the demographic/time variable, we tested for pairwise-interactions to assess effect modification among variables.

Results: 18,866 and 12,594 deaths with SLE as the underlying or a contributing cause of death were identified, respectively. SLE-ASMR decreased at an annual percent change of -2.7% during 1999-2013. Adjusted for age, sex, race/ethnicity, and geographic region, the risk for SLE-mortality was lower in 2009-2013 than in 1999-2003. Females had higher SLE-mortality risk relative to males, as did minority race/ethnicity status, residence in the South or West regions of the US relative to Northeast, and individuals ≥65 years old relative to those who were 0-64 years old. Interaction testing for effect modification revealed significant interactions between race/ethnicity*sex and between race/ethnicity*region. Stratified by race/ethnicity, SLE-mortality risk was higher in females than in males in all race/ethnic groups, but both the risk ratio and absolute mortality differences significantly differed between race/ethnic groups. For each race/ethnic group, SLE-mortality risk was greater in all other regions relative to the Northeast, with a few exceptions. Residence in the South conferred the highest SLE-mortality risk in whites, but in the minority racial/ethnic groups, the highest risk was in residents in the West region.

Conclusions: Disparities in SLE outcomes persist by sex, race/ethnicity, and geographic regions, with significant geographic differences for each race/ethnicity.


  PC134 Lupus APS Sjogren's Syndrome Top


Vitamin D deficiency: Still in game for lupus flare development? A prospective observational study in North Indian tertiary care centre

R Sriranga, Devender Bairwa1, Ravinder Goswami2, Piyush Ranjan, R Lakshmi3, V Srinivas4, Sauvik Dasgupta1, Shiv Kumar Suman1, Uma Kumar1; Departments of Medicine, 1Rheumatology, 2Endocrinology, 3Biochemistry and 4Biostatistics, AIIMS, New Delhi, India

Background: Role of vitamin D deficiency in SLE flare is still a matter of debate, because of paucity of good quality data.

Objective: To study the association of vitamin D deficiency with SLE disease severity and flare frequency in a lupus cohort at a tertiary care centre in North India.

Methods: After getting ethical clearance from institutional ethical committee, 155 patients of SLE fulfilling ACR1997 classification criteria at diagnosis were enrolled in the study. Disease activity was assessed on the basis of SLEDAI 2000. Patients having Overlap Syndrome and not willing to follow up for 6 months were excluded. Blood samples were collected from the patients at baseline and during flares and sera was stored at -70 degree. All patients were evaluated at baseline, 3 months, and 6 months; and were assessed clinically and with relevant investigations to assess for presence of SLE flare. Stored sera was analysed for 25 OH vitamin D levels by chemiluminescence assay. Vitamin D deficiency was defined as serum level less than 20 ng/ml. Statistical analysis was performed using Stata 11.2.

Results: At baseline, 103 (67%) patients had stable disease, while 52 (33%) had active disease. 23 (44%) and 29 (56%) patients had mild to moderate and severe disease respectively. During follow up 4.85% (5/103) patients developed renal flare. Mean vitamin D level among patients with active disease at recruitment was 17.88±11.32 ng/ml as compared to 18.37±12.24 ng/ml in patients with stable disease (p= 0.812). On follow up, the 5 patients who developed flare had vitamin D deficiency; however the number is very small for statistical analysis.

Conclusions: Contrary to prior studies which suggested association of vitamin D deficiency in SLE flare; our study did not show any such association, which is probably because of increased prevalence(~90%) of vitamin D deficiency in healthy Indian population. Further RCTs with long term follow up are needed to conclusively prove the association of vitamin D deficiency with SLE flare.


  PC135 Lupus APS Sjogren's Syndrome Top


Successful amelioration of lupus associated thrombocytopenia: New role for eltrombopag

Sandra Sanil, Reshmi Roongta, Vineeta Shobha; St. Johns Medical College, Bengaluru, Karnataka, India

Introduction: Eltrombopag is a thrombopoietin (TPO) receptor agonist. It induces megakaryocyte proliferation and differentiation causing increase in platelet counts. It is approved for treatment of immune thrombocytopenia and Hepatitis C related thrombocytopenia. The immunopathogenesis of ITP and lupus related thrombocytopenia share many features. Very few, small studies have been reported on efficacy of eltrombopag in Systemic Lupus Erythematosus (SLE) related thrombocytopenia, which occurs in 10-40% of the patients.

Materials: This is an ambidirectional record review of 8 patients diagnosed with SLE, using SLICC criteria. All had developed moderate to severe immune mediated thrombocytopenia during the course of their disease, necessitating the use of eltrombopag. Other drugs used for treatment of thrombocytopenia, their dose, duration of treatment and serial platelet counts were observed. Adverse effects of the drugs, if any, were noted. Time taken for response to therapy, defined as platelets >1 lakh, was also measured.

Observation: Out of 8 patients, all female, with a mean age of 32 years, 4 had severe thrombocytopenia while 4 had moderate thrombocytopenia when eltrombopag was started. All patients had received high dose IV steroids, followed by oral steroids. All patients had inadequate response to conventional immunosuppressants in varied combinations without any improvement in platelet counts. Four patients had clinically significant bleeding manifestations. They were treated with eltrombopag 50 mg/day or 75 mg/day. Duration of treatment ranged from 1 week to 70 weeks. All patients responded to eltrombopag, earliest response was noted at 5 days. Only one patient with chronic resistant thrombocytopenia required long term treatment with eltrombopag. None of the patients reported any adverse events to eltrombopag.

Conclusion: Eltrombopag is a new drug in our arsenal for treatment of immunologically mediated thrombocytopenia. It is a rapidly effective, safe orally administered medication. It boosted the platelets quickly and produced sustained effect. It indirectly helped reduce doses of steroids and other immunosuppressants thereby decreasing their cumulative side effects.


  PC136 Lupus APS Sjogren's Syndrome Top


Tacrolimus induces remission in refractory and relapsing lupus nephritis by Decreasing P-glycoprotein expression and function on peripheral blood lymphocytes

Sukesh Edavalath, Vikas Gupta, Mohit Kumar Rai, Harshit Singh, Saurabh Chaturvedi, Durga P Misra, Vikas Agarwal; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: About 15-30% of Lupus Nephritis (LN) patients do not respond to first-line immunosuppressive therapy. P-glycoprotein (P-gp) mediated efflux of corticosteroids (CS) may contribute to the treatment unresponsiveness. Tacrolimus is a P-gp inhibitor and hence, may overcome this resistance.

Objectives: We aimed to study the response to Tacrolimus, along with the expression and function of P-gp on peripheral blood lymphocytes (PBL) in patients with refractory and relapsing proliferative (ISN/RPS Class III/IV) Lupus Nephritis.

Methods: We enrolled 12 refractory/relapsing proliferative LN patients (6 patients refractory to CYC based regimens and 6 patients with renal relapse) and treated them with CS + Tacrolimus (2-3 mg/day) for 6 months. Expression and function of P-gp on PBL was measured by flow cytometry (as relative fluorescence index, RFI) before and 3 months after Tacrolimus therapy. Renal response was assessed according to ACR response criteria after 3 and 6 months of Tacrolimus therapy. The data (median and IQR) was analysed using non-parametric tests.

Results: 8 out of 12 refractory/relapsing LN patients achieved renal response (5 partial response, PR and 3 complete response, CR) as early as 3 months after start of Tacrolimus therapy, and 11 patients achieved renal response (7 PR and 4 CR) at 6 months from start of Tacrolimus therapy. Proteinuria decreased from median urine protein creatinine ratio (UPCR) of 2.80 (2.00-3.40) at baseline to 1.20 (0.66-1.73) at 3 months (p < 0.001) and to 0.80 (0.19-1.30) at 6 months (p < 0.01) [Figure 1]. There was significant decrease both in P-gp expression [RFI, 3.33 (2.87-4.97) vs 2.03 (1.25-3.86), p < 0.05) and P-gp function (RFI, 55.7 (29.7-84.1) vs 26.8 (16.1-37.0), p < 0.01) after 3 months of Tacrolimus therapy. None of the patients developed any adverse effects except one who developed rise in serum creatinine after 4 months of therapy.



Conclusion: Tacrolimus achieves renal response in refractory/relapsing proliferative LN patients by overcoming P-glycoprotein mediated treatment unresponsiveness.


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Outcome of patients of Sjogren's syndrome coming to a tertiary care OPD

S Pandya, R Solanki, T Parikh; Vedanta Institute of Medical Sciences and Sheth VS Municipal Hospital, Ahmedabad, Gujarat, India

Background: Data on long term outcome of SS patients is scarce, at least from our country.

Objective: To study outcome of patients who have visited our OPD at least once.

Methods: Ours was a cross sectional observational study. Informed consent was taken. All patients' fulfilling AECG criteria for Sjogren's syndrome who have attended our clinic at least once and whose data is recorded in our clinic's data sheet were called up telephonically. Patients from 1/7/04 to 1/7/17 were included in the analysis. Those who had attended our clinic in the last one year were counted in ' regular' follow up and the rest were ' lost to follow up'. Descriptive statistics was used for analyzing demographic data.

Results: A total of 327 patients were included in the analysis [Table 1]. 206 could be contacted telephonically. 11 patients had died (5.5% from those contacted). From the baseline data of those who died, we could find their median age at presentation to us was 44, all were females, 10 were Ro positive and 8 La positive. Of those who were contacted, 74.7% were still taking allopathic treatment, 12.6% had withheld all treatment and the rest were on alternative therapy. Overall, 66% felt >50% global improvement (patient reported, subjective). Only 36.4% were on regular follow up.



Conclusion: While the baseline features of patients of Sjogren's syndrome presenting to our OPD matched those elsewhere, only about a third were on regular follow up over 13 years, about 2/3rds were still on allopathy with >50% global improvement and 5.5% had died during the period.


  PC138 Lupus APS Sjogren's Syndrome Top


Prevalence and clinical correlation of anti ribosomal P antibody in Indian lupus patients: A single center study

Dhaval Tanna, Muzaffar Bindroo, Gayatri Ekbote, Dhiren Raval, Natasha Negalur, Wasim Kazi, Naval Mendiratta, Shruti bajad, Vinay Singal, Rajiva Gupta; Medanta Hospital, Gurgaon, Haryana, India

Background: Anti-ribosomal P protein (anti-P) autoantibodies are considered to be specific for Systemic Lupus Erythematosus (SLE) and is seen in 6%-46% of cases. Various studies showed anti Ribosomal P antibody to be associated with the presence of nephritis, hepatitis, hemolytic anemia and neurological involvement. Some groups have disputed this relationship while others have confirmed it. Here we have tried to describe the prevalence and clinical association of anti ribosomal P antibodies in Indian lupus patients.

Methods: All SLE inpatients and outpatients in department Of Rheumatology from Sept.2015 to April 2017 were enrolled. ENA was assessed by strip based micro ELISA assay for 25 antigens (ENA- 25). Method: All SLE inpatients and outpatients attending department of Rheumatology and Clinical Immunology, from September 2015 to April 2017 were enrolled. Anti Ribosomal P antibody was studied as a part of ENA which was assessed by strip based micro ELISA assay for 25 antigens (ENA-25).We recorded clinical and laboratory profile of all patients and tried to correlate it with Anti Ribosomal P Antibody.

Result: Total number of patients enrolled in study were 305. Out of them, 20 patients had positive anti ribosomal P antibody [Prevalence: 6.55%].Clinical symptoms of SLE were compared between patients with and without anti ribosomal P antibody. Among all clinical features, Patients with Anti Ribosomal P Antibody had significantly high prevalence of Lymphadenopathy as compared to those without it [50% Vs 15.08%, p:0.0001]. Out of all laboratory parameters, Leucopenia was significantly high in those with anti Ribosomal P Antibody [35% Vs 9.47%, p:0.0005] [Table 1]. No significant association was found between presence of Anti ribosomal P antibody with Neuropsychiatric lupus, nephritis, hepatitis or hemolytic anemia as documented in previous studies.



Conclusion: Though considered to be highly specific for diagnosis of SLE, anti ribosomal P antibody has low prevalence in lupus patients. Presence of anti ribosomal P antibody was significantly associated with presence of lymphadenopathy and leucopenia. No association was found between anti ribosomal P antibodies and other features such as neuropsychiatric lupus as documented in previous studies.


  PC139 Lupus APS Sjogren's Syndrome Top


Prospective evaluation of factors associated with tuberculosis in systemic lupus erythematosus

P V A Deepika, L Rajasekhar, I R Varaprasad, S Appani, P Devarasetty; Department of Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background: Tuberculosis can occur during the course of lupus as a result of immune dysregulation due to disease activity or its therapy.

Aim: To study the disease or treatment variables affecting the occurrence of tuberculosis in lupus patients.

Methods: A prospective observational study was conducted between January 2016 and April 2017.Patients satisfying SLICC criteria for SLE with active tuberculosis were included as cases. Age, disease duration matched patients with two SLEDAI assessments 6 months apart, without tuberculosis, during same period were defined as controls. Demographic data,corticosteroid and immunosuppression received 6 months prior to onset of tuberculosis, SLEDAI at diagnosis of TB and 6 months before in cases,6 months apart in controls were noted. Clinical suspicion of TB was confirmed by either identification of organism,imaging, histopathology,physician discretion or any of the combination. Appropriate descriptive statistics were used. Cases and controls were compared for baseline SLEDAI, change in SLEDAI and daily steroid dose over six months using student T test or Mann Whitney U test as applicable. Any immunosuppression used in previous six months versus no immunosuppression was compared between cases and controls using chi square test.

Results: Baseline data of cases and controls were shown in [Table 1]. TB was diagnosed by imaging in 52%, identification of organism in 40% and histopathology in 8%. Forty nine percent had extra-pulmonary and 13% had disseminated tuberculosis. Simultaneous diagnosis of TB and SLE was made in 12 cases. These 12 cases with no prior immunosuppression had significantly higher mean (SD) SLEDAI, 18.25 (9.45) than cases developing tuberculosis while on treatment (8.88, 9.28) (p=0.004). SLEDAI increased by 2.8 (8.5) in cases who developed TB on follow-up (n=36) and decreased in controls by 5.7 (2.8) and this difference was significant (p= 0.002). The difference in daily steroid dose (mg) from baseline to 6 months was similar in cases (-6.1±12.4) and controls (-1.8±7.0) (p=0.07). Controls had more frequent use of any immunosuppressive in preceding six months compared to cases (p<0.001).



Conclusions: Disease activity is higher in newly diagnosed treatment naive lupus with concurrent tuberculosis. Incidence of tuberculosis is not associated with prior immunosuppression. Controlling disease activity may prevent tuberculosis.


  PC140 Lupus APS Sjogren's Syndrome Top


Urinary system abnormalities in systemic lupus erythematosus

S Nayan Patel, P K Devarasetty, I R Varaprasad, L Rajasekhar; Nizams Institute of Medical Sciences, Hyderabad, Telangana, India

Background: The common causes of urinary system involvement in SLE patients are urinary tract infection (UTI), cyclophosphamide-induced hemorrhagic cystitis, transverse myelopathy, tuberculosis, and obstructive uropathy caused by nephrolithiasis. Lupus cystitis and hydroureteronephrosis caused by edema or chronic fibrosis of bladder wall and vesicoureteral junction is under-reported.

Objectives: To investigate the demographic and clinical profile of lupus patients with hydroureteronephrosis and/or cystitis.

Methods: A retrospective analysis was performed by screening the discharge summaries of all SLE patients admitted between January 2006 and June 2017. All patients with imaging (CT or US) evidence of cystitis (bladder wall thickening) or hydroureteronephrosis were studied. Those with renal calculi, external ureteric compression causing hydroureteronephrosis and UTI were excluded. Demographic and clinical profile were studied. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was calculated from available parameters.

Results: Lupus cystitis and or hydroureteronephrosis was noted in 16 patients. Thirteen were females. USG was available in 13 patients, CT in 11 and both USG and CT in 8. Mean (SD) age was 25.3 (± 8.2) year. SLE disease duration prior to development of hydroureteronephrosis was a median 2.0 years, range (1 to 94 months). Presenting symptoms in order of frequency were pain abdomen (10/16), vomiting (5/16) and diarrhoea (2/16). No patient had flank pain, only one had hematuria, dysuria and frequency. Concomitant lupus enteritis was present in 6/16 patients and serositis (ascites) in 3/16 patients. Nine had bilateral and 6 unilateral hydroureteronephrosis. Cystitis with hydroureteronephrosis was observed in 5/16 patients and one had cystitis without hydroureteronephrosis. Concomitant lupus nephritis was seen in 6/16 patients. Mean SLEDAI was 13.1 (± 5.9) and median ESR was 50, range (2 to 130). Elevated serum creatinine was found in 4/16 patients, which normalised in all during hospitalisation.

Conclusions: Urinary tract involvement in lupus may not be as rare as has been thought. Our findings suggest gastrointestinal symptoms were the most common presenting manifestations in lupus patients with urinary tract involvement.


  PC141 Lupus APS Sjogren's Syndrome Top


Clinico-pathological correlation of renal biopsy in lupus

Avinash Jain, Rutviz Mistry, Amita Aggarwal; SGPGI, Lucknow, Uttar Pradesh, India

Introduction: Nephritis is a major cause of morbidity and mortality in lupus. Renal biopsy, though a gold standard, is not feasible in all cases, more so in resource poor countries. We, therefore studied if clinical, serological and biochemical parameters could predict class of lupus nephritis (LN).

Methods: All patients with lupus who underwent a kidney biopsy during one year (2016-2017) were included. Data on Clinical features, anti-dsDNA, C3, C4, APLA profile, creatinine, urine analysis and renal biopsy were retrieved. A short case summary with findings of each case was mailed to rheumatologists across country to predict the class of nephritis. Results were analysed by dividing the classes into Class I-II, III-IV and V. Presence or absence of crescents was also assessed.

Results: Total 30 patients with mean age 29.2 ± 9.6 years were included. Among 30 patients, 19 had Class III-IV with/without membranous changes, 7 had Class I-II and 4 had class V disease. 22 rheumatologists across India including 3 fellows in training with varying experience from 2 years to more than two decades responded and accuracy of prediction was assessed. Results are summarised in [Table 1].



Conclusion: It is possible to predict proliferative nephritis with or without membranous nephropathy with about 79% accuracy.


  PC142 Lupus APS Sjogren's Syndrome Top


Profile of haematological abnormalities in sle patients from a tertiary care centre in South India

Anuja Rajan, Thiruchengode Natesan Tamilselvam, Selvakumar Balameena, Eupharasia Latha, Myilasamy Saravanan, M Rajavel, S Sreedevi; Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: SLE is a chronic autoimmune disorder which can involve many organ systems of the body. Most of the patients with SLE develop haematological abnormalities at some point during the course of the disease

Objective: The aim of this study is to evaluate the frequency and pattern of haematological abnormalities in patients with SLE at diagnosis and during disease flare and to explore the organ systems and auto antibodies associated with haematological abnormalities in these patients.

Methods: An observational retrospective study was conducted on 286 patients who were admitted with SLE or its complications at institute of rheumatology, MMC Chennai from 2015-2017. Clinical manifestations, organ involvement, haematological parameters, markers of disease activity and antibody profile of the patients were recorded. The data was analysed using SPSS software and the association between haematological manifestation, antibodies and organ involvement was assessed.

Results: Of the 286 patients with SLE, anaemia was the most frequent haematological abnormality with an incidence of 88.9% at diagnosis and 78.4% with flare. Leucopenia was seen in 25% of patients at diagnosis and 20.1% patients with disease flare. Thrombocytopenia was seen in 28.5 % at first visit and 28.1% at flare and was associated with antihistone antibodies. Autoimmune haemolytic anaemia was present in 24.8% of patients with anaemia and was associated with spleenomegaly and antibodies to ribosomal p. TTP was observed in 4 patients. Anaemia, thrombocytopenia and pancytopenia were associated with high disease activity. Leucopenia was associated with acute lupus skin lesions and musculoskeletal manifestations.

Conclusion: Haematologic abnormalities were present in a considerable number of patients at diagnosis and flare and correlated with high disease activity in these patients. Evaluation of a patient with SLE should include evaluation of disease activity, complements and autoantibodies like antihistone and ribosomal P antibodies. TTP should be considered during evaluation of SLE patients with thrombocytopenia.


  PC143 Lupus APS Sjogren's Syndrome Top


Retinal disease in systemic lupus erythematosus patients: A restrospective analysis

Pratyusha Rajavarapu, Phani Kumar Devarasetti, I R Varaprasad, Liza Rajasekhar; Nizams Institute of Medical Science, Hyderabad, Telangana, India

Introduction: Retinal involvement in SLE is seen in 7–26 % of cases. This vision threatening manifestation is underreported in India.

Objectives: To study the demographic and clinical data in patients with SLE retinopathy and its correlation with disease activity elsewhere.

Methods: Discharge records of patients satisfying ACR or SLICC criteria for SLE from 2006 to 2017 were screened. Patients with visual symptoms and evidence of retinopathy on fundoscopy as demonstrated by an ophthalmologist were included. Patients with diabetic and infectious retinopathy were excluded. Retinal involvement was classified as mild, moderate and severe1,2. Demographic data and clinical characteristics were noted.

Results: Twenty one cases of SLE retinopathy were identified of which females were 16. Mean (SD) age at retinopathy onset was 26.611.4 years. Median duration of retinopathy manifestation after onset of SLE was 3 months (ranging from 0 to 42). Mean ESR was 71.4733.6 mm in 1st hour. Mean (SD) SLEDAI was 23.56.4. 9 cases had bilateral eye involvement. CNS involvement was seen in 11 and nephritis in 9 cases. Retinopathy was mild to moderate in 12 and severe in 9 cases1,2. All severe cases with retinal venous or arterial thrombosis had medium titre aCL positivity. Anti dsDNA positivity was seen in 19 cases. aCL was positive in 11 of 19 patients tested. Hypocomplementemia was seen in 10 of 16 tested.

Conclusion: Retinopathy occurs earlier in India in SLE patients and is often bilateral3. One third of all cases had severe retinopathy and was associated with CNS involvement, presence of Anti ds DNA antibodies, hypocomplementemia and high SLEDAI.

References

  1. Sivaraj RR, Durrani OM, Denniston AK, Murray PI, Gordon C. Ocular manifestations of systemic lupus erythematosus. Rheumatology (Oxford) 2007;46:1757-62.
  2. Yen YC, Weng SF, Chen HA, Lin YS. Risk of retinal vein occlusion in patients with systemic lupus erythematosus: A population-based cohort study. Br J Ophthalmol 2013;97:1192-6.
  3. Ushiyama O, Ushiyama K, Koarada S, Tada Y, Suzuki N, Ohta A, et al. Retinal disease in patients with systemic lupus erythematosus. Ann Rheum Dis 2000;59:705-8.





  PC144 Lupus APS Sjogren's Syndrome Top


To study the efficacy of methotrexate in inflammatory synovitis in systemic lupus erythematosus patients patients

S Verma, S Bhalla, Q Zaheer, A N Malaviya; A&R Clinic™ and Department of Rheumatology, Indian Spinal Injuries Centre, New Delhi, India

Background: SLEDAI is the recommended tool for assessing SLE patients. Generally, MTX is not considered for inflammatory synovitis in them. No separate tool is available for evaluating joints in this disease.

Aims: 1. To study the efficacy of MTX in inflammatory synovitis among SLE patients. 2. To assess the validity of Clinical Disease Activity Index (CDAI) and DAS28 for assessment of arthritis among SLE patients.

Methods: 20 patients with SLE, classifiable by Systemic Lupus International Collaborating Clinics (SLICC) criteria were included with predominantly inflammatory polyarthritis of rheumatoid pattern. These patients were prescribed MTX. Disease activity in the joints was assessed at baseline and at follow-up. The values were compared statistically. It is to be noted that "arthritis" as used for SLEDAI does not give weightage to the number of affected joints and the results are only dichotomous. Therefore, its sensitivity for change may be questioned. As an alternative we used CDAI and DAS28; the two instruments that include the number of joints and give results as continuous variable.

Results: Out of 20 patients, assessed with DAS28, 4 (20%) were in remission (REM) or LDA and 16 (80%) were in moderate or high disease activity (MDA or HDA). When assessed with CDAI, 9 (45%) were in REM or LDA and 11 (55%) were in MDA or HDA. Post treatment in follow up, using DAS28, 19 (95%) were in REM or LDA and 1 (5%) were in MDA or HDA. Using CDAI, 20 (100%) were in REM or LDA. The difference in patients with MDA/HDA at baseline as compared to those at the last visit was statistically was highly significant (p< 0.01).

Conclusion: MTX is a highly effective drug for treating lupus synovitis. 2. CDAI could be an ideal instrument to assess inflammatory synovitis sequentially in the follow up in SLE. A similar approach has been used to assess joints in sclroderma1.

References

  1. Gordon JK, Girish G, Berrocal VJ, Zhang M, Hatzis C, Assassi S, et al. Reliability and validity of the tender and swollen joint counts and the modified rodnan skin score in early diffuse cutaneous systemic sclerosis: Analysis from the prospective registry of early systemic sclerosis cohort. J Rheumatol 2017;44:791-4.





  PC145 Lupus APS Sjogren's Syndrome Top


Clinical profile of osteonecrosis in systemic lupus erythematosus: Experience from a tertiary care centre in South India

Saranya Chinnadurai, T N Tamilselvam, M Saravanan, Euphrasia Latha, S Mythili, M Vignesh, S Sreedevi, A Aravindan; Institute of Rheumatology, MMC and RGGGH, Chennai, Tamil Nadu, India

Background: Osteonecrosis or Avascular necrosis of bone (AVN) is characterised by death of the cellular elements of bone. It is a well recognised complication of systemic lupus erythematosus (SLE) and leads to significant morbidity.

Objectives: To investigate the clinical profile of osteonecrosis in patients with systemic lupus erythematosus and to investigate the various variables including steroid dose and duration.

Methods: A retrospective analysis was done in a cohort of SLE patients who are on regular follow up at our Rheumatology OPD over a period of 5 years from 2012-2017. Osteonecrosis was diagnosed based on thorough clinical evaluation and imaging studies (CT/ MRI). Clinical profiles of SLE patients with osteonecrosis were analysed. MRI Staging was done using Modified Steinberg staging system.

Results: Among 415 SLE, 21 (5.1%) patients were diagnosed to have osteonecrosis. The mean age of the patients was 32.8 + 7.6 years. Male: female were 1:4.2. Mean disease duration of SLE was 6.6+3.4 years and mean disease duration of AVN was 2.5 + 1.9 years. The average interval between the onset of SLE and diagnosis of AVN was 4.1 + 2.7years. 61.9% (13/21) had involvement of bilateral femoral head followed by 33.3% (7/21) with involvement of one site either unilateral femoral head (4/7) or distal femur (3/7). 3 patients (14.3%) had multifocal involvement. 2 patients received core decompression surgery, while 2 patients underwent total hip replacement. The most common stage at diagnosis was modified steinberg stage IV (38%), followed by 24% stage V, 19% stage III and 9.5% each stage II and VI. Antiphospholipid antibodies were positive in 28.5% (6/21) of patients. The mean daily dose of prednisolone at the time of diagnosis of AVN was 8.5mg (range 5-20 mg). Comorbidities associated with osteonecrosis include hypertension 19% (4/21), hypothyroidism 9.5% (2/21) and osteoporosis 24% (5/21). One of the patients had chronic HCV infection and received antiviral therapy.

Conclusion: This study highlights the clinical profile of AVN and the factors that predispose SLE patients to osteonecrosis. Hence, high index of suspicion is required to identify AVN in SLE for early intervention.


  PC146 Lupus APS Sjogren's Syndrome Top


Correlation of different antinuclear antibodies with various manifestations in SLE patients

Hepciba Glory Boyidi, Chanchal Gera, Navjot Singh; Christian Medical College, Ludhiana, Punjab, India

Background: Systemic lupus erythematosus is a chronic multisystem autoimmune disorder mainly affecting young women. In SLE patients among various antinuclear antibodies, 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 role of these autoantibodies in pathogenesis of SLE including their role in variable presentation of SLE.

Aims and Objective: To study correlation of different antinuclear antibodies (ANA) with various manifestations of SLE patients.

Methods: Sixty three patients diagnosed as SLE by fulfilling Systemic Lupus International Collaborating Clinics (SLICC) 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 thirty five patients and correlation of different ANAs with various manifestations of SLE was studied. Analysis was done using SPSS version 21.0. Qualitative variables were correlated using Chi-Square test /Fisher’s exact test. A p value of <0.05 was considered statistically significant.

Results: dsDNA was significantly associated with prolonged fever (p value 0.0348). Anti SS-A/Ro60 was most common antibody and found in 80% of our patients. DCT positivity was significantly associated with ds DNA (p value 0.039) and anti SS-A/Ro60 (p value 0.021). Anti SS-B/La has shown positive correlation with anemia (p value 0.033) and thrombocytopenia (p value 0.011) and negative correlation with photosensitivity (p value 0.004). Proteinuria in our SLE patients was significantly associated with anti nucleosome antibodies (p value 0.015) and antihistone antibodies (p value 0.007). Significant association is noted between antinucleosome antibodies and non scarring alopecia (p value 0.018) as well. Differently SmD1 antibodies noted to have statistical significance to polyarthritis (p value 0.018) and splenomegaly (p value 0.044). Incidence of hypothyroidism was more in patients who had antihistone antibodies (p value 0.033).

Conclusion: The correlations of specific autoantibodies and clinical manifestations could provide clues to clinicians to predict various organ dysfunctions in SLE patients. 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.


  PC147 Lupus APS Sjogren's Syndrome Top


Prevalence and spectrum of cardio-vascular disease in lupus patients

G Amirtha, Gomes Felly, Divya Balakrishnan, Vineeta Shobha, Srilakshmi; Departments of Clinical Immunology and Rheumatology and 1Cardiology, St. John's Medical College and Hospital, Bengaluru, Karnataka, India

Background: Involvement of the heart and pulmonary vessels has been found in several clinical and autopsy studies in patients with SLE, most of which can be detected by echocardiography. More than half of lupus patients experience clinical cardiovascular manifestation during the course of their illness.

Objectives: To determine the prevalence and spectrum of cardiac abnormalities, determined by echocardiography in SLE patients at St. Johns Medical College and Hospital.

Methods: This is an ongoing retrospective study (January 2015 onwards) conducted on newly diagnosed SLE patients fulfilling (EULAR/ACR criteria 2012) presenting to SJMCH. A total of 230 SLE patients were sampled consecutively over a period of 18 months, of which ECHO reports were available in 77 patients. The echocardiogram outcome variables include; pericardial effusion, Libmann Sach endocarditis, valvular thickening and calcification, valvular regurgitations, pulmonary hypertension, diastolic dysfunction. Data obtained will be analysed using SPSS software (version 22).

Results and Conclusion: This study demonstrates a high prevalence (45/230) of cardiac abnormalities among SLE patient at presentation. Results so far have shown that majority of cardiac abnormalities comprise clinically insignificant asymptomatic pericardial and valvular thickening. Table 1: Prevalence of cardio-vascular disorders in SLE patients (n=77). CVS Disorder - Prevalence (%) Mitral Regurgitation - 25 Aortic Regurgitation - 11 Tricuspid Regurgitation - 30 Pericardial Effusion - 9 RWMA - 3 Pulmonary Artery Hypertension - 14 Diastolic Dysfunction - 11 Systolic Dysfunction - 5 Libman Sachs - 3 All patients were treated with steroids and immuno-suppressants according to disease severity. The prevalence of valvular insufficiency and Libmann Sach endocarditis are substantially high, this mandates a careful scrutiny of cardiovascular status in all patients with SLE even if they do not have clinically overt abnormalities. Treatment and outcome results shall be discussed. Association with other co-morbid illnesses needs to be studied.


  PC148 Lupus APS Sjogren's Syndrome Top


To Study autoantibody profile; its clinical correlation and disease activity in Systemic lupus erythematosus in North-East India

Naman Jain, Sanjeeb Kakati, Rebecca R Marak, Diganta Das; Assam Medical College, Dibrugarh

Background: systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with numerous patterns of clinical and immunological manifestations. Even though the presence of autoantibodies in SLE has been known, for more than 60 years, still nowadays a great effort is being made to understand the pathogenetic, diagnostic, and prognostic meaning of such autoantibodies.

Objective: To study autoantibody profile in SLE. To study correlation of various autoantibody with clinical presentation and disease activity of SLE.

Methods: An observational study of 129 patients with SLE who fulfiled ACR criteria (outdoor/indoor patients) for a duration of one year were further analysed. Antinuclear antibody assay-assessment by Immunoflorescent study, ANA profiles were determined by line immunoassay and disease activity at presentation was evaluated by SLEDAI Score and an An extensive list of signs/symptoms was evaluated.

Results: 96.12% of patients were below 40 years of age with Mean age 25.97 +/- 7.96, 95.32% of patients are female and 4.68% were male. Alopecia (73.64%), Fatigue (68.99%), renal involvement (64.34%), hematological {Anaemia} (62.02%), arthritis (54.26%), Fever (41.09%) are the common clinical presentation of the patients. The frequencies of the specific antibodies: dsDNA (58.91%), Anti Ro (60 kDa) (44.96%), Antihistone (38.76%) Anti Ro 52 kDa (37.98%), AntiSm D1 (27.13%), antinucleosome (23.26%), Anti PCNA (5.43%), Anti RibosomalP (27.13%), U1snRNP (16.2%), Anti La (13.9%), CENP-B(0.78%), Scl-7 (1.5%), AMA M2(3.1%), Anti Pm Scl (3.1%), Mi-2 (1.5%), Ku (1.5%), anti jo-1 (0%). There was significant correlation found between Alopecia [Anti Ro (60k Da) and Anti Rib-p] Malar rash (ANti La, and anti scl -70), Discoid Rash (Anti Rib-p and Anti Mi-2), SCLE (Anti-SCL-70), Mucosal ulcer (Anti NuA), Arthritis (Anti Rib P, Anti NuA) Proteinuria (Anti dsDNA, Anti NuA,Anti histone and anti SmD1 antibody), Seizure (Anti Rib-p and Anti NuA), Anemia (Anti dsDNA, AntiNuA, Anti histone), Raynaud’s phenomena (Anti U1snRNP and Anti Scl-70) and skin tightening (Anti Scl-70), Pericardial Effusion (Anti NuA, AntiPCNA, Anti Ku) Respiratory symptoms cough (Anti dsDNA, Anti NuA, Anti histone and pleural effusion- (Anti NuA and Anti Pm-SCl) and more.

Conclusion: In this various antibodies were studied in Assam Medical college and hospital, Dibrugarh, Assam. Previously reported associations of antibodies with clinical symptoms of SLE were confirmed and also found several new associations with clinical symptom and disease activity.


  PC149 Lupus APS Sjogren's Syndrome Top


Determinants of delayed diagnosis and disease course in Primary Sjogrens syndrome: Results from data mining of electronic health records

Pulukool Sandhya, Janardana Ramya, Thambu David1, Gowri Mahasampath2, John Antony Jude Prakash3, Debashish Danda; Departments of Rheumatology, 1Medicine, 2Biostatistics and 3Microbiology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Primary Sjogren's syndrome (pSS) is one of the autoimmune diseases with a significant delay in diagnosis. Factors responsible for the delay in diagnosis have not been extensively evaluated. Electronic health records (EHR) could be a unique resource to understand these factors as well as course of the disease.

Methods: EHR of patients diagnosed as pSS between January 2008 and October 2015 were reviewed. Patients were screened by tracing reports of anti-SSA antibody assay and minor salivary gland histologies. Only patients fulfilling the 2016 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for pSS were included. Variables including the details of the specialities of first consultation by the patient, initial clinical presentations, investigations ordered at first visits, number of hospital consultations prior to reaching the final diagnosis of pSS and the baseline EULAR SS Disease Activity Index (ESSDAI) were noted. Multiple logistic regression was used to identify predictors of correct diagnosis of pSS at the first visit.

Results: A total of 275 patients sought their first consultation with varying range of presenting features to 24 different specialities; rheumatology accounted for 128 (46.55%) patients as the first contact speciality. Overall, pSS was the initial impression only in 133/275(48.4%) patients; even the rheumatologists could make an initial diagnosis of pSS in only 83 out of 128 (64.84%) patients. Median (range) number of hospital visits prior to confirmed diagnosis of pSS amongst those suspected versus not suspected of the disease at first consultation were 1(1-6) and 3 (1-14), respectively (p<0.001). In multivariate analysis, a first impression of pSS, enquiry about sicca symptoms and ordering anti-SSA antibody test at first consultation were strong predictors of early diagnosis of pSS with odds (95%CI) of 5.01 (1.72,14.55) p<0.001, 4.79 (1.16,19.84) p=0.03 and 9.60 (3.00,30.67) p<0.0001,respectively. Presenting complaints, ESSDAI and duration of disease prior to presentation did not influence the time to diagnosis.

Conclusions: To the best of our knowledge, this is the first large-scale study of data mining using EHR towards recognising patient profiles and factors influencing time to diagnosis in pSS.


  PC150 Lupus APS Sjogren's Syndrome Top


SLE male versus female. How different is it?

S Mythili, T N Tamilselvam, S Balameena, M Saravanan, J Euphrasia Latha, C Saranya, M Vignesh, A Aravindan, S Sreedevi; Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Systemic lupus erythematosus (SLE) though predominantly a disease of female it is also seen in men, with a female to male ratio of 8 to 9:1. Lupus in men present with characteristic clinical features and outcome with increased severity.

Objective: To study the clinical features and disease activity in male SLE patients.

Methods: STUDY TYPE: Retrospective observational study PERIOD: 5 MONTHS (FEB2017-JULY2017) STUDY POPULATION: 36 male SLE Patients and 36 age matched female SLE Patients diagnosed by ACR criteria and attending Lupus clinic were included. In the study population data on duration of disease, clinical features at onset of disease, clinical course of the disease, treatment and disease activity were analysed.

Results: Mean age of male and female SLE patients 24.5±7.5Years [Table 1]. Duration of disease in males was 2 years IQR (1,4). In males Baseline median SLEDAI 14 IQR (10,20) and follow up SLEDAI 4 IQR(2,6). Other clinical features like malar rash, alopecia, palatal ulcer, musculoskeletal, myositis, arthritis, serositis, pericardial effusion, pleural effusion, pulmonary hypertension, chronic ILD, pneumonitis, hematological, Anemia, thrombocytopenia, leucocytopenia, neurological, psychiatry,v asculitis, hepatosplenomeghaly, lymphadenopathy, gastrointestinal manifestations did not vary significantly between male and female SLE patients.



Conclusion: In this comparative study, 13.8% of male patients had high incidence of vasculitis particularly retinal vasculitis and 8.3% developed avascular necrosis during the course of the disease. The Discoid lupus lesions, lupus nephritis and loss of weight in males were significant initial presentations. Male SLE patients typically had a persistently raised SLEDAI even after treatment compared to female counterparts.


  PC151 Lupus APS Sjogren's Syndrome Top


Hematological manifestations and their correlation with serology in Indian lupus patients: A single center study

Dhiren Raval, Muzaffar Bindroo, Gayatri Ekbote, Natasha Negalur, Wasim Kazi,Dhaval Tanna Naval Mendiratta, Shruti Bajad, Vinay Singal, Rajiva Gupta; Medanta the Medicity, Gurgaon, Haryana, India

Background: Hematological abnormalities are frequently seen in SLE. Recent studies have shown that there is a positive correlation of anti-dsDNA, anti-RO and Acl Ig G with cytopenia. There is a scarcity of studies from India addressing the association of hematological involvement with serology in SLE. We have tried to correlate hematological manifestation with serology in Indian lupus patients.

Objectives: To study hematological manifestation and their correlation with serology in Indian lupus patients.

Methods: All SLE inpatients and outpatients in Department Of Rheumatology from Sept.2015 to April 2017 were enrolled. ENA was assessed by strip based micro ELISA assay for 25 antigens(ENA-25) and APLA profile was done in all patients. Hematological manifestations like anemia, leucopenia and thrombocytopenia were recorded and correlated with ENA and APLA profile.

Result: The total number of patients enrolled in the study was 305.Hematological involvement was seen in 132/305 (43.3%) [Table 1]. Out of 132, 105 were females and 27 were males. Mean age was 35 years. Direct Coombs Test was positive in 21 /62 of anemia patients Indirect Coombs Test was positive in 4 patients. Anemia has statistically significant correlation with Anti nucleosome antibodies (p-0.019). Leucopenia has statistically significant correlation with Anti Ribosome P0 antibodies (p-0.0002) Thrombocytopenia has statistically significant correlation with Anti â€" B2GP1 Ig G antibodies (p-0.036) Pancytopenia had no significant correlation with serology.



Conclusion: Hematological involvement is common in SLE; anemia being the most common (20.3%) followed by Thrombocytopenia and leucopenia. We found that there is a significant positive correlation of anemia, leucopenia and thrombocytopenia with anti-Nucleosome, anti-Ribosome P0 and anti-B2GP1Ig G respectively. We need to have larger studies to prove this correlation in Indian population.


  PC152 Lupus APS Sjogren's Syndrome Top


Dyslipidemia in systemic lupus erythematosus: Effect of hydroxyxhloroquine and duration of disease

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

Purpose: To study lipid abnormalities in systemic lupus erythematosus (SLE);to correlate with disease activity,duration of disease and to determine the effect of hydroxychloroquine on lipid profile.

Methods: Fasting lipid profiles were performed in 72 SLE patients who attended Rheumatology OPD in a tertiary care hospital of north-east India. Those with Diabetes Mellitus, Coronary Artery Disease, Statin therapy were excluded from the study. Disease Activity was measured by SLEDAI. Statistical analysis was performed with IBM SPSS Statistics version 21 Software. p- value less than or equal 0.05 was considered as significant.

Results: Dyslipidemia was found in 44 (66.1%) patients. Hypertriglyceridemia being the commonest found in 30 (41.7%) followed by LDL abnormality in 17 (26.6%) and HDL in 13(18.1%).A significant statistical association (p=0.034) was seen between hypertriglyceridemia and disease duration ≤5yrs (93.3%) as compared to the group >5 yrs (6.7%).Also a statistically significant mean difference (p= 0.001) was seen in TG value between those with disease duration ≤5yrs and >5yrs. However no any significant statistical association was seen between hydroxychloroquine use and the values of LDL, TG, HDL and TC.

Conclusion: Hypertriglyceridemia being the commonest lipid abnormality in SLE patients during the early stage of the disease, SLE patients are at increased risk of premature atherosclerosis and coronary artery.


  PC153 Lupus APS Sjogren's Syndrome Top


Correlation of Vitamin D levels with disease activity in patients of systemic lupus erythematosus with nephritis

Shekhar Jyoti Gogoi, Chayanika Dutta, L Saikia1; Departmentas of Medicine and 1Microbiology, Assam Medical College, Dibrugarh

Introduction: Deficiency of vitamin-D is prevalent worldwide and more so in some autoimmune diseases like SLE as per studies. It may be due to early involvement of the kidneys in the disease process, photosensitivity and sunscreen-use led less sun exposure, or chronic use of certain drugs. Studies also point that there is an inverse correlation between Vitamin-D and the disease activity of SLE.

Methods: 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 comprising of 109 SLE patients and 109 age and sex-similar healthy volunteers. Vitamin-D level was assessed by MINI VIDAS, 25-0H Vitamin D Total (ELFA).For disease activity of SLE we used SLEDAI score. Presence of nephritis was assesses by routine urine examination.

Results: 58.6% cases with nephritis, 45.4% patients without nephritis and 37.61% of healthy population were vitamin D deficient, which was clinically significant [OR=5.1 p=0.02]. In SLE cases with Nephritis, a significant negative correlation was found between Vitamin D levels with SLEDAI scores (r= -0.24, p value <0.05), while cases no significant correlation was observed non nephritis SLE patients (r= -0.05, p value=0.82). When analyzed for association of deficiency serum Vitamin D with the degree of proteinuria, all (100%) of the patients with ‘++++’ were vitamin D deficient, 58.82% of ‘+++’ were vitamin D insufficient and 31.58% with ‘++’ were vitamin D deficient (OR=4.5 p=0.034).

Conclusion: Vitamin-D has a great role in immunosuppression and being easily available and relatively non-toxic, it can be of great help as an immunomodulator in suppressing the disease activity of SLE.


  PC154 Lupus APS Sjogren's Syndrome Top


To study the efficacy of methotrexate in inflammatory synovitis in systemic lupus erythematosus patients patients

S Verma, S Bhalla, Q Zaheer, A N Malaviya; A&R Clinic™ and Department of Rheumatology, Indian Spinal Injuries Centre, New Delhi, India

Background: SLEDAI is the recommended tool for assessing SLE patients. Generally, MTX is not considered for inflammatory synovitis in them. No separate tool is available for evaluating joints in this disease.

Aims: 1. To study the efficacy of MTX in inflammatory synovitis among SLE patients. 2. To assess the validity of Clinical Disease Activity Index (CDAI) and DAS28 for assessment of arthritis among SLE patients.

Methods: 20 patients with SLE, classifiable by Systemic Lupus International Collaborating Clinics (SLICC) criteria were included with predominantly inflammatory polyarthritis of rheumatoid pattern. These patients were prescribed MTX. Disease activity in the joints was assessed at baseline and at follow-up. The values were compared statistically. It is to be noted that "arthritis" as used for SLEDAI does not give weightage to the number of affected joints and the results are only dichotomous. Therefore, its sensitivity for change may be questioned. As an alternative we used CDAI and DAS28; the two instruments that include the number of joints and give results as continuous variable.

Results: Out of 20 patients, assessed with DAS28, 4 (20%) were in remission (REM) or LDA and 16 (80%) were in moderate or high disease activity (MDA or HDA). When assessed with CDAI, 9 (45%) were in REM or LDA and 11 (55%) were in MDA or HDA. Post treatment in follow up, using DAS28, 19 (95%) were in REM or LDA and 1 (5%) were in MDA or HDA. Using CDAI, 20 (100%) were in REM or LDA [Figure 1]. The difference in patients with MDA/HDA at baseline as compared to those at the last visit was statistically was highly significant (p< 0.01). Conclusion: 1. MTX is a highly effective drug for treating lupus synovitis. 2. CDAI could be an ideal instrument to assess inflammatory synovitis sequentially in the follow up in SLE. A similar approach has been used to assess joints in sclroderma1.



References

  1. Gordon JK, Girish G, Berrocal VJ, Zhang M, Hatzis C, Assassi S, et al. Reliability and validity of the tender and swollen joint counts and the modified rodnan skin score in early diffuse cutaneous systemic sclerosis: Analysis from the prospective registry of early systemic sclerosis cohort. J Rheumatol 2017;44:791-4.



  PC155 Lupus APS Sjogren'S Syndrome Top


Determinants of delayed diagnosis and disease course in South Asians with primary Sjogren's syndrome: Results from data mining of electronic health records in a tertiary care centre

Pulukool Sandhya, Janardana Ramya, Thambu David1, Gowri Mahasampath2, John Antony Jude Prakash3, Debashish Danda; Departments of Rheumatology, 1Medicine, 2Biostatistics2 and 3Microbiology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Primary Sjogren's syndrome (pSS) is one of the autoimmune diseases with a significant delay in diagnosis. Factors responsible for the delay in diagnosis have not been extensively evaluated. Electronic health records (EHR) could be a unique resource to understand these factors as well as course of the disease.

Methods: EHR of patients diagnosed as pSS between January 2008 and October 2015 were reviewed. Patients were screened by tracing reports of anti-SSA antibody assay and minor salivary gland histologies. Only patients fulfilling the 2016 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for pSS were included. Variables including the details of the specialities of first consultation by the patient, initial clinical presentations, investigations ordered at first visits, number of hospital consultations prior to reaching the final diagnosis of pSS and the baseline EULAR SS Disease Activity Index (ESSDAI) were noted. Multiple logistic regression was used to identify predictors of correct diagnosis of pSS at the first visit.

Results: A total of 275 patients sought their first consultation with varying range of presenting features to 24 different specialities; rheumatology accounted for 128 (46.55%) patients as the first contact speciality. Overall, pSS was the initial impression only in 133/275(48.4%) patients; even the rheumatologists could make an initial diagnosis of pSS in only 83 out of 128 (64.84%) patients. Median (range) number of hospital visits prior to confirmed diagnosis of pSS amongst those suspected versus not suspected of the disease at first consultation were 1(1-6) and 3 (1-14), respectively (p<0.001). In multivariate analysis, a first impression of pSS, enquiry about sicca symptoms and ordering anti-SSA antibody test at first consultation were strong predictors of early diagnosis of pSS with odds (95%CI) of 5.01 (1.72,14.55) p<0.001, 4.79 (1.16,19.84) p=0.03 and 9.60 (3.00,30.67) p<0.0001,respectively. Presenting complaints, ESSDAI and duration of disease prior to presentation did not influence the time to diagnosis.

Conclusions: To the best of our knowledge, this is the first large-scale study of data mining using EHR towards recognising patient.


  PC156 Lupus APS Sjogren's Syndrome Top


HRCT findings in pulmonary manifestations of connective tissue diseases

Amal Basnet, Lalit Duggal, Neeraj Jain, Bhandari Gurbir, Jeet Patel; Sir Ganga Ram Hospital, New Delhi, India

Background: Connective tissue diseases (CTDs) comprise a broad spectrum of systemic autoimmune diseases which can potentially impact any organ system, with the lungs being the common target; and majority of the patients being at high risk for development of clinically significant lung disease.

Objective: The objective of this study is to evaluate the HRCT findings of patients presenting with pulmonary manifestation in CTD.

Methods: All patients of CTD with lung involvement attending inpatients and outpatients in Rheumatology department at Sir Ganga Ram Hospital were recruited. The duration of the study was 6 months. The demographic and clinical profiles along with the laboratory data and radiological findings were collected. HRCT findings were expressed in terms of pleural, parenchymal and airways involvement.

Results: This study included 20 cases (15 females and 5 males) with ages ranging from 33 to 70 years (mean 52.95). The duration of the disease ranged from 2 to 27 years. The most common presentation was cough (75%) followed by dyspnea (70%). Rheumatoid arthritis was the most common disease (30%) followed by Scleroderma (25%) Sjogrens (25%), SLE (10%), MCTD (5%), UCTD (5%), and LD-CTD (5%). Ground glass opacification was the most common parenchymal abnormality noted in 70% of the patients while septal thickening and peribronchial thickening were noted in 50%. Bronchiectasis was the most common airway disease which presented in 45% of the cases while emphysema and bronchiolectasis were present in 10%. Pleural thickening was found in 35% while pleural effusion was found in 15% of the patients. Thickened fissures and honeycombing were noted in 30% and parenchymal bands and pulmonary nodules in 25% of the patients. Pulmonary hypertension was present in 25% and oesophageal dilatation and lymphadenopathy were present in 20% of the cases. UIP type pattern was seen in 30% and NSIP pattern was seen in 70%. Osteoporosis and calcification had lowest prevalence each occurring in 5% of patients.

Conclusion: Any part of the respiratory can be involved in different CTDs with ground glass opacification, septal thickening and peribronchial thickening being most commonly noted manifestation.


  PC157 Vasculitis Top


Poor obstetric outcomes in North Indian women with Takayasu's arteritis

Latika Gupta, Durga Prasanna Misra, Sakir Ahmed, Avinash Jain, Abhishek Zanwar, Amita Aggarwal, Vikas Agarwal, Able Lawrence, Ramnath Misra; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Objectives: Takayasu's arteritis (TA) affects young women in the childbearing age group. We studied obstetric outcomes in these patients before and after disease onset.

Methods: Women aged more than 18 years with Takayasu's arteritis (ACR 1990 criteria) were included. Demographic data, clinical features, menstrual status, history of conception and maternal and fetal outcomes were recorded from hospital records and telephonic interview. Results are in median and IQR.

Results: 64 women with TA of age 29 (24-38) years and disease duration 5 (4-10) years were interviewed. 74 pregnancies had occurred before disease onset in 29 women, while 20 had total 38 pregnancies after onset of disease. In eight, diagnosis was made during pregnancy. Age at disease onset was 22 (18-30) years. Type 5 disease was the most common (n=32, 59.3%), and equal number of patients had Ishikawa's class I and II disease (n=26, 40.6%). Median ITAS (n=44) was 13(7-16), DeiTak 12.5 (9-16.75) and TADS 8 (6.5-10). 16 were unmarried of whom 6 did not marry due to disease. 25 patients wanted to get pregnant, of which 8 (30%) did not do so because of her disease. 3 had infertility of which one was primary, one possibly due to disease and third related to spouse. Of the 32 who could discuss contraceptive issues, 3 had not received advice, and 7 were not using despite being advised. Obstetric outcomes were poorer in pregnancies that occurred after the onset of disease as compared with those prior to it (RR=1.5, p=0.01). Pregnancies after onset of TA carried very high risk of maternal (RR3.9, P<0.001) as well as fetal complications (RR= 2.0, p=0.001). Hypertension was the most common maternal complication [Table 1], and it occurred most often in the last trimester. The baby weight at birth was lower in births after disease (2.3 vs. 3.0, p=0.01). Wong's score ≥4 predicted lower birth weight (p=0.04). ITAS scores exhibited moderate correlation with DeiTak (r=0.78) and TADS (r=0.58). ITAS, ITAS-A, DeiTak and TADS could not predict obstetric outcomes.



Conclusion: Women with TA suffer from poor maternal as well as fetal outcomes.


  PC158 Vasculitis Top


Should LIA replace conventional testing in associated vasculitis? A rewarding experience from a tertiary care center

Dhaval Tanna, Gayatri Ekbote, Natasha Negalur, Dhiren Rawal, Naval Mendiratta, Shruti Bajad, Vinay Singal, Rajiva Gupta; Medanta the Medicity, Gurgaon, Haryana, India

Introduction: International Consensus Statement on Testing and Reporting of Antineutrophil Cytoplasmic Antibodies (ANCA) in patients suspected of ANCA-associated vasculitis (AAV) dictate that screening for ANCA to be done by indirect immunofluorescence (IIF) and positive results are to be confirmed by ELISA. Both IIF and ELISA require multiple substrates and conjugates, include various steps and generally multiple samples are tasted at a time in commercial laboratories. AAV may present with life threatening conditions and impending organ damage. In such circumstances when a single patient's sample needs to be tested for presence of ANCA, IIF and conventional ELISA may have limited utility and could be time consuming. Nitrocellulose strip based rapid diagnostic test like LIA can be used to support AAV in such scenarios when there is high pre-test probability.

Methods: All consecutive patients of proven AAV attending the Rheumatology and Clinical Immunology division of Medanta- the Medicity, Gurgaon from August 2015 to December 2016 were included. Their demographic and clinical details were analysed. ANCA COMBI test which includes antibody detection by both LIA and IIF was used. LIA was done by DTek kit while IIF by Immunoconcept kit. The study was approved by IRB.

Results: [Table 1] Forty-nine patients were included with median age of 53 years and median follow up of 21 months. 77.5% were PR3 + and 22.5% were MPO+, 89.47% of c-ANCA were PR3 + while 100% of p-ANCA had MPO+. Four patients had discordance in IIF and LIA result. Three were IIF negative and one had atypical ANCA by IIF. But all these four patients were PR3+ by LIA. All four were started on treatment with immunosupressants based on LIA result. Tissue biopsy was consistent with AAV in all four patients.



Conclusion: Rapid newer ANCA tests might be helpful as an adjunct to urgent therapeutic decisions and can replace conventional tests, but cannot supplant the critical appraisal of other clinical and laboratory findings. Histological confirmation of vasculitis is still the gold-standard and should be sought whenever possible.


  PC159 Vasculitis Top


Outcome of EUVAS protocol in ANCA associated vasculitis from a tertiary care hospital from North India

S Kansurkar1, A Zanwar2, R Gupta3, D P Misra2, A Lawrence2, V Agarwal2, A Aggarwal2, R Misra; Department of Clinical Immunology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 3All India Institute of Medical Sciences, New Delhi, India

Introduction: In ANCA associated vasculitis (AAV), cyclophosphamide administered in form of EUVAS protocol is mainstay of treatment. Present study evaluates outcome of this regimen in a group of 20 patients with AAV.

Methods: 20 patients of AAV presented between 2002 to 2016 who have received EUVAS protocol with follow up of at least 3 months were considered for retrospective analysis. BVAS scores at baseline and at every 3 months of follow up were recorded from hospital records. Maintenance therapy and any changes in therapy and reasons thereof were noted.

Results: Out of 20, 7 were male and 13 were females. 11 were PR3 AAV, 7 were MPO AAV and 2 were Eosinophilic granulomatosis with polyangiitis (EGPA). Median follow up was 45 months (range 3 to 184 months). There was involvement of different organ systems (n) like Skin (6), Eye(8), ENT (11), Lung (13), Kidney (14), nervous system (6). Median BVAS was 17.5 (Range 9 to 34). On receiving 6 cycles of Cyclophosphamide as per EUVAS protocol, only one of the patient had new or worse features, and 7 had persistent disease. 10 cases with remission received Azathioprine and were followed up for median 54 months. 6 of them had long term remission. Two cases had relapse after 20 and 60 months each. Patients with persistent disease activity (7 cases) received additional 3 cycles of Cyclophosphamide. 6 of them achieved remission. Time to relapse while on maintenance is shown in [Table 1]. Those on methotrexate developed early relapse (Mean 9 months) than those on Azathioprine (Mean 39 months).



Conclusion: This study underlines efficacy of EUVAS protocol in inducing and maintaining remission. Even after 6 cycles of a third of patients do not achieve remission and eventually require additional 3 cycles. The results are comparable with published reports.


  PC160 Vasculitis Top


Long term outcome of ANCA associated vasculitis using cyclophosphamide as induction therapy: A single centre study

Gayatri Ekbote, Natasha Negalur, Muzaffar Bindroo, Dhiren Raval, Dhaval Tanna, Wasim Kazi, Shruti Bajad, Naval Mendiratta, Vinay Singal, Rajiva Gupta; Medanta the Medicity, Gurgaon, Haryana, India

Background: ANCA associated Vasculitis (AAV) is associated with a high mortality rate despite standard immunosuppressive therapy and has been shown to be 2.7 times that of the normal population1. Cyclophosphamide (CYC), along with steroids, has been the cornerstone of therapy for AAV. Here, we aim to study the long-term outcome of AAV with cyclophosphamide therapy as an induction agent.

Methods: All consecutive patients of proven AAV attending the Outpatient and Inpatient clinic of Rheumatology and Clinical Immunology division of Medanta- the Medicity, Gurgaon from a period of August 2015 to December 2016 were included. Their clinical, demographic, laboratory and treatment details were noted and analysed. The study was approved by IRB.

Results: Forty-nine patients were included with a median age of 53 years and median follow up of 21 months. Forty-two patients received CYC as induction therapy out of which 20 patients achieved remission. Nineteen had persistent disease or relapsed after induction, more commonly in PR3 and GPA groups, with 6-month mortality being more in the MPO and MPA groups. The following table [Table 1] shows the outcome in AAV in the cohort.



Conclusions: There still remains a high relapse rate in AAV with CYC and a possible role of Rituximab as a primary induction agent needs further study.

References

  1. Tan JA, Dehghan N, Chen W, Xie H, Esdaile JM, Avina-Zubieta JA, et al. Mortality in ANCA-associated vasculitis: Ameta-analysis of observational studies. Ann Rheum Dis 2017;76:1566-74.



  PC161 Vasculitis Top


Type of Takayasu arteritis based on gender

Natasha Negalur, Gayatri Ekbote, Dhiren Raval, Muzaffar Bindroo, Naval Mendiratta, Shruti Bajad, Rajiva Gupta, Dhaval Tanna, Wasim Kazi; Medanta the Medicity, Gurgaon, Haryana, India

Background: Primarily known to affect women, Takayasu arteritis (TA) is seen in a number of males as well. Studies have shown that there are differences in clinical and radiological features of TA in males and females1,2,3. These, however, are not consistent worldwide. Here, we have tried to classify patients of TA according to gender and correlate with their angiographic findings.

Methods: All consecutive patients of TA attending the Rheumatology Outpatient as well as Inpatient department of Medanta- the Medicity hospital were included. Their demographic details, clinical, radiological and laboratory parameters were assessed and classified according to gender. The study was approved by the IRB.

Results: Out of a total of 46 patients in the cohort, there were 11 males and 35 females. The median ages were 33 and 35 years for males and females respectively. Females had more vascular (72% M vs 88% F), cardiac (18% M vs 40 % F) and ophthalmic manifestations (0 M vs 14% F) and the difference was statistically significant. Hypertension was also more commonly seen in females (27% M vs 54% F, p: 0.008). The following table [Table 1] describes the angiographic characteristics of the cohort.



Conclusion: There is a significant difference in the clinical features and radiological involvement in males and females with TA.

Refrences

  1. Lim AY, Lee GY, Jang SY, Gwag HB, Choi SH, Jeon ES, et al. Gender differences in clinical and angiographic findings of patients with Takayasu arteritis. Clin Exp Rheumatol 2015;33:S-132-7.
  2. de Saboia Mont'Alverne AR, de Paula LE, Shinjo SK. Features of the onset of Takayasu's arteritis according to gender. Arq Bras Cardiol 2013;101:359-65.
  3. Wan J, Wang T. Comparison of clinical characteristics of patients with Takayasu's arteritis by age and gender. Ann Rheum Dis 2016;75:570-1.



  PC162 Vasculitis Top


IgG4 aortitis does not masquerade as Type IV Takayasu arteritis

Harshini Shivakumar, Ruchika Goel, K Jayakanthan, Debashish Danda; Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Takayasu arteritis (TA) is a granulomatous transmural inflammation of aorta and its direct branches. Type-IV subset of TA involves involvement of infra-diaphragmatic branches and may at times be difficult to differentiate from aortitis variant of IgG4 disease. 2.

Aims and Objectives: This study aimed to assess the IgG4 levels in Indian patients with TA. Objectives were to compare serum IgG4 levels among type-I and type-IV TA as well as healthy controls; to correlate the IgG4 levels with clinical phenotype. 3.

Methods: Serum of TA patients and age and sex matched healthy controls satisfying 1990 ACR or Ishikawa criteria for TA were subjected to IgG4 estimation by nephelometry using MININEPH human IgG4 kit. Clinical and angiographic details of patients were recorded. Median IgG4 levels were compared between various groups by Mann-Whitney test using SPSS vs 18. 4.

Results: Twenty eight patients of Takayasu arteritis with a median age of 30 +/- 10.3years, disease duration of 14months (IQR 6-30), ITAS of 5 (0-11.5), DEITAK of 9 (5-11) were studied. Of them thirteen had type-IV and fifteen others had type-I or type-II TA. 15 healthy controls were also recruited. Median IgG4 levels in patients were similar to that of controls [292 (165-725) v/s 832 (194-1055), p=0.16]. IgG4 levels of >500 mg/L were observed in 42.6% of patients and 60% of controls. Of them, 15.4% of the patients and 20% of the controls had IgG4 levels of >1350 mg/L. IgG4 levels in type IV TA were similar to that in type-I/II [574 (74-946) mg/L v/s 263 (170-626) mg/L, p=0.48]. No association was observed between IgG4 levels and disease activity (p=0.29) as well as other clinical parameters. IgG4 levels were significantly higher in all 5 patients who presented with major complications as compared to the rest [1088 (393-1385) mg/L] v/s [237(74-521) mg/L, p= 0.002] 5.

Conclusion: No difference in IgG4 levels was observed between type-I/II and type IV Takayasu arteritis. Presence of complications was associated with higher IgG4 levels.


  PC163 Vasculitis Top


Clinical profile of medim and small vessel vasculitis from a tertiary care centre in South India

Sreedevi Suresh, Thiruchengode Natesan Tamilselvam, Selvakumar Balameena, Euphrasia Latha, Myilsamy Saravanan, C Saranya, Mythili V Seetharaman; Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Vasculitides comprise a heterogeneous group of inflammatory disorders that result in protean manifestations clinical presentation of medium and small vessel vasculitis pose a major dilemma for detection, therapy and follow up.

Objective: To study the varied clinical presentations of small and medium vessel vasculitis in a tertiary care rheumatic centre in South India.

Methods: A cross sectional study was conducted on 60 patients who fulfilled the chapel hill consensus criteria- 2012 for small and medium vessel vasculitis, between 2014-17.Detailed history, clinical examination, appropriate immunological tests, imaging studies and biopsies were done.

Results: Of the 60 patients, common forms of vasculitis observed in decreasing order of frequency include HSP (henoch schonlein purpura 26%), Unclassified vasculitis(20%), PAN (polyarteritis nodosa 20%), AAV (anca associated vasculitis16%), Behcet (8.3%), cryoglobulinemic vasculitis (5%), Kawasaki disease (1%). Of the patients with PAN 80% had cutaneous forms with tender nodules and gangrene of fingers, classic (20%) presented with hypertension and mononeuritis multiplex. 68% of the HSP had complicated course (proteinuria and abdominal pain) with male preponderance. Most of the unclassified vasculitis patients were females who presented with refractory skin ulcers. Commonest clinical manifestations of GPA (granulomatosis polyangiitis n=4) include otorhinosinusitis (4), multiple cranial nerve palsies (2), features of MPA (microscopic polyangitis n=4) were glomerulonephritis (4), features of EGPA (eosinophilic granulamatosis with polyangitis n=2) were mononeuritis multiplex (2). fleeting pulmonary infiltrates seen in EGPA (2) and MPA(1). Arthralgia and palpable purpuric rash were the common presentation of cryoglobulinemic vasculitis. Of the 4 patients with behcet disease (90%) presented with recurrent oral and genital aphthae. Panuveitis was seen only in one paediatric female patient with Behcet.

Conclusion: Of the small vessel vasculitis, HSP remains most common and males had complicated course with renal involvement. Cutaneous PAN is commoner than classic PAN. Unclassified vasculitis constitute 3rd common form of vasculitis. Lone patient with kawasaki had incomplete form. Cryoglobulinemic vasculitis patients had active hepatitis infections.


  PC164 Vasculitis Top


Granulomatosis with polyangiitis: Clinical profile of 17 patients

Vivek Vasdev, Ramakant Singh, Arun Hegde, Kunal Kishore, Arhun MN; Army Hospital R and R, New Delhi, India

Objective: To report the varied presentation, treatment outcomes and complications in a cohort of patients with Granulomatosis with polyangiitis (GPA).

Methods: The details of demography, clinical presentation, investigations and treatment outcomes werecollected from the medical records of all GPA cases treated at a tertiary care centre in New Delhi, between May 2016 to Jun 2017 and all were analysed.

Results: A total of seventeen patients (10 males and 07 females) were included in the study. The mean age at presentation was 40.7± 16.29 years while the mean duration at presentation was 2.7 months. Out of the total cases studied, 03 were limited GPA. The mean duration of follow up was 9.18 ± 3.89 months. The induction regimen in generalised GPA cases was as per NIH protocol, whereas, the limited forms were treated with oral prednisolone and methotrexate. The remission maintenance was achieved using different regimen as per the condition of the patients which included Azathioprine (n=06), Methotrexateand Prednisolone (n=03), prednisolone alone (n=03), rituximab (n=03) and 02 were lost to follow up. Relapse occurred in 06 patients with mean time for relapse being 7.43 ± 2.3 months. There were 07 patients who developed complications of treatment out of which 01 succumbed to it. Rest of the relapses and complications were treated as per established guidelines with good response and recovery.

Conclusion: The clinical presentation of GPA is varied ranging from limited less aggressive forms to severe life threatening illness. The treatment is prolonged requiring close follow up and monitoring to detect and treat relapses and complications.


  PC165 Vasculitis Top


Granulomatosis with polyangiitis and mimickers presenting with midline destruction

Jha Saket1, Sharma Aman1, Acharya Nupoor1, Ranjan Rajiv Kumar1, Dhooria Aadhar1, Nada Ritambara2, Das Ashim2, Jain Sanjay1; Departments of Medicine and 2Histopathology, PGIMER, Chandigarh, India

Background: Granulomatosis with polyangiits (GPA) is a systemic granulomatous necrotizing vasculitis. Sinonasal involvement is the most common manifestation of GPA. The cases presenting only with sinonasal involvement can mimic lymphoma, sarcoidosis, drugs and infection which may result in diagnostic dilemma.

Aim: The purpose of this study was to analyze the cases presenting as midline destructive lesion.

Methods: This was a retrospective study of patients presenting with midline destructive lesions. Their demographic, clinical profile, laboratory and imaging findings along with treatment outcome were noted.

Results: Six cases [Four with GPA and two with natural killer (NK)/T cell lymphoma)] were included. Mean age for GPA and NK/T cell lymphoma was 40.3 and 23 years respectively. Among GPA cases, three had limited and one had systemic disease. All cases had sinusitis, while two had lateral wall and palatal destruction. C ANCA positivity was seen in two cases while PR3 was positive in one case. Two cases which were ANCA negative, was diagnosed on repeated biopsy. NK/T cell lymphoma patients had sinus involvement and palatal perforation in both the cases. One case had left ocular proptosis with cavernous sinus thrombosis and involvement of III, IV and IV cranial nerves. Diagnosis of NK/T cell lymphoma was made on the basis of CSF flow cytometry in one case and after repeated biopsies in the other. Cyclophosphamide and rituximab were remission induction agents in 3 and 1 GPA patients respectively. Three GPA patients had favorable outcome while one died. Both patients with NK/T cell lymphoma died.

Conclusion: Midline destructive lesion can be mutilating and life threatening. Establishing the correct diagnosis can be difficult and might requiring multiple biopsies and CSF examination.


  PC166 Scleroderma, Myositis and Overlap Top


Maintenance therapy reduces mortality in scleroderma interstitial lung disease: Long term follow up of an observational study

Padmanabha Shenoy, Bazil Alias, Able Lawrence1, Kaveri Nalianda, Sreelakshmi Sreenath; Centre for Arthritis and Rheumatism Excellence, Kochi, Kerala, 1Department of Clinical Immunology, SGPGI, Lucknow, Uttar Pradesh, Indai

Background: Recently published SLS2 study could not establish the superiority of two year treatment of MMF over one year treatment with oral CYC in scleroderma interstitial lung disease (SSc-ILD). Lack of studies addressing the efficacy of maintenance therapy in SSc ILD, together with SLS2 data prompted the need to revisit the practise of long term immunosuppression. We previously reported the equivalence of IV CYC and MMF for induction in a retrospective data review. 1 We now report the effect of long term maintenance on mortality in this cohort.

Methods: SSc-ILD patients who received protocol based induction therapy with IV CYC or MMF for 6 months were followed up with 6 monthly spirometry were initiated on maintenance therapy with AZA, MMF or Methotrexate. The choice of maintenance agent was chosen after discussion between patient and rheumatologist. Data of those on follow up was obtained from medical records. Patients who defaulted were contacted telephonically or by house visits and the current status was recorded. In case of death the cause of death was ascertained by interviewing relatives and verifying medical records.

Results: Of the 57 patients who were given induction therapy 11 patients defaulted. 46 patients received maintenance therapy, with a median follow up of 62 months (range 17 to 81). Of the 11 patients who defaulted, 9 could be contacted. Of these nine none were on maintenance therapy and four (44.44 %) died all due to end stage lung disease. There were only 5 deaths (10.87%) among those who received maintenance (HR 0.246, P=0.037, [Figure 1],). Cause of death was end stage lung disease in four and infection in the other. Both the groups were comparable at baseline and after induction. In patients who were on maintenance FVC increased by 5.7 ± 14.8% from 55.1 ± 13.1 % at the end of induction to 60.8 ± 18.2 % at last follow up.



Conclusion: After induction with CYC or MMF, maintenance therapy significantly reduces mortality in SSc-ILD.

References

  1. Shenoy PD, Bavaliya M, Sashidharan S, Nalianda K, Sreenath S. Cyclophosphamide versus mycophenolate mofetil in scleroderma interstitial lung disease (SSc-ILD) as induction therapy: A single-centre, retrospective analysis. Arthritis Res Ther 2016;18:123.



  PC167 Scleroderma, Myositis and Overlap Top


Nailfold capillary changes and clinical profile in eight patients with positive Scleroderma serology and specific visceral involvement without any skin thickening

Isha Sood, Nikunjkumar V Dadhaniya, Sundeep Upadhyaya, Sirinder J Gupta, Rohini Handa; Department of Rheumatology, Indraprastha Apollo Hospitals, Delhi, India

Objective: Systemic sclerosis sine scleroderma is a variant of systemic sclerosis (SSc) where patients do not have skin thickening but have some form of SSc specific visceral involvement and serology. Here we present the clinical profile and Nailfold capillaroscopy findings of 8 patients without skin changes of scleroderma, who nevertheless had a positive SSc serology plus visceral disease compatible with scleroderma.

Methods: Patients who were ANA positive with either Anti-Scl70 or Anti centromere antibodies without any skin thickening as assessed by two observers, and with a scleroderma specific organ involvement, were included in the study. Specific organ involvement included SSc specific lung, heart, gastrointestinal involvement or digital pits and ischemia. Disease duration was from the time of first non-Raynaud symptoms. Chest radiographs, echocardiography, complete blood count and routine liver and kidney function tests were obtained in all patients. A nail fold capillaroscopy (NFC) was done in all the patients. The middle 3mm area of 8 fingers were studied (excluding thumbs). Other clinical and investigative profiles of all patients was also evaluated.

Results: A total of eight patients fulfilled the above criteria, four with interstitial lung disease (NSIP), one with PAH, one each with gastric antral vascular ectasia (GAVE) [Figure 1], digital ischemia and digital pits. The mean disease duration of the patients was 28± 18 months (range 8 mnths-3 yrs) with mean age 47±17 (age range 28-72 yrs). All ILD patients were Anti-Scl -70 positive. Patients with digital ischemia and digital pits were also positive for Anti-Scl-70. Two patients, one with Gastric antral vascular ectasia (GAVE) and one with PAH (PASP=76 on Echo) were anti-centromere positive. Raynaud's phenomenon was present in 7 patients excluding one patient with GAVE. NFC was abnormal in 7 patients. One patient with digital ischemia had normal NFC, but positive serology. All patients with abnormal NFC had active scleroderma pattern (dilated capillaries, capillary loss plus haemorrhages).



Conclusion: The nailfold capillaroscopy complements serology in distinguishing Systemic Sclerosis Sine Scleroderma organ involvement from idiopathic causes of organ involvement. Raynaud's phenomenon is the most common symptom seen in such patients


  PC168 Cleroderma, Myositis and Overlap Top


Obstetric outcomes in 81 women with inflammatory myositis

Latika Gupta, Sakir Ahmed, Amita Aggarwal; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Objectives: Idiopathic Inflammatory myositis (IIM) is a heterogenous group of diseases, which commonly affects women in the childbearing age group. We studied obstetric outcomes in myositis patients before and after disease onset.

Methods: Women aged ≥18 years with IIM (Bohan and Peter's criteria) were included. Apart from demographic data, menstrual status, history of conception and maternal and fetal outcomes were recorded. All results are expressed in median and IQR.

Results: 81 women with myositis of age 40 (32-49) years and disease duration 4 (2-9) years were interviewed. Median age at disease onset was 32 (26-43) years. Thirty-five were postmenopausal women, of whom 5 had an early menopause (14.3%). 45 patients had Dermatomyositis (DM), 20 each had Polymyositis and 16-overlap myositis. Fifteen patients wanted to get pregnant, of which 3 did not do so due to disease. Twenty-four pregnancies resulting in six women over 77.5 patient years of follow up culminated in 5 live births, 16 abortions, and 2 MTPs. Of the live births, 1 had cleft palate, 1 was LBW and 1 preterm. There were no maternal complications. All successful pregnancies were seen in those who had had previously borne children. None were positive for antiphospholipid antibodies. 63 patients conceived before disease onset, resulting in 205 pregnancies and 155 live births over 315.2 patient years of follow-up. Thirty-one had complications (17 maternal and 14 fetal). In 7 women the disease started after pregnancy after median time 1 (0.75-1.0) year. Obstetric complications were more common in pregnancies after the onset of myositis as compared with those prior to the disease (RR=7.6, p<0.0001). Pregnancies after myositis onset carried higher risk of fetal complications (RR= 2.7, p=0.002) although there was no difference in maternal complications. Conception after the onset of myositis had higher risk of resulting in abortion as compared with successful live birth (RR 3.8, p<0.0001).

Conclusion: Women with IIM suffer from poor fetal outcomes as well as high spontaneous abortion rates in the absence of clinical or serologic APS.


  PC169 Scleroderma, Myositis and Overlap Top


Serum interleukin-6, interleukin-17 and transforming growth factor beta levels in systemic sclerosis and correlation with clinical Manifestations

Anupam Wakhlu, Rasmi Ranjan Sahoo, Mohit K Rai1, Durga P Misra1, Vikas Agarwal1; Department of Rheumatology, King George's Medical University, 1Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Dysregulation in cytokines like interleukin-6 (IL-6), interleukin-17 (IL-17) and transforming growth factor-beta (TGF Î2) has been pathogenically implicated in systemic sclerosis (SSc).

Objectives: The present study aimed to assess serum IL-6, IL-17 and TGF Î2 levels in patients with SSc and correlate with clinical manifestations.

Methods: This cross-sectional, observational study included 93 patients fulfilling the 2013 revised ACR/EULAR SSc classification criteria and 33 age-and sex-matched healthy controls. Antinuclear antibody (ANA), extractable nuclear antigen (ENA) profile, chest radiograph, pulmonary function tests and electrocardiography were done. HRCT of thorax and echocardiography were done wherever indicated. Modified Rodnan skin score (MRSS) was calculated. Serum IL-6, IL-17 and TGF Î2 levels were assayed using ELISA kit [Human IL-6 and TGF-beta kit: BD Bioscience, USA; IL-17 kit: R&D, USA] and compared among disease subtypes and clinical parameters. Spearman coefficient was used to test correlation between continuous variables. P value of <0.05 was considered significant.

Results: The mean age of patients was 37.8 + 10.3 years (Female:Male: 30:1) with median duration of disease of 2.5 years. Serum IL-6, IL-17 and TGF Î2 levels were significantly higher in patients as compared to controls (IL-6: 19 vs 7 pg/ml (p<0.0001); IL-17: 39 vs 16 pg/ml (p<0.0001); TGF-Î2: 862 vs 377 pg/ml (p<0.0001). Higher levels of these cytokines were also observed in patients of diffuse cutaneous SSc, those with lung fibrosis and anti-topoisomerase positivity.

Conclusion: Serum IL-6, IL-17 and TGF Î2 levels were significantly higher in SSc patients and correlated positively with ILD and skin fibrosis.


  PC170 Scleroderma, Myositis and Overlap Top


Long term outcomes of mycophenolate in interstitial lung disease associated with scleroderma

S Padiyar, R Janardana, P Chebbi, D Danda; Christian Medical College, Vellore, Tami Nadu, India

Introduction: Interstitial lung disease [ILD] is a leading cause of mortality in scleroderma (1). Scleroderma lung study-II clearly illustrates the equivalent efficacy and a better side-effect profile of 2 years of mycophenolate mofetil [MMF] in scleroderma as compared to 1 year of oral daily cyclophosphamide over a 2 year period. We conducted this retrospective study to evaluate the long-term outcome of using MMF in Scleroderma related ILD in a real world setting. Earlier studies had smaller sample over a shorter follow up.

Aim: To study the long-term outcomes of lung functions mycophenolate mofetil in scleroderma related ILD in terms of change in FVC.

Methods: Data of all patients of scleroderma from 2013 till date who had a baseline FVC, follow up at 1, 2 and 3 year were taken for analysis. The mean change in the FVC value was noted.

Results: 101 patients had a baseline and a follow up spirometry data. The mean duration of the disease was 46 month (±36.2). 60 patients had a 1 year follow up. 38 patients had a follow up till 2 years and 22 patients had a follow up till 3 years. The mean increase in the FVC value were 4.4.1.72, 3.13 at the end of 1, 2, 3 years. At the end of 1 year, 87 percent had a stable disease, 7 % of individuals had an improvement from baseline, 6 percent had worsening. Similarly at the end of 2 years 69.7 % had a stable disease, 16 percent had improvement, and 13.3 percent had worsening. At the end of 3 years, 63 % had a stable disease, 27 percent had improvement and 10 percent had worsening.

Conclusion: Majority of individuals of scleroderma ILD patients on MMF had a stable disease or improvement during short and long term follow up.

Reference

  1. Taylor JG, Bolster MB. Bronchiolitis obliterans with organizing pneumonia associated with scleroderma and scleroderma spectrum diseases. J Clin Rheumatol 2003;9:239-45.



  PC171 Scleroderma, Myositis and Overlap Top


Prevalence and predictors of psychiatric disorders in systemic sclerosis

Rachana Sheno, Ajit K Surin2, Ramya Janardana3, Sangeetha Priya3, Rajesh G4', Ambily Anoop5, L Jeyaseelan5', Anju Kuruvilla5', Ruchika Goelì½, Debashis Danda; Departments of 1Internal Medicine, Clinical Immunology and 3Rheumatology, 4Psychiatry and 5'Biostatistics, Christian Medical College, Vellore, Tamil Nadu, 2Department of Rheumatology, Apollo Hospital, Bhuvaneshwar, Odisha, India

Background: Systemic sclerosis [SS] is a chronic inflammatory disease characterized by progressive fibrosis and vasculopathy involving skin and internal organs. Although SS is not known to affect the central nervous system directly, the prevalence of depression in these patients has been shown to be as high as 40-50%, which correlates to the degree of functional limitation, pain and social support.

Objectives: To assess the prevalence and predictors of common mental disorders in SS.

Methods: Patients were prospectively interviewed using the Revised Clinical Interview Schedule [CISR] questionnaire. An algorithm based on ICD-10 criteria was used to diagnose the following psychiatric disorders- Generalised Anxiety Disorder [GAD], Mixed Anxiety Depressive Disorder [MADD], Depression, Phobia, Panic disorder and Obsessive Compulsive Disorder [OCD] for those with CISR score ≥12. Clinical variables such as baseline Modified Rodnan skin score, duration of illness, sclerodactyly, digital ulcers, Raynaud’s phenomenon, interstitial lung disease [ILD] and gastrointestinal [GI] dysfunction were correlated with individual psychiatric symptoms as well as psychiatric diagnosis.

Results: Out of 81 patients interviewed, 58 [71.6%] had CISR score of ≥12 [50 female, 8 male]. Prevalence of MADD, Depression, OCD, GAD and Phobia were 51.7% [n=30], 22.4% [n=13], 15.5% [n=9], 6.8% [n=4] and 3.4% [n=2]. Significant positive association was found between the occurrence of obsessions and duration of illness [n=11, p=0.001]. On stratifying the disease duration as ≤ 5 years and >5 years, we observed higher occurrence of obsessions in those patients with a longer duration of illness [n=1, 2.7% vs n=10, 22.7%, p=0.010]. Regression analysis showed association of disease duration with the occurrence of obsessions, independent of GI dysfunction, digital ulcers and sclerodactyly [p=0.014, OR-27.7, 95% CI (1.9-250)]. Significant positive association was found between the presence of sclerodactyly, GI dysfunction and digital ulcers, and the diagnosis of OCD [p=0.059, p=0.037, p=0.017 respectively].

Conclusions: 71.6% of patients with SS had a common mental disorder. Longer duration of illness was significantly associated with the occurrence of obsessions. Sclerodactyly, GI dysfunction and digital ulcers correlated significantly with the diagnosis of OCD.


  PC172 Scleroderma, Myositis and Overlap Top


Correlation between myositis related autoantibody profile and nail fold videocapillaroscopic pattern in idiopathic inflammatory myositis

Ramya Janardana, Ruchika Goel, Pramod Prahlad Chebbi, Debashish Danda; Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Nailfold videocapillaroscopy [NFVC] abnormalities are a tool to assess microvascular changes in inflammatory myositis (IIM).

Aims and Objectives: To study the NFVC pattern in consecutive IIM patients and to explore any association with clinical and autoantibody profile.

Methods: Consecutive IIM patients diagnosed between December 2016 and July 2017 underwent NFVC examination using nailfold video capillaroscope Optilia with 200x magnification. NFVC parameters were recorded according to criteria reported in literature. Euroimmun line immunoassay was used to detect myositis related antibodies [MA].

Results: Twenty patients including 13 with dermatomyositis (including 3 clinically amyopathic dermatomyositis), 3 with polymyositis and 4 with overlap myositis were studied. Antibody profile was as follows: ANA in 6; Mi-2 in 4, Jo-1 in 1, PL-7 in 2 and PL-12 in 1, PM-Scl in 4 and Ro-52 positivity in 4 patients. Capillary density was reduced in all patients with a median density of 4.9 (IQR 4.0-6.8). Ramified capillaries were seen in 19 [frequency=8 (1-16)], giant capillaries in 4 and bushy pattern in 8 patients. Large confluent avascular areas were a finding in 3 individuals and 4 individuals had mild avascularity. There was no significant difference in any of the parameters in relation to the various autoantibodies tested. However, giant capillaries were present in 3/6 patients without any MA, as compared to 1/14 patients with MA (p=0.028). Numerically higher autoantibody titres were noted in those with higher frequency of bushy capillaries [6.6 (high titre MA) vs 1.6(low titre MA) vs 0.75(MA negative)]. Avascular areas were observed in 5/7 patients with ILD as compared to 2/13 patients without same (p= 0.022). Capillary density was reduced [4.6 (3.7-6.5)] vs [7 (6-8.2)], (p=0.029)] and the number of ramified capillaries was higher [12.5 (4.3-32) vs [0.5 (0-1.75)], (p=0.005] in patients with cutaneous involvement as compared to those without.

Conclusion: In our cohort of inflammatory myositis reduced capillary density and presence of ramified loops were a consistent finding. Presence of interstitial lung disease was significantly associated with avascular areas. Cutaneous involvement was significantly associated with reduced capillary density and ramified loops.


  PC173 Scleroderma, Myositis and Overlap Top


Immune mediated inflammatory myositis: A single centre ambidirectional cohort study

Vineeta Shobha, K N Sangeetha, Raghunandan Nadig1, Anita Mahadevan2; Departments of Clinical Immunology and Rheumatology and 1Neurology, St. John's Medical College and Hospital, 2Department of Neuropathology, National Institute of Mental Health and NeuroSciences, Bengaluru, Karnataka, India

Background: Myositis is an uncommon disease, characterized by inflammation of muscles. It affects all age groups and its treatment traditionally consists of long term steroids and immunosuppressants.

Aim: To review the clinical profile of patients with myositis and assess their long term outcome.

Methods: Patients diagnosed to have myositis from January 2006 to July 2017 were included in the study. Their clinical features, laboratory investigation, electromyography, autoantibody profile and histopathology reports were collected from OP and IP records. Muscle biopsy and muscle specific autoantibodies were tested at NIMHANS, while the other tests were done at our centre. Data was collected using a pre structured proforma and analysed.

Results: One hundred and fifteen patients diagnosed to have Immune Mediated Myositis (IIM) from Jan 2006 to July 2017 were enrolled, of which 76.5 % were females. Mean age at diagnosis was 37.3+/-7.6 years (95% CI). Of 115 patients, 9.5% (N=115) were children. Eleven (9.56 %) patients presented early (<2 weeks), while remaining presented after several weeks of onset of symptoms. Heliotrope rash was seen in13 patients, while 11 patients had diffuse maculopapular rash and 8 patients had gottron’s papules. Calcinosis was noted in 3 patients. Neck muscle weakness was noted at presentation in 15.6% and respiratory distress in 13.9%. Dysphagia and dysphonia was present in 4.3% each. Using Bohan and Peter’s criteria, dermatomyositis (DM) was diagnosed in 51.7%, polymyositis (PM) in 28.5%, myositis associated with connective tissue diseases in 14.3%, juvenile dermatomyositis in 3.6% and malignancy in 1.8%. One child had amyopathic dermatomyositis. CPK was normal in 25.6%, 250-5000U/L in 48.7%, >5000 in 24.35% and as high as 16,000 in 1.3%.One patient had rhabdomyolysis. ANA was negative in 32 (27.8%) patients. Among ANA positive, Ro-52 was more frequently noted (n=23). Muscle biopsy was performed in 97 patients. Of which, 63.6% biopsy confirmed DM, 12.7% had PM, 23.6 % had PM with vasculitis. MRI was performed in 10 patients, of which 90.4% confirmed inflammatory myositis. All patients received steroids (IV pulse or oral) and patients received additional immunosuppression (azathioprine -24.3%, methotrexate- 23.4%, MMF- 9.56%, cyclophosphamide-2.6%, IVIg, plasmapheresis-0.8%). Most of the patients required prolonged immunosuppression. Median duration of follow up was 5 years. Relapse was noted in less than 10 patients, due to non-compliance or on withdrawal or minimizing treatment. Very few patients were successfully taken off medications without relapse and most of them are currently on minimal dose of steroids and immunosuppressants. Major infections were noted in 7 patients. Four patients succumbed, of whom 1 patient had respiratory muscle involvement and died due to aspiration pneumonia and refractory septic shock.

Conclusions: This is the largest study in South India on clinical profile and outcome for IIMs. However as it is an ambidirectional study, further follow up of the patients under study is necessary. Abbreviations: DM-dermatomyositis, PM-polymyositis, ANA-antinuclear antibodies, CPK-creatinine phosphokinase, JDM- juvenile dermatomyositis, CTD-connective tissue disease.


  PC174 Scleroderma, Myositis and Overlap Top


Connective tissue diseases in pulmonary hypertension: A short study

Raut Amol, Samant Rohini, Shah Romi; P.D. Hinduja National Hospital, Mumbai, Maharashtra, India

Background: CTDs are diificult to treat and those complicated by pulmonary arterial hypertension (PH), which is associated with significant morbidity and mortality, becomes challenging to treat.

Objectives: To study PAH in CTDs not associated with interstitial lung disease and effect of immunosuppressive treatment on it.

Methods: Retrospectively studied patients of CTD associated PH not having ILD excluded by Chest CT in outpatient dept. for 2 yrs.

Results: Out of 16 patients studied 10 were MCTD (62.5%), SLE 4 (25 %), 1 SSc and 1 LSSc. 15 females and one male patient with SLE. One SLE and SSc patient had associated APLA. Average baseline PH was 87 mmhg with maximum PH 126. All patients received PDE-4 inhibitors either sildenafil or Tadalafil, ET-1 antagonist by 7 patients, Corticosteroids tapered upto maximum 5 mg and immunosuppressive therapy either IV cyclophosphamide pulses every 15 days, mycofenolate monotherapy or following IV Cyclophosphamide. Two patients received azathioprine. Response to therapy (decrease in PH >20) was seen in 7 Out of 16 patients (43%) out of which 1 SLE and 1 LSSC were on azathioprine, rest of the patients received IV cyclophosphamide pulses with steroids and PDE 4 inhibitors. Maximum reduction in PH was 47 mmHg seen in one SLE. Two patients on mycofenolate given for other reason developed PH and eventually required cyclophosphamide. 11 of 16 (69%) patients had dilated RA/RV at baseline out of which 3 reverted back to normal cardiac size after treatment (2 MCTD and 1 SLE). NYHA functional class improved in 11 out of 16 patients (69%), worsened in 1 patient and didn't improve in 4 patients those who have had class III to IV at the baseline. Two patients died, 1 due to CVA and the other due to respiratory infection.

Conclusions: 1. Early detection and initiation of appropriate immunosuppressive therapy is essential to reduce morbidity and mortality. 2. Those who have severe PH and higher NYHA class at baseline are less likely to respond to therapy. 3. Inadequate response to treatment may be due to severity of underlying CTD and other unidentified factors.


  PC175 Scleroderma, Myositis and Overlap Top


The myositis autoantibody phenotypes in inflammatory myositis



Natasha Negalur, Gayatri Ekbote, Dhiren Raval, Dhaval Tanna, Naval Mendiratta, Vinay Singal, Rajiva Gupta; Medanta the Medicity, Gurgaon, Haryana, India

Background: Inflammatory mypoathies (IM) are often associated with autoantibodies, which can be Myositis specific or Myositis associated antibodies (MSA and MAA respectively). In India, 30- 35% patients of IM have MSA and MAA. Here, we have studied the autoantibody phenotypes in IM.

Methods: This study was conducted in the Department of Rheumatology at Medanta hospital, Gurgaon. All consecutive patients having IM were included and their clinical and laboratory data were collected. Patients were classified into groups into Dermatomyositis (DM), Polymyositis (PM), Inclusion Body myositis (IBM), Overlap myositis (OM), Cancer associated myositis (CAM), Necrotising autoimmune myositis (NAM) and Juvenile myositis (JM). MSA and MAA antibodies were analysed and correlated with clinical features. The study was approved by the IRB.

Results: Forty two patients were included in the study with 11 males (26.1%) and 31 females (73.8%) and median age of 42 years. The most common MAA were Jo-1 and Mi-2 [5 patients (11%)] followed by SRP (7%). Ro was the most common MAA with 11 patients having positive values (26%). Mi-2 was the most common MSA in DM (26%) whereas Jo-1 was highest in PM (25%). [Table 1] shows the distribution of antibodies and association with clinical features. Conclusion: Mi2 and Jo1 were the most common MSA in the cohort. However, a larger cohort is needed for further association study.




  PC176 Scleroderma, Myositis and Overlap Top


Esophageal manometry, esophagogastroduodenoscopy and duodenal mucosal histopathology in systemic sclerosis

M B Adarsh, Saroj K Sinha1, Shefali Khanna Sharma, Kaushal K Prasad1, Varun Dhir, Surjit Singh; Departments of Internal Medicine and 1Gastroenterology, PGIMER, Chandigarh, India

Introduction: Systemic sclerosis (SSc) is known to involve the gastrointestinal (GI) tract resulting in dysmotility, gastroesophageal reflux disease and mucosal changes causing significant morbidity.

Methods: It was a prospective, cross sectional, single centre study of 23 patients with SSc diagnosed on the basis of standard criteria. Clinical details including the gastrointestinal symptoms were recorded in proforma. All of them underwent esophagogastroduodenoscopy with duodenal biopsy and 21 underwent esophageal manometry.

Results: Regurgitation, heartburn and dysphagia were seen in 9 (82%), 16 (69%) and 10 (43%) patients respectively. On endoscopy, 19 patients (83%) showed changes of reflux esophagitis (4 had grade C or D esophagitis) and 3 had esophageal candidiasis. Among the 21 patients who underwent esophageal manometry, 13 (62%) had absent peristalsis, 6 (28%) had ineffective peristalsis (48%) and 10 had hypotensive lower esophageal sphincture (LES). Duodenal biopsy showed partial villous atrophy in 9 (39%) patients, increased intraepithelial lymphocytes in 18 (78%) and excess of mononuclear inflammatory cells in lamina propria in 21 (91%). Partial villous atrophy was seen more commonly in those with abnormal esophageal peristalsis and a hypotensive LES.

Conclusion: Most of the patients with SSc had esophageal dysmotility in the form of absent peristalsis, ineffective esophageal peristalsis and hypotensive LES. Histology of descending duodenum revealed partial villous atrophy and/or chronic inflammatory changes in most of the patients with SSc.


  PC177 Scleroderma, Myositis and Overlap Top


Spectrum of cutaneous manifestations and correlation between the cutaneous and pulmonary involvement in patients of scleroderma

Jyotsna Oak, Sunil Kumar Singh, Rupali Mathur; Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Mumbai, Maharashtra, India

Background: Scleroderma is a complex heterogeneous disease that is characterised by small vessel vasculopathy, autoantibody production and excessive collagen deposit in skin and internal organs. Pulmonary complication is the most common cause of morbidity and mortality in scleroderma. Early detection and medical intervention are important to delay disease progression and improve survival.

Objectives: The primary objective of the study is to understand the spectrum of cutaneous manifestations in patients of scleroderma and to determine pattern of pulmonary involvement in limited and diffuse disease. Secondary objective is to determine any correlation between cutaneous and pulmonary manifestations.

Methods: This is an observational study done on patients aged 18 years and above fulfilling the American College of Rheumatology criteria attending the Rheumatology OPD with a sample size of 54 over 1 year. Assessment of the cutaneous manifestation is done by the clinical signs of cutaneous involvement and calculation of the Modified Rodnan skin Score. Assessment of pulmonary involvement included chest X ray, HRCT chest, 2 D Echo, pulmonary function test, ABG as per the availability.

Results: 54 patients completed the study (50 females and 4 males). Patients with diffuse Scl was 70.3% and limited cutaneous 29.6%. The Modified Rodnan skin score varied from 7 to 54, mean score was 25.81±10.04 Raynaud’s phenomenon was noted in 90.7% patients. Cutaneous involvement was 100% in which 92.5% skin tightening, 77.7% sclerodactyly, 57.4% digital pits and ulcers, 33.3% diffuse cutaneous edema, 16.6% telangiectasia, 12.9% calcinosis and 11.1% had salt and pepper skin. Pulmonary involvement was noted in 70.3% patients in which 59.2% of Interstitial Lung disease and 16.6% of pulmonary hypertension was seen. 5 out of 16 cases of lcSSc had Interstitial lung disease while 3 out of 16 had pulmonary hypertension. 27 out of 32 cases of dcSSc had ILD while 6 had Pulmonary hypertension.

Conclusion: Cutaneous involvement was universal, however its extent and severity was not significantly correlated with pulmonary manifestations. No significant correlation was observed between skin score, HRCT and PASP for all patients.


  PC178 Scleroderma, Myositis and Overlap Top


Mixed connective tissue disorder

Clinical and immunological profile of Mixed connective tissue disorder: Not so uncommon in India!!

Sham Santhanam; Glenegales Global Hospital, Chennai, Tamil Nadu, India

Aim: To study the clinical and immunological profile of patients with features of mixed connective tissue disorder (MCTD).

Methods: We retrospectively analyzed the data of patients presenting to the rheumatology outpatient department from October 2014 to June 2017. 21 patients satisfied the diagnostic criteria by Kasukawa et al for MCTD and also the Alarcon-Segovia criteria except for the anti U1RNP titer.

Results: Of the 21 patients, 17 were newly diagnosed and rest were already diagnosed with MCTD. Among the 17 patients, 7 patients were being treated as rheumatoid arthritis. All of them were females with median age of 45 years. The median disease duration was 42 months. All of them had inflammatory arthritis, 13/21 had myositis, 11/21 had sclerodactyly, 10/21 had typical history of Raynaud phenomenon, 9/21 had cold peripheries without history. 15/21 tested positive for rheumatoid factor, 2/7 tested positive for ACPA. 18 patients tested positive for ANA by immunofluorescence with speckled pattern and 3 tested positive for ANA by ELISA. All of them tested positive for U1RNP by immunoblot. 3 tested positive for RO -52, 3 SS-A, 2 for nucleosome and 2 for Sm. C4 was low in 8/21 and C3 in 3/21 and 1 for both. 12/21 had interstitial lung diseases (ILD) (10-NSIP; 2- UIP) and 8/21 had pulmonary hypertension (PHT), of which 7 had both ILD and PHT. The median disease duration of patients with ILD was 60 and with PHT also was 60. 5 had coexisting Sjogren's syndrome, 3 had serositis, 2 had nephritis, 1 had episcleritis, 1 had cutaneous vasculitis and 1 trigeminal neuropathy.

Conclusion: MCTD is not so uncommon in India and has to suspected in patients with inflammatory arthritis with subtle symptoms/signs of a connective tissue disorder. The diagnosis helps in the early recognition of ILD, PHT and in treating them adequately.


  PC179 Scleroderma, Myositis and Overlap Top


Localized scleroderma: A case series from a tertiary care centre

Sreedevi Suresh, Thiruchengode Natesan Tamilselvam, Selvakumar Balameena, Euphrasia Latha, Myilasamy Saravanan, C Saranya, Vignesh Mantaram, A Aravindan; Postgraduate Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Localised scleroderma is an entity with involvement of skin mostly in the form of sclerosis, rarely involving subcutaneous tissue and muscle. Two types of localised scleroderma are known of which linear is more common in children while morphea in adults.

Methods: 8 patients were found to have localised scleroderma on clinical examination and they underwent skin biopsy.

Results: Of the 8 patients studied, 2 cases had linear scleroderma on limbs,1 had en coup sabre on face, 1 circumscribed superficial morphea, 2 mixed morphea in extremities and 2 generalised morphea were found. Male:female ratio being 2:6.Average duration of illness 2.5yrs.Mean age being 18.5+9.81 years. one female patient evolved in to systemic sclerosis over a period of 3 years. Contracture was noted 7 year old boy with linear scleroderma. A female patient had SLE. An adult having deep morphea had severe wasting of underlying subcutaneous tissue with infected wound.

Conclusion: Localised scleroderma is not uncommon and is noted in both adults and children. Localised form cause local disfigurement and impact on cosmetic and aesthetic aspects it rarely evolve into systemic sclerosis. Early recognition is of paramount importance to initiate early intervention.


  PC180 Scleroderma, Myositis and Overlap Top


A single centre study of Clinical Profile and Pattern of ILD in Systemic Sclerosis (SSc) Patients from North India

Udit Aggarwal*, GhanShyam Pangtey*, Taru Garg**, Pooja Abbey***; Departments of *Medicine, **Dermatology and ***Radio-diagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi, India

Background: SSc is an uncommon autoimmune disease and very small data is available from India therefore a study was planned to find the Clinical profile and pattern of ILD in SSc patients.

Methods: It was a Descriptive observational study with a study period of 2 years. A total of 30 patients with Systemic sclerosis score of >9 according to 2013 ACR/EULAR criteria were enrolled after obtaining an informed consent. Each patient was subjected to appropriate history, clinical examination and investigations including- blood investigations, chest radiograph, HRCT and PFT. All data was entered in Excel sheet and analysis was done using SPSS version 21.0. The p-value <0.05 was taken as significant.

Results: A total of 30 patients enrolled. All patients were females. The mean age of the patients was 37.93+11.04 years and the average disease duration was 9+4.48 years. SSc patients were divided into two types, lcSSc (80%) and dcSSc (20%). The frequency of various variables was GERD (56.67%), cutaneous manifestations - skin thickening (93.33%), raynauds phenomenon (93.33%), fingertip lesions (46.67%), telengiectasias (30%). Auto-antibodies were positive in 80% patients. ILD was found in 24 (80%) patients. Out of the 24 patients with ILD, 10 (41.67%) patients had UIP ILD and 14 (58.33%) patients had NON-UIP ILD in which NSIP was the most common (11/14=78.57%), followed by COP (2/14=14.28%) and unclassifiable pneumonia (1/14=7.14%).

Discussion: Mean age of our patients was 37.93 years and duration of the disease was 9+4.48 years suggesting a young cohort with chronic disease. Lung involvement was seen in 80% SSc patients. Previous studies from India also reported high proportion of lung involvement and NSIP ILD to be the most predominant form of ILD [1-2].

Conclusion: Lung involvement in the form of ILD was seen in 80% of the SSc patients in our North Indian cohort. NSIP was the most common form of ILD. As lung involvement and respiratory failure is predominant cause of mortality in SSc. The future long term comparative prospective studies should be done to know the prognosis of NSIP/UIP ILD of SSc vs ILD of idiopathic cause. 1. Krishnamurthy V, et al 2. Sharma VK, et al


  PC181 OA, Osteoporosis Crystal Arthropathy Top


Does fat distribution in body correlates with radiological features of osteoarthritis?

Atul Kakar, Darshit Shah, Atul Gogia, V R Manohar, Rishikesh Desai, Manish Dhawan, K K Saxena; Sir Ganga Ram Institute of Post Graduate Medical Education and Research, New Delhi, India

Introduction: Osteoarthritis is multifactorial and is an interplay of systemic and local factors. Obesity is a modifiable risk factor for osteoarthritis. It is usually thought that adipose mass increases the mechanical load on the joint leading to increase risk of osteoarthritis.

Objective: If body fat distribution correlates with radiological features of osteoarthritis?

Methodology: Patients more than 50 years having symptomatic osteoarthritis were enrolled. Patients with any other inflammatory arthritis, unilateral osteoarthritis, who had undergone knee replacement and with history of prior sports injury were excluded. Body mass Index (BMI), Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) questionnaire, Kellgren Lawrence (KL) grading and fat distribution was recorded in the study.

Results: 220 patients with knee osteoarthritis were diagnosed during study period and 28 were excluded from study. Maximum number of patients (47.9%) were in 7th decade of life and 71.9% of subjects were female. Mean WOMAC score was 72.76, mean KL grade was 2.88. The lean body mass (Muscle mass, Bone mass) was 61.25% and fat was 38.75% measured with DEXA scan. The body fat distribution of subjects were classified into upper limb fat, lower limb fat and trunk fat with the help of fat analyser. Neither upper limb fat, trunk fat nor the lower body fat percent correlated with either KL or WOMAC. WOMAC score had a significant correlation with BMI (r value 0.265, p< 0.001) except stiffness (r value 0.009). BMI was found to be associated with KL grade (r value 0.320, p<0.001). Our study did not show any correlation of body fat distribution with WOMAC score or KL grading.

Conclusion: Increased BMI has a strong positive association with poor patient reported outcomes measured by WOMAC scoring. Also, a similar positive association was observed with KL grading. Body fat distribution did not show any correlation with WOMAC score or KL grading.


  PC182 OA, Osteoporosis Crystal Arthropathy Top


Alteration of serum Biomarkers in primary knee Osteoarthritis

Satarupa Dutta, Subhankar Halder, Biswadip Ghosh; Department of Rheumatology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

Background: Osteoarthritis (OA) is one of the most prevalent of the chronic rheumatic diseases and is a leading cause of pain and disability in most countries worldwide. Pain and disability is no less than any other common inflammatory joint diseases. Many patients with osteoarthritis show some features of inflammation. Traditional thinking is that, OA is a degenerative disease with main site of pathology at the cartilage.

Objective: To demonstrate evidence and extent of inflammation in primary osteoarthritis of knee.

Methods: Total 69 patients with knee OA with clinical inflammation (hot and swollen knee) were recruited. Patients were subdivided in systemic Non-Inflammatory group (14 Patients) and Inflammatory group (55 Patients) on the basis of serum levels of Erythrocyte Sedimentation Rate and C Reactive Protein. WOMAC Index (Modified-CRD Pune Version) of these patients were measured. Then Osteoprotegerin (OPG), Receptor Activator of Nuclear Factor Kappa-B Ligand (RANKL), Myeloperoxidase (MPO), Fibrin, Human COLL2-1 NO2 from the serum of the patients were measured by using ELISA and compared with 10 healthy controls.

Results: Statistical analysis shows that, the serum level of RANKL is significantly (P<0.01) lower in patients of Inflammatory group, when compared with healthy control group and serum level of OPG is significantly (P<0.001) higher in both Non-Inflammatory group and Inflammatory group, when compared with healthy control group. Other biomarkers, i.e, MPO, Fibrin, Human COLL2-1 NO2 showed no significant change in both the patient groups when compared with healthy control group.

Conclusion: Increased serum level of osteoprotegerin and decreased serum level of RANKL indicate abnormality in bone in patients with knee osteoarthritis. Serum level of MPO, Fibrin and COLL2-1 NO2, which are marker of cartilage damage, showed no significant change in patients with primary OA. It is time to shift our attention outside cartilage in knee OA, particularly in patients with systemic inflammation, as evidenced in raised ESR/CRP. Anti-inflammatory drugs may be helpful for treatment of the patients with primary Knee OA.


  PC183 OA, Osteoporosis Crystal Arthropathy Top


Effectiveness and safety of a combination of intra-articular corticosteroid and local anesthetic in Indian patients with knee osteoarthritis: A pilot study

Rakesh Kumar Jagdish1*, M K Bhatnagar1,2, Ayush Malhotra1; 1Santosh Medical College and Hospital, Ghaziabad, Uttar Pradesh, 2Lady Harding Medical College, New Delhi, India

Background: Osteoarthritis is a chronic degenerative disorder of multifactorial etiology characterized by the loss of articular cartilage, resulting in joint pain, stiffness, swelling, and disability without any clear answer to its treatment and cure. Studies from intra-articular steroid with local anesthetic uses in osteoarthritis are rare from India.

Objective: To determine the effectiveness and safety of administering a combination of intra-articular corticosteroid and local anesthetic in Indian patients with knee osteoarthritis.

Methods: This, prospective, open-label, observational single-center pilot study was conducted at the Rheumatology Clinic of a tertiary care centre, from December 2015 to December 2016. This, prospective, open-label, observational single-center pilot study included patients(n=20) between 35-70 years of age, suffering from chronic knee pain for at least three months prior to inclusion, with a clinical or radiological diagnosis of knee osteoarthritis, dissatisfied with previous non-surgical management. Patients were administered injection methylprednisolone 80 mg (2ml) plus lignocaine 1% (0.5 ml) intra-articularly which were followed with five scheduled visits i.e. baseline (visit 1), day 1 (visit 2), 6 weeks (visit 3), 12 weeks (visit 4), and 24 weeks (visit 5). Patients were evaluated on a Visual Analogue Scale [VAS] for pain and patient reported self-assessment questionnaire to evaluate other clinical effectiveness parameters.

Results: Mean age of the study population was 52.55b + 7.91 years. Majority (85%) were females. After administration of the injection, pain (as measured by the VAS scale) improved within a day and there was complete (100%) pain relief in all patients (as per subjective assessment) at week 1. The VAS score reduced from 8.90 + 0.968 at baseline to 6.35 + 1.387 on day 1 (mean reduction of 2.55 + 1.191) and 5.30 + 0.923 at week 1 (mean reduction of -3.60 + 1.273). For each of the clinical effectiveness parameters, a significantly greater proportion of patients showed improved status than those who worsened or remained the same. Seventy percent (14/20) patients reported decrease in frequency of non-steroidal anti-inflammatory drug (NSAID) usage (p=0.0368).

Conclusion: Combination injection of intra-articular corticosteroid and local anesthetic is safe and effective in Indian patients with osteoarthritis. It achieves immediate pain relief, with effects lasting for at least 6 months and helps decrease NSAID usage in most patients.


  PC184 Fibromyalgia Top


ACR 2016 criteria for fibromyalgia: Poor specificity in a tertiary setting

Sakir Ahmed, Amita Aggarwal, Able Lawrence; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Introduction: The tender point based 1990 ACR criteria for fibromyalgia (FM) is physician centric. The 2010 Fibromyalgia Diagnostic Criteria was designed for primary care, and is more patient centric. The proposed 2016 ACR criteria simplifies diagnosis and incorporates persistent widespread pain as entry criterion. Though it has not been validated in tertiary care, it is being recommended for the same.

Objective: To validate the ACR 2016 criteria for FM in a tertiary setting.

Methodology: Patients referred to a tertiary care centre with suspicion of Fibromyalgia were evaluated using the Proposed 2016 revision. Patients with inflammatory rheumatological diseases were excluded. Considering the American College of Rheumatology (ACR) 1990 Classification and Expert opinion as gold standards, sensitivity, specificity, and likelihood ratios were calculated. Also, validated Hindi language versions of Brief Patient Health Questionnaire (BPHQ), Generalised Anxiety Disorder-7 (GAD7), and Toronto Alexithymia Scale-20 (TAS20) were filled up.

Results: 150 patients were recruited of which 110 were diagnosed as FM by the expert. ACR 1990 criteria was met in 115. 95 fulfilled the ACR 2016 criteria. Compared to expert opinion, sensitivity, specificity, positive and negative likelihood ratios were 70%, 55%, 81% and 40% respectively. Compared to ACR 1990 criteria, these were 81%, 31%, 66% and 48% respectively. The ACR 1990 criteria had sensitivity and specificity of 92% and 65% respectively compared to expert opinion. Visual Analogue Scale (VAS) for pain and anxiety score (GAD7) were significantly higher in FM than non-FMS. There was no significant difference between depression (BPHQ) and alexithymia (TQS20) scores.

Discussion: Non-tender point based criteria have been validated in primary care. However, in tertiary care where patients are referred to as fibromyalgia, there are mimics with similar comorbidities as evident by high BPHQ, GAD7 and TAS20 scores. Even after exclusion of other rheumatological conditions, the 2016 Criteria has poor specificity. Thus, it should be used as a screening tool than a diagnostic criterion in tertiary care.


  PC185 Paediatric Rheumatology Top


HLA-B*27 subtypes, clinical phenotype and cytokine profile of the south Indian Tamil enthesitis related arthritis Patients: A cross-section observational study

K G Chengappa, Emmanual Dantis, Jain Ankit, Seth Gaurav, Belani Pooja, K C Shanoj, V S Negi; Department of Clinical Immunology, JIPMER, Puducherry, India

Background: Enthesitis related arthritis is a major subgroup of JIA reported from India. The distribution of HLA-B*27 subtypes has shown variance in ERA patients from different populations. There is a paucity of data on the possibility of an association between disease manifestation, HLA-B*27 subtype and the cytokine profile of these patients.

Objectives: 1. To assess the distribution of HLA-B*27 subtypes and disease phenotypes in JIA-ERA. 2. To assess the cytokine profile (TNF- alpha, TGF- beta, IL-6, IL-10, IL-17, IL-21, IL-22, IL-23) in ERA patients.

Methods: JIA patients attending the Clinical Immunology OPD in our institute were classified into different disease subgroups and the ERA patients were enrolled into our study. Relevant clinical and demographic data were documented in a predesigned proforma. HLA- B*27 typing was done for all the ERA patients on ARMS PCR and the positive samples were subjected to HLA-B*27 subtyping. The above mentioned cytokines were analysed in the plasma of 154 patients and 18 controls by ELISA and all the variables were analysed using appropriate statistical tests.

Results: Out of the 160 patients enrolled in our study 109 (68.1%) were HLA-B*27 positive [Figure 1]. The HLA-B*27 positive patients had a significantly higher number of deformities, family members with spondyloarthrtis and uveitis (p=<0.05) as compared to HLA-B*27 negative patients. HLA-B*27 subtyping was done in 98 samples which showed HLA-B*27:04,:05,:07, and :02 subtypes [Figure 1] Individuals with HLA-B*27:04 differed from the :05 patients in having an earlier age of disease onset 12.4 +/- 2.6 years and a lower incidence of a family member suffering from spondyloarthroitis (PRR-0.34). The ERA patients had a significantly higher concentration of TNF-α, Interleukin-6, 17, 22 and 23 than healthy controls (p=<0.05). Interleukin-10 and TGF- Î2 levels showed a trend towards lower levels in cases but failed to attain statistical significance.



Conclusions: Our study population has a higher prevalence of HLA-B*27:04 subtype which significantly differs from other studies across India, possibly reflecting a pathogen driven selection of the:04 allele. We found higher levels of Th-1 and Th-17 cytokines in the peripheral blood among the ERA patients, thus confirming the role of Th-1 and IL-17/23 axis in the disease pathophysiology.


  PC186 Paediatric Rheumatology Top


Clinical and ultrasonographic assessment of peripheral enthesitis and correlation with disease activity in juvenile idiopathic arthritis â€"enthesitis related arthritis

Harikrishnan V, Rasmi Ranjan Sahoo, Saumya Ranjan Tripathy, Urmila Dhakad, Puneet Kumar, Anupam Wakhlu, Siddharth Kumar Das; Department of Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India

Background: Enthesitis is a distinct clinical hallmark of spondyloarthropathy in children and adults. Ultrasonography (US) is a powerful tool to detect enthesitis. Studies on US detected enthesitis in juvenile idiopathic arthritis - enthesitis related arthritis (JIA-ERA) from India are limited.

Objective: This study aimed to assess the prevalence of clinical and US detected enthesitis in JIA and to correlate with disease activity.

Methods: 51 JIA-ERA patients fulfilling the ILAR classification criteria were evaluated clinically and sonologically. Enthesitis was assessed using the MASES (Maastricht Ankylosing Spondylitis enthesitis score) and additionally at peripheral entheseal sites (bilateral plantar fascia, Achilles tendon, quadriceps tendon and patellar tendons proximally and distally). The disease activity was assessed using JADAS, BASDAI and BASFI.US was done using multi-frequency linear-array transducer (8-13MHz) of Logiq E; GE Medical Systems. B-mode settings used: dynamic range (40-50dB); GS- frequency (11-13 MHz); GS gain (60 dB). Power Doppler (PD) mode settings used: pulsed repetition frequency (400-800 Hz); PD-gain (highest possible gain with minimum background noise). US parameters assessed were according to OMERACT 2014 guidelines. US score was calculated by adding 1 point for each finding and the grade of PD. PD was graded on a scale of 0-3 and PD score was calculated by adding the grade of PD. Prevalence of clinical and US detected enthesitis was estimated and compared and correlated with disease activity parameters.

Results: The mean age of patients at examination was 14.3± 3.2 years with mean duration of disease 34.6±4.9 months. Male: Female ratio was 7.5:1. Clinical enthesitis was seen in 36 patients (70.5%) and US detected enthesitis in all (p<0.0001). The mean JADAS score, BASDAI and BASFI were 17.1±10.2, 4.1 ±2.4and 37.2±27.2 respectively, indicating active disease. The US enthesitis score correlated positively with JADAS (r = 0.3, P=0.03), BASDAI (r = 0.39, P=0.005) and BASFI(r = 0.38, P=0.006). PD score correlated positively with BASFI (r= 0.33, P- 0.02), but did not correlate with JADAS (r = 0.22, P=0.1), BASDAI (r= 0.24, P= 0.08).

Conclusion: US is more sensitive in detecting enthesitis in JIA-ERA and US score positively correlated with disease activity.


  PC187 Paediatric Rheumatology Top


Juvenile Reactive Arthritis: Is it different from other childhood spondyloarthropathies?

Latika Gupta, Sakir Ahmed, Abhishek Zanwar, Ramnath Misra, Amita Aggarwal; Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Introduction: Juvenile reA (jReA) forms part of the spectrum of spondyloarthritides of childhood. Only small case series of jReA are described in literature. Thus we studied clinical profile of jReA at our centre.

Methods: Data was retrieved from files of jReA patients (Braun and Seiper's criteria, 1990), and compared with other childhood SpA (ERA, AS, USpA).

Results: 60 children (52 M, 8F) of jReA of age 15.5 (12-18) years and disease duration 2.75 (1-36) months were assessed. Post enteric ReA was the most type, seen in 48 children. Knee joint was most often involved (45). Other clinical features were IBP (20), dactylitis (13), enthesitis (18), skin changes (13) and eye involvement (12). Five had positive family history of spA. Of the 17 followed up for ≥6 months, 14 (82.3%) developed chronic/relapsing disease. Assessment of 18 children with undifferentiated spondyloarthritis (ReA like presentation without preceding history of infection) yielded similar clinico-laboratory profile to jReA. Of the 12 followed up over 9.5 (4.8-37) months, 7 (58.3%) evolved to chronicity. 41 patients of AS with age of disease onset 15(13-16.1) years and disease duration 6.5 (3-12) years were studied. Gender distribution was similar to jReA. Patients with jAS had delayed symptoms onset (15 vs. 13 years, p=0.02) as compared with jReA. Axial symptoms occurred in all with jAS (RR2.4, p<0.0001) while peripheral arthritis was less common (RR 0.5, p<0.0001). Acute phase reactant levels were similarly elevated in both groups. 60 children with JIA-ERA of age 15 (13-16), disease duration 25 (10.8-48) and JSPADA 3.3 (1.9-5.0) were also assessed. Children with ERA had similar gender distribution but longer disease duration (25 vs 2.5 months, p<0.0001), and higher age of disease onset (15 vs. 13, p=0.02) as compared with jReA. Enthesitis was more common in ERA (RR 2.4, P<0.0001). 14 children were reassessed at 3 months, of which 3 were in remission. Rates of remission were similar to those in jReA (p=0.23).

Conclusion: JReA and other spondyloarthritides of childhood form a continuum with similar clinico-laboratory profiles with minor differences suggesting possibly shared pathogenesis.


  PC188 Paediatric Rheumatology Top


Factors affecting disease activity and damage in JIA, a prospective observational study

Narendra Vadlamudi, I R Varaprasad, Liza Rajasekhar; Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background: There are few reports on prospectively followed cohorts of JIA from India. Data regarding factors affecting health care utilization and its influence on disease activity and damage is scarce.

Aim: To Study outcomes in JIA using validated measures of disease activity, damage and identify social economic factors and barriers affecting disease activity and damage.

Methods: All patients satisfying ILAR criteria of JIA and attending the Rheumatology services over a period of 3 months (October 2016-December 2016) were enrolled prospectively in this study. Evaluation included complete history, physical examination, and laboratory investigation. Disease activity was assessed by juvenile arthritis disease activity score (JADAS), in juvenile spondyloarthritis (JSpA) and enthesitis related arthritis (ERA) by JSpA disease activity score (JSpADA). Damage was assessed by juvenile arthritis damage index (JADI). Growth was assessed using IAP growth charts. Socio-economic (SE) factors studied were kuppuswami SE status, time to diagnosis, distance to healthcare, drug compliance, and patient reported barriers for therapy. Descriptive statistics are presented as mean (SD).Continuous variables were analysed using Spearman's correlation coefficient and categorical variables using independent sample t-test. Multiple regression was done using time to diagnosis, duration of disease, expenses, age at diagnosis, distance to medical care and category of JIA as factors affecting JADI.

Results: One-hundred and thirteen patients females (57.5 %) were enrolled. Mean age was 12.1 (5.4) years with a median disease duration of 2.5 (0.1 to 20) years. The most common subgroup was poly-articular JIA 52% followed by ERA 20%, SOJIA 16%, oligo-articular 9.7% and PSA 1 [Table 1]. Mean time to diagnosis was 1.54 (1.77) years. 18% has health insurance, Monthly expenditure was Rs 2106 (1353). [Table 1] lists the outcomes, socioeconomic status and barriers to therapy. High disease activity and damage was associated with lower height and weight achieved (r= -0.198, p < 0.054; r= -0.248 p =0.008, respectively). Female sex, longer duration of disease (p=0.018, p=0.00 respectively) had higher damage and damage was associated with increased health care spending (p=0.05).



Conclusions: Patients with JIA at presentation have high disease activity and articular damage leading to poor growth. Females, longer disease duration accumulates more damage, leading to increased health expenditure.


  PC189 Paediatric Rheumatology Top


Why do patients drop out from pediatric rheumatology clinics?: A telephonic survey of 146 patients

Abhay Shivpuri*, Manjari Agarwal**, Shilpa Sharma#, Sujata Sawhney$; *,**,$Division of Pediatric Rheumatology, Institute of Child Health, Sir Ganga Ram Hospital,New Delhi, #KLE University, Belgaum, Karnataka, India

Background: Lack of regular follow up and a high drop- out rate is a common cause of concern in PRCs. This can cause considerable concerns for the treating team and also cause treatment disruptions and place the children at risk of ongoing disease, drug toxicity and potential disease related damage. Little information is available in the literature for the causes of such drop outs. This study was designed to systematically analyze the reasons for drop outs from follow up. To the best of our knowledge this has not been from any centre in India.

Methods: 146 consecutive patients who had not attended the clinic for more than one year were contacted telephonically and requested to complete a questionnaire over the phone that took 10-15 minutes to complete. For the purpose of this study, children with Juvenile idiopathic arthritis and childhood SLE were contacted. The questionnaire included details of child's age, sex, primary diagnosis, socio economic status, current medical status and possible reasons for drop out. Of the total 146 patients contacted, 31 were excluded as the telephone numbers were unreachable or had changed. A further 14 families declined consent for telephonic interview. Thus data was collected on 101 children.

Results: Data was collected from 101 patients aged 1.5 years to 16 years. The demographic details and reasons for drop-out are listed in [Table 1].



Conclusion: This study has shown that of the families who dropped out from clinical care, 41.5% were off all drugs, were well and did not seek care elsewhere. 60% (59/101) required medical care but only 35% (36/101) of them sought medical care with other treating units. Cost of travel to get care and medical cost incurred was a major reason for dropping out as seen in 63%. One child had expired and one family was visiting a faith healer. 9% families has personal issues due to which they dropped out and 10% families had relocated and thus had changed medical teams. This study thus highlights the necessity of sensitizing families regarding regular follow up with the treating unit to help prevent disease related damage and possible comorbidities.


  PC190 Paediatric Rheumatology Top


Assessment of treatment responses and estimation of the extent of remission in the different categories of Juvenile Idiopathic Arthritis: A single-centre study

Debanjali Sinha, Sumantro Mondal, Pradyot Sinha Mahapatra, Alakendu Ghosh; Department of Rheumatology, IPGMER and SSKM Hospital, Kolkata, West Bengal, India Background: Juvenile Idiopathic Arthritis (JIA) is the most common chronic rheumatologic disease in children. Optimum treatment is required for their overall improvement.

Objective: To assess treatment responses in the different categories of JIA.

Methods: This is a single-centre, prospective study on children with JIA done between 2015-2017. They were treated in a tertiary centre according to ACR recommendations for JIA. Responses to treatment were monitored by JADAS 27, achievement of JADAS inactive or low disease state, Wallace remission criteria and PedACR30 and 70 responses and quantification of CHAQ-Disability index at end of follow-up.

Results: 220 children with JIA were included in the study: 80 (36.3%) had Polyarticular JIA, 71 (32.2%) Enthesitis-related, 34 (15.4%) each had Systemic and Oligoarticular and 1 (0.5%) Psoriatic JIA. After a mean treatment duration of 1.63 years, there was significant drop in mean JADAS27 (from 26.8 to 10). There was significant difference in follow-up JADAS27 between Systemic and Oligoarticular JIA; being non-significant between the other groups. Inactive disease state according to JADAS27 was achieved in 14% children: 13.75% Polyarticular, 15.49% Enthesitis-related, 8.82% Systemic and 17.65% of Oligoarticular JIA; no significant difference prevailed between the different groups. JADAS27 low disease activity was achieved in 19/80 (23.8%) Polyarticular, 19/71 (26.8%) Enthesitis-related, 8/34 (23.5%) Systemic and 15/35 (44.1%) of Oligoarticular JIA. Wallace remission criteria was satisfied by 15% of Polyarticular, 39.4% of Enthesitis-related, 14.7% Systemic and 26.47% of Oligoarticular JIA; there was significant difference between the groups. PedACR30 response was achieved in 91% of Polyarticular, 77.46% Enthesitis-related, 76.5% Systemic and 82.4% of Oligoarticular JIA. PedACR70 response was seen in 65% of Polyarticular, 43.6% Enthesitis-related and 47% each in the Systemic and Oligoarticular JIA. PedACR70 was significantly different between groups, but not PedACR30. Significantly more steroid (88.45%) and biologic (44.1%) was used in Systemic JIA. Mean CHAQ-DI at follow-up was 0.5:- 0.31 Polyarticular, 0.23 Enthesitis-related, 1.37 Systemic and 0.08 in Oligoarticular JIA. CHAQ-DI is significantly more in Systemic and Polyarticular JIA versus Oligoarticular.

Conclusion: Remission occurred in only 14% children with JIA, despite optimum treatment. Disability was most in Systemic JIA. This is the first Indian study of its kind.


  PC191 paediatric rheumatology Top


Experience with Biologic therapy in children with Juvenile Idiopathic Arthritis â€" A single-centre study from the eastern part of India

Debanjali Sinha, Sumantro Mondal, Pradyot Sinha Mahapatra, Alakendu Ghosh; Department of Rheumatology, IPGMER and SSKM Hospital, Kolkata, West Bengal, India

Background: Biologics enable better disease control and prevent or retard damage due to active disease in a substantial number of children with Juvenile Idiopathic arthritis (JIA), refractory to conventional treatment.

Objective: To assess the treatment responses to biologic therapy in the different categories of JIA.

Methods: This is a single-centre, prospective study of children with Juvenile Idiopathic arthritis, who were refractory to conventional treatment and required treatment with biologic therapy. The study was done in the study period 2015-2017. After biologics they were followed up for 6 months. We followed the ACR 2013 (for Systemic JIA) and 2011 (for the rest) treatment recommendations for JIA. The responses to treatment were monitored by JADAS27 based on whether inactive (≤1) or low disease activity (≤2 in oligoarthritis and ≤3.8 in polyarthritis) is achieved or not.

Results: A total of 220 children were included in the study: 80 (36.3%) had Polyarticular JIA, 71 (32.2%) Enthesitis related, 34 (15.4%) each had Systemic and Oligoarticular and 1 (0.5%) Psoriatic JIA. Biologic therapy was given to 28/220 (12.7%) children, out of which 15/28 (53.6%) was given to Systemic JIA, 7/28 (25%) to Enthesitis related JIA. 5/28 (17.9%) to RF+ve Polyarticular JIA (none in RF-ve) and 1/28 (3.6%) to Oligoarticular JIA. Biologic use was significantly more in Systemic JIA, 14/34 (41.2%) in comparison to the other variants, 7/71 (9.8%) of Enthesitis related JIA, 5/80 (6.2%) of Polyarticular JIA and 1/34 (3%) of Oligoarticular JIA. In Systemic JIA, Tocilizumab was used in all patients, Polyarticular JIA patients received Rituximab and the Oligoarticular and ERJIA children received Anti-TNF (5 Infliximab and 3 Etanercept). At 6 months, 19/28 (67.9%) of the children achieved JADAS27 inactive state, 3/28 (10.7%) achieved JADAS27 low disease activity and 6/28 (21.4%) children flared within 6 months. 66.7% of Systemic, 80% of Polyarticular, 100% of Enhesitis related and none of Oligoarticular JIA responded to biologics. Out of the 6 children who flared, 4 were Systemic JIA, 1 Polyarticular and 1 Oligoarticular JIA.

Conclusion: Inspite of biologic therapy, 21% children with JIA flared within 6 months. Longer follow-up with larger number of children is needed in future.


  PC192 Paediatric Rheumatology Top


Juvenile systemic sclerosis: Diffuse more than limited disease

Sakir Ahmed, Able Lawrence, Vikas Agarwal, Ramnath Misra, Amita Aggarwal; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Introduction: Among the connective tissue diseases in children, Juvenile Systemic Sclerosis (JSSc) is probably the rarest. One multi-national study had 155 patients from 29 centres. No large series are available from our country though we have previously reported a series of 23 patients.

Methods: Retrospective review was done of case records of systemic sclerosis patients seen between 1990 to 2017 at a large tertiary care center, with age of onset less than 16 years, and fulfilling 2007 classification criteria for JSSc. Localized scleroderma and overlap syndromes were excluded. All results are given as median (IQR)

Results: Among more than 725 patients of SSc seen at this centre 64 (8.8%) had JSSc. Most were girls (53/64). Median age at disease onset was 14 (11-15) and median time to diagnosis was 4 (1-8) years. 50 had diffuse SSc while 14 had limited SSc. Median modified Rodnan skin score was 24 (17-31) for diffuse SSc and 12 (6 -16) for limited SSc. Twenty three patients had interstitital lung disease (ILD), 19 had gastroesophageal reflux, 10 had pulmonary arterial hypertension (PAH) and 3 had inflammatory myositis. No renal crisis was seen. There was no significant difference in clinical features of patients with limited or diffuse disease. ANA was positive in 48 patients and ENA data was available in 33 patients of which 27 had antibodies to topoisomerase I (Scl-70), 9 to Ro, 1 each to Jo-1 and U1RNP whereas 3 had no antibodies to ENA. Among 64 only 36 patients followed up. The median follow up was 1 (0.5-3.75) years. There was one death, one avascular necrosis of femoral head, and 4 cases of gangrene in the patients who had followed up. There was no statistically significant difference between clinical features or follow-up between the patients presenting in the first fifteen years (1988-2002) or the latter fifteen (2003-2017).

Conclusion: Juvenile SSc is rare even at a large tertiary care hospital. There is a need to increase awareness for early diagnosis. A multi-institutional registry across India is needed too.


  PC193 Paediatric Rheumatology Top


Clinical profile and short term outcome in Systemic onset variant of Juvenile Idiopathic Arthritis

Pooja Belani, K G Chengappa, Gaurav Seth, K C Shanoj, Manoj Khatri, V S Negi; Department of Clinical Immunology, JIPMER, Puducherry, India

Background: Systemic onset juvenile idiopathic arthritis (SOJIA) is the category of JIA considered to be most commonly associated with morbidity and mortality.

Objectives: To analyze the clinical and demographic profile and factors predicting treatment response in SOJIA.

Methods: Fifty five patients of SOJIA under follow up at the Department of Clinical Immunology, JIPMER, were analyzed retrospectively for their clinical and demographic features. Of them 40 had follow up data of minimum 2 years and analyzed for treatment response and disease associated damage. JADAS10 was calculated at baseline, at the start of specific treatment, at 1 year and at 2 years after starting treatment. JADI A and JADI E was calculated at the end of 2 years.

Results: The mean age of the study participants was 8.41ï'±3.95 years. Male: Female ratio was 3:2. Mean JADAS 10 at baseline was 18.96 ï'± 6.9. The median disease duration was 4 months (IQR 1, 18). The ratio of male: female and JADAS 10 at baseline did not differ significantly between responders and non-responders. Fever and arthritis were the most common manifestations (100% and 78% patients respectively). Rash and lymphadenopathy was present in 36% of study subjects. Macrophage Activation Syndrome (MAS) was the initial presentation in 3 patients (5.4%).Mean age at onset in responders was 6.27 ï'± 2.9 years as compared to non-responders 8.9 ï'± 3.5 years, with p=0.015,mean diff (CI)= -2.62 [(-4.70)- (-0.54)].Response rate in systemic predominant SOJIA although higher as compared to arthritis predominant (percentage of responder versus non responders in systemic predominant was 63.63 % and 36.36% respectively, versus arthritis predominant 44.8% and 55.1% respectively, with, but failed to reach statistical significance (P=0.288 OR (95% CI=0.464(0.11-1.94). Median JADI A score amongst responders versus non-responder was significantly different (p=0.024).

Conclusion: Retrospective analysis of our data suggests responders had relatively young age at onset. Though baseline disease didn’t differ between responders and non responders, the response was better in syste.


  PC194 Paediatric Rheumatology Top


Spectrum of Clinical Manifestations of Juvenile Idiopathic arthritis in the Eastern part of India

Debanjali Sinha, Sumantro Mondal, Pradyot Sinha Mahapatra, Alakendu Ghosh; Department of Rheumatology, IPGMER and SSKM Hospital, Kolkata, West Bengal, India

Background: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatologic disease in children. However, there is scarce data describing JIA in India.

Objective: To assess the disease characteristics of JIA in the eastern part of India.

Methods: This is a single-centre, cross-sectional study on children with Juvenile Idiopathic Arthritis conducted in the time period 2015-2017. The baseline demographic parameters, baseline disease activity as measured by JADAS27 and status of growth were collected.

Results: 220 children of JIA were included in the study: 80 (36.3%) had Polyarticular, 71 (32.2%) Enthesitis-related, 34 (15.4%) each had Systemic and Oligoarticular and 1 (0.5%) Psoriatic JIA. The mean age of onset of the patients were: 8.83 years for Polyarticular, 10.82 years for Enthesitis-related, 7.33 years for Systemic and 7.14 years for Oligoarticular JIA. The age of onset of Enthesitis-related JIA was significantly more than the others. Female:male ratios were 3.4:1 in Polyarticular, 1:9 Enthesitis-related, 1.3:1 Systemic and 1.4:1 Oligoarticular JIA. RF was positive in 47/80 (58.8%) of Polyarticular, HLA-B27 was positive in 48/71 (67.6%) Enthesitis-related and ANA positive in 11.8% of Oligoarticular JIA. 29/34 (85.3%) of Oligoarticular JIA were of Persistent variety, rest Extended. Uveitis was found in 8/71 (11.3%) of Enthesitis-related JIA. No subclinical or evident uveitis was detected in the other variants of JIA. Mean delay from onset of disease to initiation of treatment was 1.7 years. Mean baseline JADAS27 and cJADAS were 28.7 and 25.1 overall, 36.7 and 31.6 in Polyarticular, 21.1 and 17.2 in Enthesitis-related, 40.5 and 32.9 in Systemic and 16.5 and 13.7 in Oligoarticular JIA respectively. Two children with Polyarticular JIA had Interstitial Lung Disease and one with Oligoarticular JIA had associated an Primary Immunodeficiency Syndrome. Two children with Systemic JIA had Macrophage Activation syndrome and another had associated features of Enthesitis-related JIA including sacroiliitis. 8.2% children had stunting of growth: 8/35 (23.6%) in Systemic, 7/80 (8.8%) Polyarticular, 3/71 (4.2%) Enthesitis-related and none in Oligoarticular JIA. 37.2% children were on steroids (88.24% in Systemic JIA).

Conclusion: Polyarticular JIA is the commonest type of JIA in this study. Certain disease characteristics are different from studies in western countries.


  PC195 Paediatric Rheumatology Top


Paediatric takayasu arteritis: A retrospective study

Shah Romi, C Balakrishnan, Mangat Gurmeet, Kakade Girish; P.D.Hinduja National Hospital, Mumbai, Maharashtra, India

Background: paediatric takayasu is underdiagnosed and undertreated entity with various management related issues.

Objectives: Objective was to study the clinical features and outcome of patients with takayasu's aortoarteritis in the paediatric age.

Methods: It's a retrospective study of patietns over last 7 years who were referred from paediatric department with a diagnosis of takayasu's arteritis.

Results: Over the last 7 years we saw 5 (females 3) patients with paediatric Takayasu's aortoarteritis. Median age was 13 years (range: 5-16 years). Median duration of illness before diagnosis was 8 months (range: 1-72 months). Median follow up was 24 months (0 -80 months). Presenting complaints were fatigue (3/5), fever (3/5), headache (2/5), weight loss (2/5), carotidynia (2/5), cardiac failure (1/5), myalgia (1/5) and dizziness (1/5). Median ESR was 55mm/hr (range: 25 -136) at diagnosis. CT angio was showing type 5 of angiographic features in all patients. All were treated with steroid. Four were treated with methotrexate and 2 responded. Three patients were given mycophenolate mofetil. Two of these were MTX non-responders. One patient did not respond to either MTX or MMF was first given two doses of Infliximab and is now on maintenance steroid and azathioprine. On follow up ESR reduced to median 22 (range: 8 -42) patients. PET scan was done in 2 patients. One of these has shown significant resolution of inflammation on the repeat scan. 2 patients underwent surgical procedure without any complications.

Conclusion: In conclusion TA of paediatric onset seemed to involve the whole of aorta (Type 5). They responded to steroid and other immunosuupressants. One patient who needed trial with multiple immunosuppressants including TNF blocker had long standing disease.


  PC196 Paediatric Rheumatology Top


Comparison of Criteria of the international study group for Behcet's Disease, International classifications criteria for Behcet's disease and paediatric criteria for behcets disease 2015 classification criteria on cohort from a single centre in South India

Vishnu S Chandran, Suma Balan1; Departments of Rheumatology and 1Pediatric Rheumatology, Amrita Institute of Medical Sciences, Kochi, Kerala India

Background: Behcet's disease (BD) is a chronic systemic vasculitis which is well known for its multisystem, relapsing nature and absence of pathogonomic feature. Despite having more than 17 accepted criteria for the disease, a revision was inevitable. The most acceptable criteria among them are the criteria of the international study group for BD (ISG), International classifications criteria for Behcet's disease (ICBD) 2014 and Paediatric criteria for BD 2015 (PEDBD) with variable performance. In adults, ICBD demonstrated an unbiased estimate of sensitivity of 94.8%, considerably higher than that of the ISG criteria (85.0%) and specificity (90.5%) was lower than that of the ISG-criteria (96.0%), yet reasonably high. The new international PEDBD classification also demonstrated a higher sensitivity (91.7%) but lower specificity (42.9%) in comparison to the ISG criteria

Objectives: The objective of the study is to compare the performance PEDBD with criteria of ISG and ICBD.

Methods: 18 patients were diagnosed clinically in the pediatric rheumatology op and were included in the cohort with 2 patients having other autoimmune diseases with symptoms similar to BD. Performance of the ISG, ICBD2014 and PEDBD2015 were evaluated in terms of sensitivity, specificity, positive predictive value(PPV), negative predictive value(NPV), negative likelihood ratio(NLR), positive likelihood ratio(PLR), diagnostic odd ratio(DOR), and Youden's index(YI).

Results: ICBD has the maximum sensitivity (93.33%) when compared to PEDBD (66.67%) and ISG (46.67%).ISG had 100% specificity, with a comparable value in ICBD (66.67%), and lower in PEDBD (33.33%,). PPV was similar in all the three criteria with ISG 100%, ICBD93.33%, PEDBD 83.33%. NPV in ICBD was 66.67%, ISG was 20% and PEDBD 16.67%. PLR of ICBD was 2.8 and PEDBD was 1. The DOR in ICBD was 28, PEDBD was 4.YI was 0.4667 in ISG, 0.6 in ICBD and 0 in PEDBD.

Conclusions: ICBD criteria holds a higher statistical significance in the diagnosis of the behcets disease even in the pediatric age group. PEDBD has a comparable sensitivity and PPV although the NPV and specificity are very low in comparison to the other criteria. It was observed that Gastrointestinal manifestations were florid in PEDBD false negative patients. In short ICBD was found to be superior to ISG and comparable to PEDBD in Sensitivity, Specificity and PPV.


  PC197 Paediatric Rheumatology Top


Juvenile idiopathic arthritis: A single centre study

Romi Shah, Mangat Gurmeet, C Balakrishnan; P.D.Hinduja National Hospital, Mumbai, Maharashtra, India

Background: Due to a paucity of trained paediatric rheumatologists, adult rheumatologists continue to take care of JIA patients. We wanted to study the spectrum JIA seen in our adult rheumatology clinic.

Objectives: Our aim was to study the clinical profile, DMARDS usage and asses disease activity in JIA and SOJIA patients referred to us from a nearby public hospital.

Methods: This is a retrospective study of 27 patients with JIA referred to us from a public hospital over a 7 years period. We analysed the epidemiology, clinical profile, disease activity, drugs used and outcome in these patients.

Results: Out of 27 patients with JIA, 13 had systemic onset JIA (SOJIA). Amongst SOJIA patients male to female ratio was 7:6. Mean age was 7.6 yrs (SD 2.97 years). Mean duration of illness was 13 months (SD months). Common clinical features included: Fever: 13 (100%), arthritis: 10 (77%), rash: 8(61%), lymphadenopathy: 2 (15%), splenomegaly: 2 (15%) and uveitis 1 (7%). Mean follow up was 25 month (SD: +/- 26). Methotrexate was the most commonly used DMARD. 2 patients needed additional HCQ. Steroid was started in 8 patients and tapered out in 3. Eight satisfied Wallace's criteria for clinical remission with medication (1). 2 patients developed joint damage in spite of treatment. Among the other JIA patients (n=14) male: female ratio was 9:5 and mean age was 9.1 years (SD 3.5 years). Ten had oligoarticular and 4 had polyarticular JIA. 2 (14%) patients with oligoarticular involvement had uveitis. 8 patients were negative for rheumatoid factor (2 polyarticular and 6 oligoarticular) and 3 were positive (2 polyarticular and 1 oligoarticular). Mean follow was 16 months (SD +/- 21 months). 3 patients were on 3 DMARDS, 4 on 2 DMARDS, and 7 were on single DMARD. According to cJADAS10 criteria 5, 3 and 6 patients were classified as having respectively high moderate and inactive disease activity. 6 patients achieved clinical remission albeit with medication.

Conclusions: We saw a small number of JIA in our clinic. The spectrum was skewed towards SOJIA due tertiary referral setting. Methotrexate was the commonest DMARD used. About 50% of SOJIA achieved remission on treatment. Of the non SOJIA oligoarticular disease was common. Of all non SOJIA patients half needed more than one DMARD. More than half whilst on therapy achieved remission or moderate disease activity.


  PC198 Paediatric Rheumatology Top


Profile of a pediatric rheumatology practice at a tertiary level centre in Northern India

Abhay Shivpuri, Manjari Agarwal, Sumidha Mittal, Sujata Sawhney; Division of Pediatric Rheumatology, Institute of Child Health, Sir Ganga Ram Hospital, New Delhi, India

Background: We established a pediatric rheumatology service at our institution 16 years ago. We wanted to systematically study the referral patterns to our unit and compare our data to that from western countries. Though data on the patient populations seen in various western pediatric rheumatology centres is available, no data has been published from India. We anticipated that the referrals to our centre would be different to those seen in the west as we have different subtypes of juvenile arthritis, more infectious diseases and less referral for positive auto immune tests and pain amplification.

Objectives: To describe the referral patterns from a pediatric rheumatology practice based at a tertiary level centre in India.

Methods: We studied the referral patterns including the demographic details of the patients, place of residence, referral source and the diagnosis of 1000 consecutive referrals to the out- patient clinic(OPC) from 01st January 2015 till 30th June 2017.This information was captured at the 1st visit of every out- patient referral and the diagnosis was updated as needed.

Results: Demographics: Of 1000 consecutive patients seen in the OPC 448 were girls and 552 boys [Table 1]. Median age at 1st visit- 9.77 years (1week- 31years). Residence: 584 children from NCR (Delhi, Gurgaon, Faridabad and NOIDA). 396 children from other states and 20 international patients. Diagnoses: The commonest referral was for juvenile idiopathic arthritis (JIA) (34%). The next 20% of referrals, equally common at 10% each were for connective tissue diseases (10.1%) and vasculitis (10.2%). Infections comprised 9.4 % of the total patients seen. Approximately 2% each had malignancies, pain amplification syndrome, non specific arthralgias and rickets. Of the children referred for non specific aches and pains, 10.5% were clinically normal. Referral source: Patients were referred by Pediatricians (54.4%), Physicians (22.4%), Orthopedicians (9.1%) and self referred (8.5%)



Conclusion: 40% of children travel from outside the NCR for pediatric rheumatic consultation. 60% of patients are referred by paediatricians. One third of new referrals are JIA. When compared to the western centres we see much less pain amplification, mechanical joint pains and autoinflammatory syndromes.


  PC199 Paediatric Rheumatology Top


Inflammation but not Infection: A case series of chronic recurrent multifocal osteomyelitis in children

Sandeep Surendran, Suma Balan; Department of Rheumatology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Background: Chronic recurrent multifocal osteomyelitis (CRMO) is a little known inflammatory bone disease occurring primarily in children and adolescents. Delays in referral and diagnosis may lead to prolonged courses of antibiotics, unnecessary radiation exposure from scans and surgery including bone biopsies.

Objectives: To report and describe a case series of Chronic recurrent multifocal osteomyelitis (CRMO) in children and to discuss therapeutic options along with their response.

Methods: Children (aged<18 years) diagnosed with CRMO between January 2016 and June 2017, reviewed at Amrita Institute of Medical Sciences were included and all available data collected. All the cases were diagnosed were based on history along with MRI scanning or whole-body-bone scintigraphy.

Results: Mean age was 10.2 years. The average period from the onset of the first CRMO symptoms to the diagnosis was 18 months (3 months to 48 months). The most commonly affected initial joint was the femur. The median number of lesions seen were 3 (range 2-7) and the most common regions were the lower limbs and the pelvis bones. The most common misdiagnosis made were that of an infection related osteomyelitis and malignancy 4 of the 18 children has associated psoriatic features 5 of the 18 children has lytic lesions on X-ray. The mean ESR was 44 mm/hr. The mean CRP was 7.8 mg/dl. 5 of the 18 children had bone biopsies done outside. 8 of 18 patient went into remission with NSAIDS alone. The remaining 10 patients needed a second line agent (palmidronate/methotrexate) to induced remission.

Conclusion: Inflammatory osteomyelitis is a condition which clinicians need to be aware of. MR Imaging and whole-body-bone scintigraphy helps in diagnosis. Early diagnosis can avoid unnecessary scans and biopsy as well as initiate correct treatment at the earliest. Bisphosphonate therapy or Methotrexate can be of benefit to patients with poor response to NSAIDs alone.


  PC200 Paediatric Rheumatology Top


Paediatric rheumatology: Diversity of rheumatological diagnosis in a new medical college hospital

Subramanian Nallasivan, Yuva Vishalini, Jenish Rajma, Jeya Balaji, V Ramasamy; Velammal Medical College Hospital and Research Institute, Madurai, Tamil Nadu, India

Background: In India, Paediatric rheumatic diseases are protean with JIA and SLE topping the list. Rheumatic fever and arthritis have become less common. Paediatric rheumatologists are often faced with the difficulties in disease control, balancing immunosuppression and the risk of infections. Above all, getting the family convinced about the diagnosis and long term compliance is much more an arduous task.

Methods: All children who attended the paediatric services with rheumatology problems from April 2016 over 15 months period were reviewed retrospectively and there clinical profile analysed. On an average 1500 children attend the department and 57 patients had rheumatological symptoms. All of them were reviewed by Consultant Rheumatologist and Paediatricians.

Results: 22 patients had non rheumatological diagnosis Leukaemia ITP Autoimmune haemolytic anemia Rheumatic fever Septic arthritis Infections PSRA 3 4 3 7 2 1 (TB) 2 These children were managed as per current good practice guidelines. 35 patients had definite rheumatological diseases. Mean age was 11.4 yrs. F:M= 19:16 JIA SLE SSc MCTD Reactive arthritis others 11 5 1 2 7 9 Autoimmune rheumatological diseases are equally common in girls and boys. JIA tops the list, followed by SLE and we had one Scleroderma with ILD. Others included Spondyloarthritis, Kikuchi disease, CRMO and fibro muscular dysplasia. Most patients with autoimmune diseases had corticosteroids and DMARDs. Children with JIA are doing well with Methotrexate; one of them is using Etanercept biosimilar with disease free remission. Azathioprine, Cyclophosphamide and mycophenolate are other drugs for maintaining disease control. One girl with lupus nephritis had malignant hypertension and encephalopathy, MDT approach was devised with Paediatricians, rheumatologists, nutritionists. One child with SLE had vasculitis ulcer with sepsis needing hindfoot amputation. All children are under regular follow up and parents are satisfied.

Conclusion: The snap shot of Paediatric rheumatology patients highlights the diverse but common diagnosis in childhood and may increase due to better awareness and diagnostics. We hope that the newly established paediatric rheumatology clinic would help the patients, students and the doctors.


  PC201 Paediatric Rheumatology Top


Systemic onset juvenile idiopathic arthritis with Macrophage activation syndrome at onset - a patient series from India

Jyothi Raghuram, Anand P Rao1,2; Columbia Asia Hospital, Whitefield and Pediatric Rheumatology Clinic, Indira Gandhi Institute of Child Health, 1DNB, EULAR Fellow Gaslini Hospital, Genoa, Italy, 2Manipal Hospital and Paediatric Rheumatology Clinic, Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India

Background: Systemic onset juvenile idiopathic arthritis (SoJIA) is typically associated with fever, evanescent rash, arthritis, lymphadenopathy and hepatosplenomegaly. Laboratory parameters show anemia, leucocytosis, thrombocytosis, raised acute phase reactants (CRP, ESR). Macrophage activation syndrome (MAS) is a potentially life threatening complication of childhood rheumatic disorders with cytopenia, elevated liver enzymes, markedly raised serum ferritin and triglyceride levels with hypofibrinoginemia and paradoxically decreasing ESR. A bone marrow biopsy or aspirate usually shows hemophagocytosis. MAS can occur at onset of SoJIA itself, leading to difficulty in diagnosis and increased risk of mortality if not promptly treated.

Objectives: We present 6 cases of SoJIA with MAS at onset of disease, with the objective to improve awareness towards SoJIA and to stress that MAS can occur at onset of SoJIA leading to further difficulty in diagnosis.

Methods: A retrospective review of case sheets of children who were diagnosed as SoJIA with features of MAS at onset, seen at the Pediatric Rheumatology clinic in a corporate hospital and a government hospital from 2011-2016. 6 cases were analysed with respect to clinical and laboratory features, treatment and outcome.

Results: Of 149 cases of SoJIA, 6 cases were diagnosed with MAS at onset of disease (4.02%) as per the 2016 Classification criteria for MAS. The mean age at presentation was 6 years (1.5 y â€" 10 y). The female:male ratio was 1:5. Mean duration of fever prior to onset of MAS was 25 days. Only 2 cases had transient arthritis. Clinical features and laboratory parameters are discussed. All cases had markedly raised serum ferritin levels and hemophagocytosis was demonstrated in 5/6 cases. All 6 children received initial treatment with steroids. Mean follow-up period was 44 months (10 â€" 120 months). Response to treatment and outcome is discussed.

Conclusions: SoJIA is an important differential diagnosis in children with prolonged fever. Arthritis may not manifest until much later. Upto 10%-20% of cases of SoJIA may present with MAS at onset and these children are generally very sick and are treated as infections, sepsis or PUO. Prompt diagnosis with aggressive management is essential to prevent mortality.


  PC202 Paediatric Rheumatology Top


Clinical Profile of Juvenile Idiopathic Arthritis from a Tertiary Care Centre in North India

Hegde Arun, Vasdev Vivek, M N Arjun, Kishore Kunal, Singh Ramakant; Army Hospital Research and Referral, Delhi, India

Backgound: Juvenile idiopathic arthritis (JIA) is defined as arthritis of unknown etiology, that begins before the age of 16 years and persists for at least 6 weeks, provided other causes are excluded.

Objective: In this study, we describe the clinical profile of patients with JIA attending the rheumatology clinic of a tertiary care hospital of the armed forces.

Methods: Hospital records of all the patients with JIA, attending the rheumatology outpatient department of our tertiary care institution, between Jan 2013 and Jan 2017 were reviewed. The records were analysed with regard to age at onset, clinical features, laboratory parameters and pattern of DMARD/biological agent use.

Results: The study population comprised 56 children (18 girls, 38 boys). The distribution of JIA as per subtype, Age at onset and sex predilection is given in Tables 1 and 2 respectively In children with ERA, knee (70%) and ankle (65%) were most commonly involved joints. 70% were HLA B27(PCR) positive. 4/20 had bilateral sacroilitis on X ray.10 underwent imaging by MRI, amongst which 5 showed active sacroiliitis, out of which 4 were non-radiographic Spondyloarthritis. 16/20 children required DMARDs (Tab Sulfasalazine) while 6 required biological DMARDs (bDMARDS), out of which 3 received Etanercept and 3, Infliximab. Amongst children with polyarticular JIA, the wrist (75%) and knee (75%) were the most commonly involved. Only 1 child was ANA positive, however none had uveitis. 6/16 required bDMARDs, with 4 children requiring Etanercept, while 1 child each required Tocilizumab and Abatacept. Amongst children with SOJIA, the wrist (93.3%) was most commonly involved. Average Haemoglobin, ESR, CRP and Ferritin levels at baseline were 10.7 gm, 53 mm, 31 mg/L and 1968 ng/ml respectively. All children required csDMARDs (Tab Methotrexate), one child required Cyclosporine in addition. 7 children required bDMARDs in form of Inj Tocilizumab. Amongst children with oligoarticular JIA, the wrist (60%) and knee (60%) were most commonly involved. 1 child was ANA positive, however none developed uveitis. Only one patient required bDMARDs (Tocilizumab).

Conclusions: JIA-ERA was the commonest subtype, in comparison to polyarticular subtype, which has been reported from other Indian studies. Also, the incidence of ANA positivity and uveitis is very less in the Indian population. Table 1: Patterns of JIA Type N % Oligoarticular 5 8.93 Polyarticular 16 28.57 ERA 20 35.71 SOJIA 15 26.79 Psoriatic 0 0 Undifferentiated 0 0 Table 2: Distribution of JIA as per age and sex Types Total Females Males Mean age (in years) SD Min. Max. Oligoarticular 5 3 2 9.2 1.3 7 10 Polyarticular 16 11 5 11.5 5.5 3 19 SOJIA 15 2 13 9.8 5.3 2.5 19 ERA 20 2 18 13.0 3.3 9 20


  PC203 Primary Immunodeficiency Top


Pyoderma gangrenosum like lesion: A rare presentation of Leucocyte adhesion deficiency

Avinash Jain, Vikas Gupta, Shobhita Katiyar, Sajal Ajmani, Able Lawrence, Vikas Agarwal, RN Misra, Amita Aggarwal; Department of Clinical Immunology, SGPGIMS, Lucknow, Uttar Pradesh, India

Introduction: Pyoderma gangrenosum is an uncommon neutrophilic dermatosis affecting 3 to 10 per million per year with average age of onset between 40 to 60 years. It manifests as sterile ulcerative skin lesion and more than half the cases are associated with an underlying systemic illness. Leucocyte adhesion deficiency-I (LAD-I) is a rare primary immunodeficiency which presents in infancy and often results in failure to thrive and death in first year of life. Survival into adulthood is uncommon unless patients are affected by the milder form with higher CD18 expression (≥5-30 %).

Methods: We retrospectively reviewed the case records of all patients diagnosed with LAD, at our institute, a tertiary care referral centre in North India. Data on clinical features and laboratory abnormalities were extracted with special emphasis on atypical and delayed presentation in late adolescence in form of pyoderma gangrenosum.

Results: Three out of seven patients diagnosed as severe LAD [CD 18 expression: 0% [Figure 1]c] presented with pyoderma gangrenosum [Figure 1]a, [Figure 1]b. Age of onset and duration of symptoms were 14 ± 2.6 and 7.6 ± 6.4 years respectively. All of them had fever, neutrophilic leucocytosis with mean neutrophil count 32918 per cu. mm (25214 - 46000). None had delayed cord separation. Interestingly, they were treated with steroids and antibiotics and blood transfusion for persistent blood loss through ulcers. Though skin ulcerations are common, predominant clinical presentation as PG is unusual in LAD-I, particularly the severe phenotype. All three had positive family history but still resulted in diagnostic and management delay.



Conclusion: Diagnosis of Leucocyte adhesion deficiency should not be restricted to infants and children with typical clinical features, but should also be suspected in adolescents presenting with fever, neutrophillic leucocytosis and PG like lesions.


  PC204 Infection Related Rheumatic Disease Top


Methotrexate in early Chikungunya Arthritis: A 6 month randomized controlled open label trial

M B Adarsh, Shefali K Sharma, Preksha Dwivedi, Mini P Singh1, Varun Dhir, R K Ratho, Sanjay Jain; Departments of Internal Medicine and 1Virology, PGIMER, Chandigarh, India

Introduction: This study assesses the efficacy of Methotrexate in early Chikungunya arthritis.

Methods: It is a randomized controlled open label assessor blinded trial with a crossover design.60 Patients with Chikungunya fever (WHO Criteria) having persistent arthritis (between 30-90 days of onset) with at least 3 or more tender or swollen joints (28 joint count) were randomized by block randomization to either of NSAID arm or MTX arm. MTX arm was given oral Methotrexate (10 mg/week, increased by 5 mg per visit to a maximum of 25 mg) along with NSAIDs for 15 days initially and then SOS. NSAID arm was given NSAIDs (Naproxyn 1 gm/day or Etoricoxib 120 mg/day) till remission and then SOS. Methyl prednisolone 80 mg intramuscularly was given if there was 5 or more swollen or tender joints. Patients were followed at 1,2,4 and 6 months. After 2 months patients in NSAID arm who haven't achieved remission were shifted to group B. The primary endpoint was remission (no tender or swollen joints) at 6 months. Secondary endpoints were change in CDAI, Indian HAQ,total steroid use,total NSAID use and serious adverse effects. Two in NSAID arm and one in MTX arm lost to follow up. Intention to treat analysis was used. Trial was registered (NCT03058471).

Results: Groups were matched except for the age. TJC (11.4 ± 6.9, 14.4 ± 7.6), SJC (6.6 ± 5.6, 7.8 ± 5.4), CDAI (28.2 ± 14.7, 32.7 ± 14.2) and HAQ (1.1 ± 0.6, 1.2 ± 0.6) were matched between two. Remission was achieved by 28 in the NSAID arm and 26 in MTX arm (p=0.18). Results are in [Table 1]. There was no significant difference in time to achieve remission, steroid need, HAQ, CDAI, TJC and SJC. NSAID use was higher in NSAID arm. Four in NSAID arm were shifted (cross over) to methotrexate arm at 2 months and all of them achieved remission by 6 months. Those who have not achieved remission had higher disease activity at baseline (TJC (p=0.03), SJC (p=0.03) and CDAI (p=0.04)). Duration of fever or duration of the arthritis did not differ significantly in the primary outcome. 17 in methotrexate arm and 12 in NSAID arm had non significant non-articular pain (heel, foot, neck and upper back pain) at 6 months.



Conclusion: Initiation of Methotrexate early in Chikungunya arthritis did not change the outcome. NSAIDs and Steroid help to achieve remission in most patients. Methotrexate may be considered in those who have not achieved remission.


  PC205 Infection Related Rheumatic Disease Top


Sero-prevalence of campylobacter jejuni,  Chlamydia trachomatis Scientific Name Search  and  Chlamydia pneumoniae Scientific Name Search in patients with chronic reactive arthritis

J Euphrasia Latha, Sivakumar V, Therese Mary, Vignesh Mantharam, Kumudha Manoharan, T Shanthi; Institute of Rheumatology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India

Background: Patients with reactive arthritis with preceding infections should be treated with antibiotics to hasten improvement. In patients with probable chronic reactive arthritis, sero-prevalence may be suggestive of an etio-pathogenic agent which may be missed during the routine evaluation.

Objective: 1. To study the seroprevalence of C.jejuni, C.trachomatis, C.pneumoniae in patients with chronic reactive arthritis.

Methods: Patients with chronic reactive arthritis for more than 5 months were included in the study. Serological testing was done using ELISA plates for C.jejuni, C.trachomatis, C.pneumoniae in 30 patients and 10 healthy controls.

Results: During the study period, 30 patients with chronic reactive arthritis were recruited. Mean age was 28.43±9.05 years and mean disease duration was 7.26±1.6 months. Median Tender joint count was 5 (4,7) and swollen joint count was 4 (4,6). Median Major enthesitis index (MEI) was 1 (0,3). 12/30 (40%) patients tested positive for C.jejuni, 10/30 (33%) tested positive for C.pneumoniae and 2/30 (7%) tested positive for C.trachomatis. None of the tests were positive in the healthy controls. Clinical profile of seropositive group did not differ from seronegative group (p=ns), thus inciting more and more pathogenic organisms causing such spondyloarthritis.

Conclusion: There is an increased seroprevalence of antibodies to C.jejuni, C.trachomatis and C.pneumoniae in patients with chronic reactive arthritis. Seropositivity does not influence the severity of reactive arthritis and more studies are required to identify other causative agents for chronic reactive arthritis.


  PC206 Infection Related Rheumatic Disease Top


Seroprevalence of  Brucella More Details in polyarthritis: A study from a tertiary care centre, South India

Therese Mary Dhason, Aravindan Ambigapathy, Mythili Seetharaman, Euphrasialatha Jairaj1, Sivakumar Vengudusamy, Balasubramaniam Veeramangalam, Kumutha Manoharan, Sumathi Narasimman, Suresh Surapillai Radhakrishnan, Srimathi Ponnusamy, Balachandran Palaniappan; Institute of Rheumatology, MMC, 1Institute of Rheumatology, Chennai, Tamil Nadu, India

Background: Polyarthritis is one of the clinical manifestations of  Brucellosis More Details. The diagnostic methods available are isolation of the organism by blood culture, PCR, Brucella agglutination test and ELISA test to detect IgM and IgG antibodies. ELISA is probably the second most common serologic diagnostic method.

Objective: To find out the Seroprevalence of Brucella IgM and IgG antibodies in patients with polyarthritis.

Methods: Duration of study: January 2017 to June 2017 Type of study : Prospective study Inclusion criteria : Patients with polyarthritis Study group Total: n 80 Diseased : n 60 Controls : n 20 Methods Brucella antibodies : IgM and IgG ELISA Statistics : SPSS.

Results: Please see [Table 1] and [Table 2] and [Figure 1].



Conclusion: Brucella antibodies were found to be statistically not significant in patients with polyarthritis. Among the antibody positive diseased, the isotype IgM was detected to be more prevalent than IgG.


  PC207 Infection Related Rheumatic Disease Top


Rheumatoid arthritis like presentation of Hansen's disease

Sukdev Manna; IMS, BHU, Varanasi, Uttar Pradesh, India

Introduction: Leprosy is a chronic multisystem granulomatous infectious disease caused by Mycobacterium leprae. High numbers of people are still affected by this disease in some of the developing countries however, it is rarely seen in non-endemic regions. Cutaneous and neurological manifestations are the common and classical presentations of leprosy. Musculoskeletal involvement is the third most common manifestation but is less frequently reported. Joint involvement (may or may be as a part of Leprae reaction) can present as acute symmetrical polyarthritis or chronic polyarthritis resembling rheumatoid arthritis.

Case Presentation: We report a case of 18-year-old man who developed bilateral symmetrical polyarthritis including small and large joints of hands and feet fulfillng the clinical but not laboratory criteria of RA, later found to have sufficient clinical and laboratory evidence suggesting multibacillary hansens disease without definite evidence of leprae reaction. Patient improved with steroid and anti leprosy treatment.

Discussion and Evaluation: Exact pathogenesis for musculoskeletal manifestations is still not fully known. Patient suffering from leprosy with arthritis may present to rheumatology clinic creating a diagnostic dilema. Our patient presented with symmetrical polyarthritis with constitutional symptoms resembling RA which was actually a manifestation of hansens disease not related to leprae reaction.

Conclusion: Having good knowledge of musculoskeletal manifestation of leprosy, will help narrow differential diagnosis and will prevent unnecessary diagnostic workup.


  PC208 Reproduction and Rheumatology Top


Unmet needs in the usage of contraception in reproductively active female SLE patients a review from a tertiary care center

Vignesh Mantharam, T N Tamilselvam, S Balameena, C Saranya, S Mythili, A Aravindan; Institute of Rheumatology, Madras Medical College and Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India

Background: Usage of appropriate contraceptive measures in patients with Systemic Lupus erythematosus is essential to avoid unplanned pregnancies and related complications. The aim of this study was to study the awareness of safe methods of contraception in reproductively active SLE patients and to find out the unmet needs in utilizing such measures in our center.

Methods: In this descriptive study, after getting informed consent, 100 married female SLE patients between the age of 18-40 years of age, who attended Rheumatology OPD were interviewed regarding menstrual, marital, obstetric, sexual and contraceptive history. Among the patients who did not use contraceptive measures, the unmet needs preventing their proper usage was assessed.

Results: Mean age of the study group was 30.7±5.7 years, with a median disease duration of 4 years (IQR 2, 6). 53/100 patients have not undergone permanent sterilisation at the time of diagnosing SLE, making them susceptible to unplanned pregnancies as shown in [Figure 1]. 50% are aware of the need of contraception; only 35% approach obstetricians for contraception, while only 20% end up with a perfect use. This has led to increased incidence of unprotected sex (40%) and accidental pregnancies ending up in termination due to high disease activity or usage of teratogenic drugs (22%). Lack of awareness (49), lack of physician’s counselling (44), peer/family pressure (15), fear of medical condition contraindicating contraception (11), husband not willing for male sterilisation (11), choosing abstinence in fear of contraception (12), fear of surgery or side effects of surgery (4) were the common responses (n=146 responses) for not utilising contraception.



Conclusion: Almost half of the studied population was naturally protected because of permanent sterilisation done prior to the onset of SLE. Lack of awareness, inappropriate counselling by the treating physicians, peer or family pressure were the common unmet needs in practising safe contraception. Male condom and IUCD were the only temporary measures followed. Awareness about female condom, injectable and oral contraceptives and male sterilisation was non-existent. Despite high disease activity and use of teratogenic drugs, there was increased rate of unprotected sex. The unmet needs assessed in this study should be explored further to avoid accidental pregnancies and complications related to medical termination in SLE.


  PC209 Imaging in Rheumatology Top


Articular cartilage of knee and first MTP joint are the Preferred Sites of Urate Crystal Deposition in Asymptomatic Hyperuricemic individuals

Danveer Bhadu, Siddharth K Das, Archana Wakhlu, Urmila Dhakad, Meha Sharma; Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, India

Objective: To determine the sites of urate crystal deposition in patients of asymptomatic hyperuricemia (AH) by ultrasound.

Methods: Twenty four AH (serum uric acid (SUA) ≥7mg/dl) and fifty controls (SUA <7mg/dl) with age more than 18 years were included in this study. Double contour sign (DCS) was looked for at three articular cartilage sites (first metatarsal (1st MTP), tibiotalar and femoral condyle) whereas hyperechoic aggregates (HAGs) were looked for at one joint site (radiocarpal joint) and two tendon sites (patellar tendon and triceps tendon).

Results: Eight out of 24 AH patients had ultrasound evidence of urate crystal deposition in 1st MTP joint area followed by knee joint area which was detected in 6 patients. The detection rate of ultrasound abnormalities in AH patients was 45.8% with two joint area (knee and 1st MTP) and 50% with global assessment. Amongst controls, 16% were found to have these abnormal ultrasound findings by both two joint area and global exams.

Conclusion: The highest predilection of urate crystal deposition in AH patients is the articular cartilage of Knee and 1st MTP joint.


  PC210 Imaging in Rheumatology Top


Hydroxychloroquine retinal toxicity screening: A cross sectional study

Nikunjkumar V Dadhaniya, Isha Sood, Sundeep Upadhyaya, Rohini Handa, Sirinder J Gupta; Department of Rheumatology, Indraprastha Apollo hospitals, New Delhi, India

Background: Hydroxychloroquine (HCQ) is a commonly used drug in various autoimmune rheumatic diseases. Though HCQ is not associated with serious systemic side effects, retinal toxicity is a potential concern. When retinal toxicity is detected by conventional methods like fundus examination and visual fields, irreversible damage to the retina may already have occurred.1 Newer methods for retinal toxicity screening such as- Spectral domain optical coherence tomography (SD-OCT), fundus auto fluorescence (FAF) and multifocal electroretinography (MFERG) could lead to earlier detection of toxicity.

Objective: This study was undertaken to screen for HCQ toxicity in patients with autoimmune rheumatic diseases, who have been on long term HCQ therapy and to evaluate whether the newer technique, SD-OCT enhances the early detection rate.

Methods: Patients with autoimmune rheumatic disease who had been on HCQ for more than 5 years and/or had taken a cumulative dose of more than 500 grams, but had no visual symptoms were included in the study. All patients underwent an ophthalmic examination including vision, colour vision, Amsler’s grid, fundus examination, visual field testing (with 10-2 and 30-2 protocols) and SD-OCT.

Results: Retinal toxicity was noted in 6 out of 60 (10%) patients. Findings suggestive of toxicity were noted on fundus examination in 4 patients, visual fields were abnormal in 2 patients, whereas SD-OCT was abnormal in all six patients. Mean duration of HCQ use, cumulative dose of HCQ and actual body weight based daily dose of HCQ were 9.6 years, 1092 grams and 4.82 gram/kg respectively, in patients without toxicity as compared to 12.8 years, 1514 grams and 5.13 grams/kg in patients with HCQ retinal toxicity.

Conclusion: HCQ retinal toxicity is more common than earlier recognized. Inclusion of more sensitive screening tests like SD-OCT could lead to earlier detection of retinal toxicity and might help in preventing permanent changes from developing. Further confirmation of these findings, by screening larger number of patients, would be helpful.

Reference

  1. Marmor MF, Kellner U, Lai TY, Melles RB, Mieler WF; American Academy of Ophthalmology. Recommendations on screening for chloroquine and hydroxychloroquine retinopathy (2016 revision). Ophthalmology 2016;123:1386-94.



  PC211 Imaging in Rheumatology Top


Clinical utility of fluorine 18 fluorodeoxyglucose positron emission computed tomography in rheumatology

K Sirisha, S Aparna, Ranadheer Gupta Manthri1; Department of Rheumatology and1Department of Nuclear Medicine, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India

Background: Several studies with Fluorine 18 Fluorodeoxyglucose positron emission tomography with computerised tomography [(18) FDG PET/CT] have indicated that FDG uptake in affected tissues reflects the disease activity. In addition, usage of PET/CT for early detection, extent and monitoring of the treatment response has been reported.

Objective: To investigate the diagnostic performance of PET CT in establishing the diagnosis and its potential role in evaluating the extent and activity of disease.

Methods: In this observational study of 8,461 patients seeking rheumatology services from March 2016 to June 2017, PET CT was carried out in 25 patients. Clinical findings and scan findings were noted. The sensitivity, specificity, positive and negative predictive value (PPV and NPV) was calculated. A final diagnosis was made by physician assessment, other investigations and tissue biopsy where indicated.

Results: Twenty five PET/CT requests were identified (21 females, mean age - 41.7±16 years). A final diagnosis was established in 52% of patients. The indications included: establishing diagnosis [n=17 (68%)], disease activity/extent [n=5 (20%)], and both [n=3 (12%)]. Two sub-groups were analysed; first presentation with no diagnosis [n=21 (84%)] and prior established rheumatological diagnosis [n=4 (16%)]. First presentation: PET/CT identified abnormalities in 16 (76%) cases. It contributed to diagnosis in 9 (43%) (sarcoidosis-1, Koch’s-2, rheumatoid arthritis (ra)-3, systemic lupus erythematosus (SLE) with myositis-1, Takayasu arteritis-1, mitochondrial myopathy-1). Seven (33%) were identified to have abnormalities with no contribution to a final diagnosis 6-fibromyalgia, 1- Behcet’s disease. The sensitivity, specificity, PPV and NPV this setting were 100.00%, 41.67 %, 65.00%, 100.00 % respectively. Established rheumatological diagnoses included SLE-1, RA-2, Spondyloarthropathy (SPA)-1. PET /CT identified abnormalities in all the 4 (Koch’s - SLE and SPA, active disease - 2 RA patients). PET/CT was done as a part of pyrexia of unknown origin (PUO) work up in 4 patients (one SLE with myositis and one Takayasu arteritis at first presentation, one Koch’s in established SLE and one active disease in established RA).

Conclusion: PET/CT has high diagnostic sensitivity and reasonable moderate specificity in rheumatology practice with respect to establishing the diagnosis as well as to detect the extent and activity of disease.


  PC212 Miscellaneous Rheumatic and Inflammatory Disease Top


Evaluation of Hemophilic Arthropathy using Ultrasonography and its comparison to MRI

Shounak Ghosh1, Debanjali Sinha1, Alakendu Ghosh1, Krishnendu Roy2; 1Deratment of Rheumatology, IPGMER, Kolkatta, 2Department of Medicine, Medical College, Kolkata, West Bengal, India

Background: India has the world's second highest burden of Hemophilia patients. MRI study of Hemophilic joints is the existing gold standard for assessment of joint pathology. However, Ultrasonography is a novel and much cheaper alternative to MRI, and our study aims to assess joint pathology in Hemophilic arthropathy patients and correlate the findings with MRI.

Objectives: 1. To assess knee and elbow joint damage using MRI 2. To assess knee and elbow joint damage using Power Doppler USG 3. To determine the strength of correlation between the findings.

Methods: We studied 58 patients of Hemophilia using MRI of most affected joint (either knee or elbow) and Ultrasonography of the same joint. We checked the following parameters: Synovial thickness, cartilage erosion, synovial vascularity, intra-articular collection, subchondral cyst, hemosiderin deposits, and joint space narrowing. We excluded patients less than 5 and more than 30 years of age. We stratified the strength of agreement using statistical analysis on the aforementioned 7 parameters between MRI results and Ultrasonography results. Categorical variables were compared using Cohen's weighted Kappa Coefficient (KC), and numerical measurements were compared using Intraclass Correlation Coefficient (ICC).

Results: Of the 58 patients, 14 patients had moderate and 44 had severe Hemophilia. There was excellent agreement between USG and MRI findings on synovial thickness (ICC=0.980), medial cartilage thickness (ICC=0.996), central cartilage thickness (ICC=0.995), and lateral cartilage thickness (ICC=0.995). There was very strong agreement between the USG and MRI findings on synovial vascularity (Kappa coefficient = 0.839), intra-articular collection (Kappa coefficient = 0.802) and joint space narrowing (Kappa coefficient = 0.931). However, there was poor correlation between the MRI and USG findings on hemosiderin deposits (KC = 0.176) and subchondral bone cysts (KC = 0.408).

Conclusion: Power Doppler Ultrasonography holds promise as a cheaper imaging tool to diagnose and follow up patients with Hemophilic arthropathy in most areas of measurement as compared to MRI, but fails to yet meet the standards of MRI in areas like hemosiderin deposition and subchondral cyst detection, and hence probably cannot yet absolutely be held as an equivalent to MRI scanning in Hemophilia.


  PC213 Miscellaneous Rheumatic and Inflammatory Disease Arthritis in sarcoidosis Top


A multi-centre study

V Agrawal1, A Aggarwal1, P Aggarwal2, A C Chowdhury3, P Ghosh3, A Jain1, A Lawrence1, D P Misra1, R Misra1, M S Mohapatra1, Nath A1, V S Negi4, S Pandya5, V V Reddy6, S Prasad7, V Shobha8, Y P Singh9, S R Tripathy10, A Wakhlu10; 1SGPGIMS, 10KGMU, Lucknow, Uttar Pradesh, 2Punjab Rheumatology Clinic, Ludhiana, Punjab, 3IPGMER, Kolkata, West Bengal, 4JIPMER, Pondicherry, 5VIMS, Ahmedabad, Gujarat, 6Vizag Rheumatology and Immunology Centre, Visakhapatnam, Andhra Pradesh, 7Apollo BGS Hospital, Mysore, 8St John's Medical College, 9Manipal Hospital, Bengaluru, Karnataka, India

For the Arthritis in Sarcoidosis Group (ASG)

Background: 10-15% of sarcoid patients are associated with arthropathy. Chronic arthritis is less common around 1%. Data on articular manifestations of the disease from India is sparse.

Objective: To study the clinical manifestations of sarcoid arthritis patients from India.

Methods: Case records of patients presenting to ten rheumatology centres from 2005 to 2017 with sarcoidosis were retrospectively reviewed. Joint involvement was assessed clinically, classified as acute or chronic depending on duration of symptoms lesser or greater than 6 months respectively.

Results: A total of 123 patients with sarcoid arthritis were reviewed. Table 1 sums up the mode of presentation 58 patients were classified as Lofgren syndrome. Pattern of joint involvement revealed ankle as most commonly affected in both the groups. Wrist, MCP, MTP and PIP involvement was significantly more common in chronic sarcoid [Table 1]. Peripheral lymphadenopathy, plaques and uveitis were more frequent (p<0.05) in chronic sarcoid arthritis (42.8%, 21.4%, 21.4% respectively) compared to those with acute sarcoid arthritis (15.7 %, 5.2 %, 6.3% respectively). 45 of 61 patients with acute arthritis with follow up details had achieved complete remission.14/28 chronic sarcoid arthritis patients with a median follow up of 2 years had achieved complete remission with 19, 15 and 8 patients on steroids, methotrexate and hydroxychloroquine respectively. One patient with concomitant interstitial lung disease had died due to lung infection.



Conclusion: Acute oligoarthritis was the commonest presentation with ankle most commonly affected joint. Wrist, PIP, MCP, MTP involvement were more common in chronic sarcoidosis. One of the limitation was retrospective analysis.


  PC214 Miscellaneous Rheumatic and Inflammatory Disease Top


Efficacy of rituximab in resistant palindromic rheumatism: First report in literature

Sreelakshmi Sreenath, Mithun Rathen1, Somy Cherian, Glindow Antony, Padmanabha Shenoy; Centre for Arthritis and Rheumatism, 1Department of Medicine, AIMS, Cochin, Kerala, India

Background: Palindromic rheumatism (PR) although often considered as a benign disease can be severe and resistant to DMARDs in some patients. In these patients it can result in almost daily attacks, migrating from joint to joint resulting in poor quality of life. Rituximab has been proven to be effective in treatment of seropositive RA.

Objectives: To determine the efficacy and safety of Rituximab in patients with seropositive PR who had an inadequate response toCsDMARDs.

Methods: PR was diagnosed based on criteria proposed by Hannonen P et al. Seropositive (ACPA±RF positivity) PR patients who had active disease despite being treated with two Cs DMARDs for >3 months, were treated with Rituximab. Active disease was defined as > 4 attacks per month requiring intake of NSAIDS. All the patients were started on 500mg of rituximab after baseline work up. If complete control of palindromic attacks was not achieved another 500 mg infusion was given after 2 weeks.

Results: Thirty three patients with a mean age of 48.15±13.51 years and mean disease duration of6±3.25 years were included. Despite the maximum tolerable dose of combination DMARDs they had mean attack rate of 5.30 ± 2.38 attacks per month and treated with 500 mg of Rituximab. At one month follow up all patients achieved complete control of disease. During a mean follow up of 14.17±8.62 months 12 patients relapsed after a mean duration of 10.33±5.75 months. They were retreated with rituximab and all responded. None of the patients evolved into RA during the study period. No serious adverse events were observed.

Conclusions: In seropositive PR, Rituximab not only controls the attacks but also prevents progression to RA. To best of our knowledge this is the first report regarding efficacy of rituximab in PR. Although itneeds to be proved in a larger blinded RCT this early data indicates that Rituximab may be a therapeutic option to preventdevelopment of RA in seropositive patients.


  PC215 Miscellaneous Rheumatic and Inflammatory Disease Top


Socio-economic variables associated with rheumatological emergencies at a tertiary care centre

K Chanakya, P Deepika, K Sunitha, K Mahesh Babu, M Sudha Rani, D Phani Kumar, I R Varaprasad, L Rajasekhar; Department of Clinical Immunology and Rheumatology, ARSR Trust, Government of Telangana, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background: Information on outcome and economics of rheumatological emergencies (RE) is scarce.

Objectives: To assess prevalence of, survival in and hospital expenditure of RE.

Methods: Clinical and billing details of patients with rheumatic disease admitted in emergency or intensive care (EIC) between January 2011 to December 2015 were collected. Billed hospital expenditure (BHE) was taken as percentage of average Indian per capita income (PCI) (1.19lakhs, as per 2016 census). Bills of self-paying patients excluded pharmacy bills. Descriptive statistics were used. Inter-group comparisons with student t- test were done and p< 0.05 was considered significant.

Results: Of 3685 admissions, 605 emergencies were identified, of which 484 (80%) occurred in females. The third decade was the most frequent age at presentation 172 (28.5%). The mean (SD) distance to tertiary care centre was 145 ± 130 kms. Conditions requiring EIC are listed in [Table 1] and disease frequency in patients requiring EIC in ]Table 2]. Infections occurred in 44.1% (267) and multi-organ dysfunction (MODS) in 15.5% (94) patients. 62.7% (59) of those with MODS had SLE. Seventy-seven deaths occurred, 55% (43) and 10.3% (8) in SLE and scleroderma respectively. Median BHE in rupees in non survivors was 48500 (2012-2,60,000) which is 40% of PCI, MODS 41137 (2068-7,00,000) 34% of the PCI, was higher compared to survivors 32,594 (699-8,00,000) 27% of the PCI, though not statistically significant. Patients with governmental support (n=333) had significantly higher BHE than self-paying patients (p<0.0001). Annual mortality rates were static. Duration of hospital stay and cost of hospitalization were similar in survivors and nonsurvivors.



Conclusions: One sixth of rheumatology admissions required emergency/intensive care. In hospital mortality rate was 12.7%. SLE accounts for half of these admissions, MODS and mortality. Infections are frequent. Unit cost of emergency hospitalization is nearly one-third of the per capita income.


  PC216 Miscellaneous Rheumatic and Inflammatory Disease Top


Biologic use in rheumatology: An audit

V Sharma, T Parikh, R Sharma, A Digole, N Jain, S Chikani, P Srivastava, M Bavaliya, N Patel, A Parmar, S Pandya; Vedanta Institute of Medical Sciences and Sheth VS Municipal Hospital, Ahmedabad, Gujarat, India

Background: Efficacy and safety of biologics including biosimilars in community practice can be best judged by maintenance of usage data or registries.

Objective: To make an audit of biologic use by rheumatologists from all over the state of Gujarat.

Methods: Ours was a cross sectional retrospective analysis. Practicing rheumatologists were asked to fill proformas which included all details of biologic use including demographic data of patients, indications for use, which biologic used, side/adverse effects, screening for diseases/viruses, vaccination status etc. Study duration was of two years (July 2015 to July 2017). Informed consent was taken. Descriptive statistics was used to analyse this data.

Results: A total of 12 rheumatologists contributed to the data [Table 1]. Of 119 patients where data on source of financing was available, 95 (79.8%) paid from their pockets. Of data available from 317 patients of which biologic was used, Rituximab was used in 146 (46.05%), Etanercept in 78 (24.6%), Infliximab in 42 (14.2%), Adalimumab in 30 (10.7%) and Tocilizumab, Golimumab and Abatacept in 10, 2 and 1 patient respectively. Of these, 249 (78.5%) were biosimilars. Mortality was documented in 7 patients, 4 of whom died of infection and 2 of disease flare. All these were given rituximab.



Conclusion: Rituximab was the most used biologic. Majority rheumatologists used biosimilars (78.5%) as most patients had to self finance themselves (79.8%). Rheumatoid arthritis and Spondyloarthritis (Ankylosing Spondylitis) were the most common indications. While 40% were screened for TB, only 13.2 % were vaccinated. Most biologics were safe.


  PC217 Miscellaneous Rheumatic and Inflammatory Disease Top


Clinical profile and treatment outcomes of Adult Onset Still's Disease at a tertiary care hospital in India

Preksha Dwivedi, Sanjay Jain, Aman Sharma, Varun Dhir, M B Adarsh, Shanker Naidu, Aadhaar Dhooria; Department of Internal Medicine, PGIMER, Chandigarh, India

Background: Adult Onset Still's disease (AOSD) is a rare disorder characterised by systemic inflammation. Diagnosis is challenging due to its nonspecific clinical features and lack of specific diagnostic test.

Objective: Since there are very few published series on AOSD, the present study was planned to report clinical, laboratory characteristics and treatment outcomes in these patients.

Methods: Medical records of 34 patients admitted or attended rheumatology outpatient department with a final diagnosis of AOSD according to Yamaguchi criteria were retrospectively evaluated with emphasis on clinical manifestations, laboratory findings, treatment received and outcomes.

Results: The classic triad of fever, rash and arthritis is present in 38.23% patients. Mean delay in diagnosis of Still's disease from first symptom onset was 12.24 months. Biphasic illness was seen in 11.7% of patients, with arthritis phase preceded by systemic illness. Fever was the most common (91.17%) initial manifestation, the mean temp recorded was 39.1oC.Rash at fever onset was present in 52.94%. Lymphadenopathy was present in 55.55%. Arthritis was present in 85.29% patients, with polyarticular involvement in 58.82%, oligoarticular in 17.64% and monoarticular in 5.8%.The most common joint affected was wrist (61.7%)followed by knee (55.8%). Neutrophilic leucocytosis and hyperferritinemia was observed in 94.11% and 92.85% respectively. Mean ferritin values was 6847 ngm/ml. Liver abnormalities were present in 91.17% patients. Serositis was not remarkable in our cohort. 52.94% patients received methotrexate. 11.1% of patients were unresponsive to methotrexate. Steroids and methotrexate was started upfront in 47.05% of patients, thus steroids could be tapered rapidly and relapses were also less frequent on tapering steroids. The mean duration of follow-up was 9 months. The overall outcome of disease was good with only 11.7% patient developing complications such as deforming arthritis, amyloidosis,macrophage activation syndrome.

Conclusion: The diagnosis of Adult onset Still's disease is enigmatic, but the overall course of disease is relatively benign provided the disease is diagnosed on time and treated appropriately.


  PC218 Miscellaneous Rheumatic and Inflammatory Disease Top


Jasvinder Singh; University of Alabama at Birmingham, Birmingham, AL, USA

Objective: To examine the impact of gout on sleep.

Methods: Nine nominal groups were conducted, oversampling for African-Americans and women with gout. Patients discussed and rank-ordered their concerns.

Results: Nine nominal groups with 46 gout patients were conducted with mean age, 61 years (standard deviation [sd], 10.6) and gout duration, 14.9 years (sd, 12). Of these, 63% were men, 46% African-American, 52% married, 46% retired; 63% were using allopurinol and >50% natural supplements currently. The most frequently cited highly-ranked concerns among the nine nominal groups were: (1) severe and complete sleep interruption by severe gout flare pain (9 groups); (2) inability to get into a comfortable position during sleep (6 groups); (3) anxiety and depression associated with severe gout pain (7 groups); (4) sleep interruption by moderate chronic joint pain (3 groups); (6) inability to get REM Sleep (1 group); (7) effect on daily functioning (2 groups); (8) frequent trips to the bathroom interfering with sleep (2 groups); (9) medication side effects (4 groups); (10) frequent trips to the emergency room (1 group); and (11) joint swelling interfering with sleep (2 groups); (12) interference with sleep apnea management (2 groups): (13) affect on other medical conditions (4 groups); and (14) cumulative effect on sleep (1 group). Compared to men, women ranked the concern of chronic gout pain (outside of gout flares) interfering with sleep high.

Conclusions: Gout has significant impact on sleep quantity, quality and architecture. Some differences exist in the impact of gout on sleep by gender.


  PC219 Miscellaneous Rheumatic and Inflammatory Disease Top


Emergencies in rheumatology: A retrospective study

Romi Shah, Rohini Samant; P.D.Hinduja National Hospital, Mumbai, Maharashtra, India

Background: Although compared to other medical branches rheumatology is considered a cold branch, life threatening complications do occur and it is important to identify and properly manage them.

Objectives: To study the outcome of various rheumatological emergencies and common diagnostic dilemmas in ICU settings.

Methods: We have retrospectively studied the patients who were admitted in our ICU over 4 years from 2012 to 2016.

Results: Out of 29 PATIENTS, 13 (44%) SLE, 4 (13%) AAV, 3(10%) MCTD, 2 (6%) APLA, 1 DM, 2 RA, 1 IGG4, 4 (10%) SSc 1 PAN (3 %) was there. Out of these 9 patients expired (3 LUPUS, 2 AAV, 2 RA 1 PAN, 1 SSc). We have divided the patients in categories of aggressive disease (n=15), Infection (n=4), Known complication (n =3), Unusual presentation (n=3), Unrelated complication (n=3), New onset (n=1). 6 patients among 15 aggressive disease category expired, whereas 2 and 1 patients respectively from infection and unusual presentation category expired. Rituximab was used in total 13 patients (10 lupus, 1 MCTD with PAH, 1 APLA with EVAN's syndrome, 1 PM /DM, 1 IgG4RD) out which 2 patients developed definite infections (TB and nocardiosis), 2 patients died with no definite diagnosis of infection or aggressive disease. Other patients improved exceptionally well.

Conclusions: Lessons learnt were: 1. Elderly and immunosuppression are important risk factors leading to increased chances of infection and there so mortality, so early empirical initiation of antibiotics in this group is recommended. 2. Pulmonary embolism is a great masquerader and high suspicion is the only key to diagnose. 3. Biologics carry high risk of infections. Unusual infections can be of trouble if not suspected. Communication with microbiology regarding high suspicion of infection and chances of unusual organism isolation should be made. 4. In aggressive lupus patients repeated complement levels with appropriate clinical background is useful. 5. Awareness about non rheumatologic comorbidities in rheumatological patients is necessary. 6. PLEX is a useful modality in emergency settings.


  PC220 Miscellaneous Rheumatic and Inflammatory Disease Top


A study of clinical profile of patients with connective tissue disorders related interstitial lung disease at a tertiary care hospital in North India

Kishore Kunal, Vasdev Vivek, Hegde Arun, M N Arjun; Department of Rheumatology and Clinical Immunology, Army Hospital R and R, New Delhi, India

Background: Interstitial lung diseases (ILD) are frequently seen in patients of connective tissue disorders (CTDs). The true incidence and prevalence of ILD occurring in association with CTD are unknown. Prevalence estimates for CTD-ILD range from 25–90% for Systemic sclerosis (SSc), from 5–70% for Polymyositis/Dermatomyositis(PM/DM), and from 8–38% for primary Sjögren's syndrome (pSS). Many patients of Undifferentiated CTDs (UCTD) also have associated ILD.

Objective: To study the clinical profile of patients with CTD associated ILD at a tertiary care centre.

Methods: This is a cross sectional, observational study where 70 patients with CTD associated ILD were selected and their demographic, clinical, autoantibody profile, lung function tests, radiological pattern and nailfold capillaroscopic (NFC) parameters were recorded and assessed. These patients were further subdivided into 5 groups namely SSc, MCTD, DM, pSS and UCTD.

Results: The mean age of all CTD patients was 43 years with female predominance (n= 71). Overall 49 patients had UIP pattern on HRCT chest and remaining 22 had NSIP pattern [Table 1]. The mean duration of disease was 52 months and the major autoantibodies associated were Scl-70, U1RNP, Jo-1 and Ro-60/52 for Systemic sclerosis, MCTD, DM and pSS respectively. There was no definite association of UCTD-ILD with any autoantibodies.



Conclusion: This is a small observational study in patients of CTD related ILD in India. It has also incorporated NFC findings along with the demographic, clinical and radiological details.


  PC221 Miscellaneous Rheumatic and Inflammatory Disease Top


Looks may be deceptive in DRESS

Rutviz Mistry, Avinash Jain, Abhro, Durga P Misra, Narendra Krishnani, Vikas Agarwal; SGPGI, Lucknow, Uttar Pradesh, India

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 8 cases of DRESS who presented to Rheumatology Clinic as suspected connective tissue disease or sepsis in Northern India with an aim to report the etiology, characteristics, treatment and prognosis. All the eight patients fulfilled clinical criteria for diagnosis (two probable, six definite). The age of patients ranged from 9-53 years with majority in their second decade. Majority of the patients were referred to us when their counts were rising in the setting of fever and skin rashes with a suspicion of sepsis. The clinical and laboratory features of these patients are as follows [Table 1]: All the patients were treated with oral steroids showing signs of clinical improvement within 4-5 days. Rashes and leucocyte count were first to respond. Hepatitis responded within a week. They were gradually tapered off from steroids over next 3-4 months except for one patient who was lost to follow up. Skin eruptions, arthritis, multi-organ failure of DRESS closely mimics rheumatological disorders or sepsis. As early diagnosis is imperative for successful outcome, low threshold of suspicion is necessary for this condition.




  PC222 Miscellaneous Rheumatic and Inflammatory Disease Top


Allopurinol reduces the risk of myocardial infarction in the elderly: A Study of Medicare Claims

Jasvinder Singh, Shaohua Yu; University of Alabama at Birmingham, Birmingham, AL, USA

Background: Previous observational studies that examined the association of allopurinol with myocardial infarction (MI) provided contradictory results. One study showed that allopurinol reduced the risk and the other study showed an increased risk with allopurinol. Therefore, our objective was to assess whether allopurinol use is associated with a reduction in the risk of MI in the elderly.

Methods: We used the 2006-2012 5% random sample of Medicare beneficiaries to study the association of new allopurinol initiation and the risk of incident MI, in a cohort study. Multivariable-adjusted Cox regression models adjusted for age, gender, race and Charlson index, in addition to various cardioprotective medications (beta-blockers, ACE inhibitors, diuretics, statins). We calculated hazards ratio (HR) with 95% confidence intervals (CI). Sensitivity analyses adjusted for coronary artery disease (CAD) risk factors including hypertension, hyperlipidemia, diabetes, and smoking.

Results: 1,544 of the 29,298 episodes of incident allopurinol use were associated with incident MI (5.3% episodes). Allopurinol use was associated with reduced hazards of MI, with a HR of 0.85 (95% CI, 0.77 to 0.95). Compared to no allopurinol use, longer durations of allopurinol use were associated with a lower HR of MI: 1-180 days, 0.98 (95% CI, 0.84 to 1.14); 181 days to 2 years, 0.83 (95% CI, 0.72 to 0.95); and >2 years, 0.70 (95% CI, 0.56 to 0.88). Other factors associated with a higher hazard of MI were: age 75-<85 and ≥85, male gender, higher Charlson index score and the use of an ACE-inhibitor. Adjustment for CAD risk factors confirmed these findings.

Conclusion: Incident allopurinol use was associated with a reduction in the risk of incident MI in the elderly. Longer durations of allopurinol use reduced the risk of incident MI incrementally. Future studies should assess the underlying mechanisms for MI prevention and assess the risk-benefit ratio for allopurinol use.


  PC223 Miscellaneous Rheumatic and Inflammatory Disease Top


Pilot study: FRAX score (without bone mineral density) to predict fracture risk in patients with Rheumatic diseases.

Nibha Jain, Sapan Pandya, Puja Srivastava; Department of Rheumatology, VS Hospital, Ahmedabad, Gujarat, India

Background: Osteoporosis is a major health concern for postmenopausal women and older men, as well as in younger patients with inflammatory disorders like Rheumatoid Arthritis (RA). Poor bone health and increased fracture risk is an important contributor of morbidity and mortality in these patients, and its assessment via bone mineral density (BMD) is either not accessible or not affordable at many centres.

Objective: Hence, we tried to estimate the fracture risk using "FRAX score without BMD", in patients with rheumatic diseases.

Methods: All patients with > age 40 years, visiting Rheumatology OPD from Jan-june 2017, were included after informed consent. FRAX score was calculated using the online calculator without using BMD values.

Results: Total 106 patients were included, 55 had RA with moderate disease activity (Mean DAS28 ESR=3.8+1.3). Estimation of fracture risk using FRAX without BMD was significantly low, as compared, however, FRAX alone also indicates the higher fracture risk in inflammatory disorders vs non-inflammatory disorder [Table 1]. Further, in patients with inflammatory disorders, males and females have comparative risk of fracture.



Conclusion: Although FRAX (without BMD) can be used to predict fracture risk, it grossly underestimates the risk as compared to FRAX with BMD values.


  PC224 Miscellaneous Rheumatic and Inflammatory Disease Top


Clinicopathological profile of sarcoidosis

C Shyamala, B N Shivaprasad, Apollo Bgs Hospitals, Mysore, Karnataka, India

Background: Sarcoidosis is a multisystem disorder of unknown etiology affecting middle age group. Most commonly affected organs are lymph nodes, lungs, skin and eyes. It is characterized by noncaseating granulomatous inflammation of sites of disease. Diagnosis is based on clinical, radiological and histological examination.

Objective: The purpose of our study is to present clinicopathological profile of established sarcoidosis cases which is grossly underdiagnosed in our country.

Material and Methods: 20 patients diagnosed to have sarcoidosis were included in this study. A detailed clinical evaluation, laboratory investigations were carried out and treated accordingly.

Results: In this study, we present 20 cases of sarcoidosis with varied presentations. 16 were females and 4 male patients. Most commonly affected organs were lymph nodes 12, erythema nodosum 11, ILD 4, eyes 3, parotid 2, lacrimal 1, generalised lymphadenopathy in 1 case, cranial nerves 1, liver 1, heart 1. Loefgren's syndrome was noted in 2 cases. Elevated serum ACE levels were noted in 6 cases. Hypercalcaemia was seen in 1 case. Isolated cases of hepatic, skin, lacrimal, cardiac, glandular sarcoidosis, post whipple’s sarcoidosis were present in the study. There was remission with glucocorticoids in most of the cases and four cases were treated with glucocorticoids and azathioprine.

Conclusion: Sarcoidosis is a multisystem disorder of unknown cause. In our study we noted it commonly affects young and middle-aged adults. Lungs were frequently involved followed by lymph nodes, skin and eyes. We had isolated cases of hepatic, cardiac, lacrimal and skin sarcoidosis which is typically uncommon. Sarcoidosis is often misdiagnosed as tuberculosis in India. A high index of clinical suspicion and focussed attempts to secure histopathological confirmation of the diagnosis early in the illness are required to ascertain the diagnosis.


  PC225 Miscellaneous Rheumatic and Inflammatory Disease Top


Chronic recurrent multifocal osteomyelitis: A report of three cases from a single center

Vikas Gupta, Avinash Jain, Amita Aggarwal; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Chronic recurrent multifocal osteomyelitis (CRMO), also known as chronic nonbacterial osteomyelitis (CNO), is a little-known autoinflammatory bone disorder primarily affecting children and adolescents, and sometimes young adults. Diagnosis is often difficult and delayed due to its varied and nonspecific initial symptoms and clinical course.

Objectives: To study the spectrum of CRMO at a tertiary care centre in North India.

Methods: We retrospectively reviewed the case records of patients diagnosed as CRMO at our centre in last 2 years.

Results: Three patients (all males) were diagnosed as CRMO, according to the diagnostic criteria by Jansson et al. The age at symptom onset ranged from 14 to 23 years and the age at diagnosis ranged from 16 to 24 years. Diagnostic delay ranged from 1.5 years to 7 years. One patient underwent two surgeries including a bone biopsy before the diagnosis was made. All three patients presented with recurrent bony pain and swelling. Two patients had multifocal osteomyelitis involving multiple sites (tibia, femur, humerus, radius and ulna) and one patient had unifocal osteomyelitis involving a single site (right femur). There were no other systemic complaints. No patient had clinically apparent inflammatory bowel disease, any associated features of spondyloarthropathy, or positive family history. Bone biopsy done in one patient, before referral to our centre, showed inflammatory granulation tissue, and the cultures were sterile. Bone scan was done in two patients and revealed asymptomatic sites in both of them. All patients had initially received at least one course of antibiotics. One patient achieved remission on NSAIDs alone, however, another patient required Methotrexate to control disease activity.

Conclusion: Awareness about CRMO among the clinicians can help in early diagnosis of this condition and prevent unnecessary investigations, and surgical procedures. In addition to children and adolescents with typical manifestations, CRMO should also be suspected in young adults, in patients presenting with unifocal involvement, and those having non-recurrent or persistent disease course.


  PC226 Miscellaneous Rheumatic and Inflammatory Disease Top


Cost effective use of biological therapy in Indian scenario

Rakesh Kumar Jagdish, Shailly1, M K Bhatnagar2; Kailash and Max Hospital, Noida, Uttar Pradesh, 1Chest and TB Hospital, Government Medical College, Patiala, Punjab, 2Lady Harding Medical College, New Delhi, India

Background: Biological use in rheumatology has become affordable to extent after the introduction of biosimilars and their cost effectiveness can be increased by proper and timely tapering.

Objective: To understand the cost effective use of biological therapy in Indian scenario.

Methods: This is a retrospective study at single tertiary care centre of 36 patients between the age of 15 to 85 years with different rheumatological diseases having poor response or dissatisfaction with first line medicines and who has received biological therapy. Demographic and clinical parameters were evaluated.

Results: Average age of study population was 50.86 years, 58.33% patients were female. Rheumatoid arthritis was most common primary diagnosis in 47.22% cases, followed by spondyloarthritis in 44.44% cases and 8.33% other cases. Average duration of diseases was 8.83 years, average duration of first line treatment before biological therapy was 5.75 years. Anti-TNF was the most common class of biologicals used in 77.77% of cases (etanercept in 47.22%, adalimumab in 25%, infliximab in 5.55%). Rituximab was used in 19.44% cases and secukinumab in one case. Average duration of biological therapy was 15.92 months. Biological tapering was successfully done in 33.33% of cases (in 22.22% of cases dosing interval increased and 11.11% cases were able to stop biological). Biologicals stopped in 22.22% of cases (due to successful tapering in 11.11% cases, biological failure in 5.55% cases and side effects in 5.55% cases). Response to treatment was earliest and most sustained with rituximab. Biological failure occurs in two cases, one primary failure with adalimumab in rheumatoid arthritis, and one secondary failure with etanercept in psoriatic arthritis, which then treated with secukinumab. Minor side effects were noticed during rituximab infusion in two cases with throat irritation and choking sensation while infusion, which got subsided with decreasing the infusion rate. Major side effect which leads to stopping the medicine were observed in two cases, one etanercept (non healing cellulitis) and one adalimumab (non tubercular lymphadenopathy). Overall toxicity was low and manageable.

Conclusions: We can conclude that wiser use of biological agents can help to stop and taper the biologicals in cost constrained settings.


  PC227 Miscellaneous Rheumatic and Inflammatory Disease Top


Study of pattern of rheumatologic and musculoskeletal disorders and their awareness among patients attending rheumatology clinic

Rakesh Kumar Jagdish1*, M K Bhatnagar1,2, Ayush Malhotra1; 1Santosh Medical College and Hospital, Ghaziabad, Uttar Pradesh, 2Lady Hardinge Medical College, New Delhi, India

Background: Rheumatological disorders are characterized by a wide spectrum of diseases like inflammatory diseases with articular, extra articular and systemic manifestations, degenerative joint and spine diseases, soft tissue rheumatism and metabolic bone diseases still rheumatology is less understood subspecialty of internal medicine.

Objectives: We planned this study to assess the pattern of rheumatologic and musculoskeletal disorders and their awareness among patients attending newly started rheumatology clinic at a medical college and hospital, in first 500 new cases.

Methods: All patients with age 15 years and above with rheumatological diagnosis were included in the study. Detailed history and physical examination done and information regarding the awareness about rheumatological branch, diseases and the commonly used medications like calcium, vitamin D supplements and non-steroidal anti-inflammatory drugs (NSAIDs) were recorded.

Results: Out of first 500 cases 39% cases were of inflammatory joint diseases followed by 22.8% cases of degenerative joint diseases, 15% cases of soft tissue rheumatism, 13.6% cases were of metabolic bone diseases, 8.8% cases of infection related arthritis and 0.8% cases were of benign hypermobility syndrome (BJHS). Among all parameters, awareness about calcium was maximum (42.52%), followed by awareness for vitamin D (23.68%), NSAIDs awareness (21.36%) and least for rheumatology branch (12.32%). Co morbidities were present in 37.4% of cases. Hypertension (7.6%), anaemia (7%), hypothyroidism (6%), diabetes (5.6%), obesity (2.6%), dyslipidemia (2.4%), vitamin B12 deficiency (2.4%) and metabolic syndrome in 2.2% cases. All cardio metabolic risk factors constituted 20.4% of cases.

Conclusion: We conclude that inflammatory joint diseases are most common in a specialised rheumatology clinic, followed by degenerative joint and spine diseases, soft tissue rheumatism, and metabolic bone diseases. Cardio metabolic risk factors are predominantly associated with rheumatological patients and therefore we suggest that proactive screening of these factors should be routinely done for prevention of complications. Patients need more and more public awareness programme to increase their knowledge towards the rheumatology branch and commonly used medicines. Training programmes for physicians should be stated in rheumatology at each institution in this regard.


  PC228 Miscellaneous Rheumatic and Inflammatory Disease Top


Comparison of arthritis in MCTD and SLE

Gunasekaran Sambandam, Mahesh Prakash, Ranjana Minz, Shefali Khanna Sharma, Aman Sharma, Varun Dhir; Post Graduate Institute of Medical Education and Research, Chandigarh, India

Background: Mixed connective disease is a multi-system overlap syndrome with features of SLE, rheumatoid arthritis, scleroderma and polymyositis/dermatomyositis and high titres of U1 RNP. Arthritis is thought to be more common that can be erosive and deforming. We wanted to compare the occurrence of arthritis in MCTD vs SLE patients, both on clinical examination, ultrasound evidence of synovitis and use of methotrexate.

Methods: In this cross sectional study, we used the Kasukawa diagnostic criteria for MCTD and SLICC criteria for SLE patients. Patients were matched for gender and disease duration. A Fluorescence Enzyme Immunoassay in a 1:10 dilution was used for U1RNP.Any past history of arthralgia or arthritis was noted and clinical examination of joints for tenderness, swelling and deformity was done. Functional disability was assessed using the Indian Health assessment questionnaire. Hand radiographs were assessed by for erosions. In addition, ultrasound (9-14 Mhz linear probe) assessment of the non-dominant wrist, metacarpal joints for synovitis and tenosynovitis done using the OMERACT definition.

Results: Forty patients were recruited in each group of MCTD and SLE patients. They had similar gender distribution (F:M=38/2, 38/2), disease duration (3.7±2.3, 4.7±3.1, p=0.1) but slightly younger in age (31.8±13.3, 36±10.2, p=0.01) in MCTD than SLE. ANA was positive in all patients and U1RNP was positive in all MCTD patients. History suggestive of inflammatory arthralgia/arthritis and current arthritis on examination (26 vs 15, p=0.03) was significantly higher in MCTD than SLE [Table 1]. PIP involvement was significantly more in the MCTD [Table 1]. Use of methotrexate was also significantly more in the MCTD group (8 vs 2, p= 0.043). However, there was no difference in ultrasound evidence of synovitis (p= 0.32) or tenosynovitis (p=0.51).Also there was difference in functional disability (0.73 vs 0.74, p=0.7) and presence of erosions. More patients in MCTD group had deformity (4 vs 0) and rheumatoid factor positivity (11 vs 2, p = 0.005).



Conclusion: Clinical arthritis was significantly more common in MCTD patients compared to SLE patients and was associated with increased methotrexate use.


  PC229 Miscellaneous Rheumatic and Inflammatory Disease Top


Profile of patients diagnosed as AOSD admitted to Department of Rheumatology IPGMER and SSKM Hospital Kolkata

Deepak Rath, Debanjali Sinha, Gitabali Sircar, Parasar Ghosh, Pradyot Sinha Mahapatra, Alakendu Ghosh; Department of Rheumatology, IPGMER and SSKM Hospital, Kolkata, West Bengal, India

Background: Adult Onset Still's disease is an uncommon cause of fever and arthritis and is often misdiagnosed initially. It is a cause of morbidity and suffering for the patients. It can be diagnosed based on various criteria, of which Yamaguchi criteria is widely used. We are presenting our cohort of patients who had been admitted to us and diagnosed as AOSD based on their clinical, physical and serological parameters.

Objectives: To study the clinical and laboratorial parameters of patients diagnosed as AOSD in the rheumatology ward.

Methods: Retrospective analysis of case records of patients admitted to Rheumatology Ward.

Results: 32 patients admitted to the rheumatology ward between January 2014 till June 2017 were diagnosed as a case of AOSD based on Yamaguchi criteria. There were 25 females and 7 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% of the population, 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 were 62.5%, 46.9% and 43.8% respectively. Mean Hb was 8.6 ± 1.6 g/dl, mean TLC: 17379 ± 7199/μl, mean ferritin levels were 9775 ng/ml; and the mean LDH values were 1055 IU/ml, mean albumin and globulin levels were 3.2 ± 0.4 g/dl and 3.6 ± 0.7 g/dl respectively. Transaminitis was seen in 71.9% of the study population. 78.1 % of the patients were treated with steroids, while 62.5% of the patients were given methotrexate. Only 2 patients were given Tocilizumab.

Conclusions: All patients had presented with fever with a duration of two weeks or more. Joint involvement was seen in all patients with arthritis predominating. Clinical features of reticulo-endothelial system involvement were seen in the majority of the patients. Globulin fraction was greater than albumin levels in 24 of the patients. Results from our study population are comparable to previous available studies.


  PC230 Miscellaneous Rheumatic and Inflammatory Disease Top


Monocenter study of clinical patterns of interstitial lung disease in rheumatic diseases

Manisha A Daware, Udit Goel, B V Muralimohan, Nithin Hegde; Narayana Health City, Bengaluru, Karnataka, India

Background: Association of interstitial lung disease with Rheumatic diseases influence the prognosis of later. There are different forms of lung involvement in different Rheumatic diseases, and outcome changes depend on type of lung affection.

Objectives: The main objective of this study was to determine the presence of different forms of Rheumatic diseases in patients presented with ILD, and to assess different patterns of ILD.

Methods: Prospectively screening for Rheumatic diseases was done in all the adult patients who presented at Narayana Health City with diagnosed or suspected ILD, over a period of 18 months time (Nov-2014 to Feb-2016). Minimum set of investigations (CBC, RF, ANA, ANA profile, spirometry and CT/HRCT Thorax) were done in all the patients. Paediatric and patients with occupation induced ILD or having diseases which may cause ILD were excluded. Selected patients underwent 2D Echocardiography, DLCO and Bronchoscopy (including BAL) was done where indicated.

Results: Total of 70 patients was screened during the study period. Out of which 78% were female and 22% were male. Rheumatoid arthritis (RA), was the most common Rheumatic disease (48.6%) followed by Systemic sclerosis (21.4%) and others (30%) which include other forms of CTDs (Sjogren syndrome, MCTD, inflammatory myositis, UCTD and overlap syndrome). Rheumatoid factor was strongly positive in 58.6% patients whereas anti CCP antibody test was done in 41 % patients and was strongly positive in 27 patients. ANA (by IIF) was positive in 54.3% patients (majority speckled pattern). Pulmonary function test was done in all the patients, and 97.1 % patients had restrictive pattern. As per HRCT chest 67.1% patients had NSIP pattern (both cellular and fibrotic), 32.9 % had UIP pattern.

Conclusion: NSIP was the most common presentation in different rheumatic diseases. Though ours study included small number of ILD patients, further studies are needed to find correlation between different antibodies and presence and severity of ILD even if patient is asymptomatic for rheumatic disease at the time of presentation. HRCT chest is an important and useful tool to find out ILD at the early stage and recognize the pattern of lung involvement to choose immunomodulation versus.


  PC231 Miscellaneous Rheumatic and Inflammatory Disease Top


Travelling with adult onset still's disease 5 years' experience from a tertiary care centre in South India

Sivakumar Vengudusamy, T N Tamilselvam, S Balameena, M Saravanan, V A Sowndhariya, R Anuja; Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Adult onset still's disease is a rare systemic inflammatory disorder of unknown aetiology. It is characterised by fever, skin rash, joint symptoms, sore throat, hepatomegaly, lymphadenopathy, leukocytosis, liver enzyme elevation, elevated ferritin levels and it’s a diagnosis of exclusion.

Aim: To assess the clinical and laboratory aspects of AOSD patients in our population

Methods: A Retrospective study of 11 patients fulfilling Yamaguchi criteria for AOSD were analysed.

Results: The mean age was 23.7 years; there were 6 males. Mean duration of symptoms was 7.8 months. In addition to prolonged fever (n=11), patients presented with rash (n=7), arthropathy (n=11), hepato-splenomegaly (n=3), peripheral lymphadenopathy (n=6), sore throat (n=3), neutrophilic leucocytosis (n=11), abnormal aminotransferases (n=8), negative RF and ANA in all patients. Most commonly affected joint is knee followed by wrist. 8 patients had oligoarthritis. One patient had a history of papillary carcinoma of thyroid in the past.

Conclusion: Increased awareness and a high index of suspicion is required for the diagnosis of AOSD. Male preponderance is seen. Fever, arthropathy, leucocytosis and negative RF/ANA are present in almost 100% 0f patients.


  PC232 Miscellaneous Rheumatic and Inflammatory Disease Top


Mortality pattern in rheumatic diseases: Data from a tertiary care hospital in Northern India

Bilal Ahmad Rather, Fayaz Ahmad Sofi, Mushtaq Ahmad, Shariq Rashid Masoodi1; Departments of Rheumatolgy and 1Endocrinology, Shere-Kashmir Institute of Medical Sciences, Srinagar, Kashmir, India

Background: Accurate understanding of mortality pattern is important for public health point of view as well as for clinical management of rheumatic diseases.

Objectives: To study the mortality of rheumatic diseases.

Methods: Death register from Jan 2004 to Dec 2014 was analyzed for the data.

Results: In 10 years, a total of 4,30,343 admissions were recorded. During this period 19,391 (4.51%) patients died. Among these 133 (0.7%) deaths occurred in rheumatic disease patients. Majority (83.3%) of the patients with rheumatic disease were in the age group of 20-70 years with almost similar distribution in different subgroups. The mean age at death in patients with non rheumatic disease (n=19,258) was 48.03 ± 23.38 years (range 1 day â€"110 years) compared to 43.94 ±17.79 years (range 11â€"85 years) in patients with Rheumatic disease (p= 0.044). Majority (72.2%) of rheumatic disease patients.were females. There were 41 (30.8%) SLE, 37 (27.8%) RA, 18 (13.5%) Vacuities, 12 (9.02%) MCTD, 9 (6.8%) APLA, 8 (6%) Systemic sclerosis, 4 (3%) Sjogren's syndrome, two each polymyositis and rhupus. Sepsis, cardiovascular diseases, and organ failure (respiratory and renal) were the main causes of death in patients with rheumatic diseases.

Conclusion: Patients Rheumatic diseases have premature mortality compared to the non Rheumatic patients and sepsis being the leading cause of death.


  PR233 Rheumatoid Arthritis Top


TB prophylaxis along with anti-TNF drug lands patient in to ICU

Divya Agarwal, Mehul Lapsiwala1, Praveen Gupta, Nitin Jain, Anunay Agarwal1, Ashok Kumar1; Fortis Memorial Research Hospital, Gurugram, Haryana, 1Fortis Flt. Lt., Rajan Dhall Hospital, New Delhi, India

History: A 67-year old male, known case of RA (8 years duration) with hypertension, was admitted to hospital in June 2017 with drowsiness and loss of appetite for 1 week. There was no history of headache, vomiting, fever, seizures, slurring of speech, weakness of limbs or skin rash. He was on methotrexate + leflunomide with intermittent glucocorticoids. A week ago he was started on etanercept biosimilar and isoniazid + rifampicin (for LTBI).

Examination Findings: Temperature 96.2ºF, pulse 44/min, respiratory rate 14/min, BP 90/54 mmHg, sPO2 88%. Patient was comatose with GCS: E2V2M4. Ankle jerks showed delayed relaxation. He had multiple rheumatoid nodules and deformities [Figure 1]. Other systems were normal.



Investigations: TSH 98 uIU/ml, FT3 <0.4 pg/ml and FT4 <1ng/dl. pH 7.1, HCO3 8, pCO2 23, Na/K 116/4.9. Brain NCCT and MRI normal. Hb 11.7gm%, TLC 12250/mm3, platelet 1,71,000/mm3, AST/ALT 53/21 IU/ml, ALP 89 U/ml, total protein 7.6 gm%, A/G 3.8/3.8, creatinine 2mg%, serum procalcitonin 0.56ng/ml, blood and urine cultures: sterile, CSF normal, chest x-ray: left upper zone opacity, QFT gold, Mantoux, HBsAg, anti-HCV negative.

Management: Bradycardia, hypothermia, typical ankle jerks, raised TSH, hyponatremia and metabolic acidosis established diagnosis of myxoedema coma. Patient recovered with ventilatory support for 4 days along with thyroxine and other supportive measures. His Intacept, rifampicin and isoniazid were discontinued.

Course and Outcome: After successful treatment of myxoedema coma, he was discharged and managed with oral thyroxine 100 mcg/day and conventional DMARDs. Challenges and lessons: In a patient on anti-TNF therapy, infective aetiology is the usual suspicion for any serious complication. In the present case, it was not difficult to establish the diagnosis of myxoedema coma. The question was what precipitated myxoedema coma. Detailed review of literature revealed rifampicin as a well-known trigger for myxoedema coma in the setting of untreated hypothyroidism. This patient was never investigated for hypothyroidism in the past. This was surprising because it is a routine practice to ask for TSH test in the work-up of a patient with rheumatic disease. The learning point here is to rule out hypothyroidism before starting anti-tubercular therapy in any patient to avoid this preventable morbidity.


  PR234 Spondyloarthropathy Top


A rare coexistence of primary hyperparathyroidism with ankylosing spondylitis

Akhil Pawan Goel, Puneet Kumar, Rasmi Ranjan Sahoo, V Harikrishnan, Sourav Pradhan, Anupam Wakhlu; Department of Rheumatology, King George Medical University, Lucknow, Uttar Pradesh, India

Background: Hypercalcemia due to primary hyperparathyroidism is a life threatening complication. It requires early diagnosis and urgent intervention. We report a case of ankylosing spondylitis presenting with features of hypercalcemia due to primary hyperparathyroidism.

Case Report: A 26 year old male patient presented with inflammatory back pain and arthritis involving both knees and ankles for last 5 years. He also had colicky abdominal pain and constipation for last 3 months. There were no features of psoriasis, enthesitis, dactylitis, uveitis or IBD. Examination revealed tenderness in both knee and ankle joints with painful restriction of both hip joints. He also had kyphosis of the thoracic spine. Rest of the systemic examination was unremarkable. Investigations showed Hb:11.7 g/dl, TLC:8300/mm3, platelet count:1.5 lac/mm3, ESR:30mm (WG), CRP:4.3mg/l, Ionized calcium-8.17 mg/dl (4.5-5.5); total S. calcium-12 mg/dl (8-10.5); serum phosphate- 1.64 (2.5-4.8); serum alkaline phosphatase- 835 IU/L (50-270); iPTH-1668 pg/ml (11.7-61.1); vitamin D was 24 pg/ml; TSH was 2.42 Âμg/dl.ECG was normal. HLA-B27 was negative. X-ray pelvis showed bilateral grade 3 sacroiliitis with bilateral reduced hip joint space; X-ray dorsolumbar spine revealed collapse of multiple vertebrae; X-ray of the skull revealed characteristic salt and pepper appearance; X-ray hands showed subperiosteal resorption of phalanges and cystic lesions suggestive of osteitis fibrosa cystica; USG neck showed a cystic lesion (24x17mm) involving lower part of left lobe of thyroid gland; USG abdomen showed focal cortical calcification in the right kidney. Tc99m MIBI parathyroid scan was suggestive of left inferior parathyroid adenoma; MRI sacroiliac joints showed hyperintensities on T2 weighted images indicative of acute sacroiliitis. The patient was managed with adequate hydration, furosemide, zolendronate and calcitonin nasal spray alongwith NSAIDs. The patient underwent parathyroidectomy and is doing well on sulfasalazine and NSAIDs at 6 months follow-up. Ankylosing spondylitis and primary hyperparathyroidism are separate entities that have no causative or pathological relationship and their co-existence has very rarely been reported in literature. Primary hyperparathyroidism may lead to subchondral erosions and pseudowidening of sacroiliac joints mimicking chronic sacroiliitis. However, presence of bone marrow edema on MRI favour’s spondyloarthritis.


  PR235 Spondyloarthropathy Top


Case Report of Secukinumab Use in Psoriatic Arthritis

Rakesh Kumar Jagdish1,2, Shailly3, M K Bhatnagar4,5; 1Kailash and Max Hospital, Noida, 4Santosh Medical College and Hospital, Ghaziabad, Uttar Pradesh, 2AIIMS, Delhi, 3TB and Chest Hospital, Government Medical College, Patiala, Punjab, 5Lady Hardinge Medical College, New Delhi, India

Background: There are very few case reports of Secukinumab use in psoriatic arthritis. Most patients with psoriatic arthritis benefit from anti-TNF therapy but some do not respond, therefore unmet needs remain. Secukinumab is a fully human monoclonal antibody that selectively neutralizes circulating IL-17a. Research suggests that IL-17a may play an important role in psoriasis, psoriatic arthritis and ankylosing spondylitis.

Objectives: To discuss the use of Secukinumab use in psoriatic arthritis.

Methods: A 76 years old male, non smoker, non alcoholic, was diagnosed as psoriatic arthritis 3 years back on the basis of psoriatic skin rash, asymmetrical polyarthritis including distal interphalangeal joints and asymmetric sacroiliitis with raised inflammatory markers. He was allergic to sulphasalazine and intolerant to methotrexate. He was given leflunomide 20 mg daily but he did not responded well to it and therefore anti-TNF agent subcutaneous injection etanercept 50 mg weekly was added to it. He responded well and remains stable for about 2.5 years but any attempt to taper anti TNF resulted in recurrence of symptoms and signs, so patient was continued on same dose. In spite of regular and adequate dose of etanercept and leflunomide, patient developed skin rash on dorsum of both hands associated with gradually increasing inflammatory type of low back pain and polyarthralgia from last 3 months. On examination his tender joint count (TJC) was 15/68, swollen joint count (SJC) was 0/66,without any deformity, Patients pain score was 6/10 score, patient global assessment score was 8/10, with increase in CRP-32 mg/L, ESR-41 mm/h, Disease Activity in PSoriatic Arthritis (DAPSA)-32.2 (High activity). Secondary anti-TNF Failure was considered and etanercept was stopped. Patient was shifted to subcutaneous injection Secukinumab 300 mg every weekly for 4 weeks than monthly, he responded very well within one month, with disappearance of rash and improvement in back pain without any side effect. His DAPSA score significantly improved from 32.2 (high activity) to 2.3 (remission) with marked dermatological improvement.

Results and Conclusions: Secukinumab showed efficacy among patients who had received previous anti-TNF therapy and it is especially useful in Anti-TNF related problems like unacceptable side-effect, primary failure, secondary failure, and immunogenicity.


  PR236 Spondyloarthropathy Top


Pseudo-septic arthritis of a knee: a rare first presenting feature of ankylosing spondylitis

Swapnil Khose, Jayashree Kharaje; Department of Medicine and Rheumatology Division, ESIC Model Hospital cum ODC and PGIMSR, Mumbai, Maharashtra, India

Background: Ankylosing spondylitis is a spondyloarthropathy characterized by axial arthritis, peripheral arthritis, ocular inflammation, enthesitis. Monoarthritis mimicking septic arthritis as presenting feature of Ankylosing spondylitis is unusual.

Case Report: A 31 year old young male admitted with a complaint of acute onset left knee swelling associated with pain and mild fever. There was no history of trauma or other joint involvement like low backache. No past history of Koch's or Koch's contact. There were no signs of extra articular manifestations of Ankylosing spondylitis. On routine blood investigation, WBC was 14800/ cmm, ESR 40 mm and CRP was positive. Rheumatoid factor was negative. There was mild effusion in suprapatellar bursa on ultrasonography of left knee. On MRI left knee, there was peripheral enhancing abscess in intermuscular plane anterior to proximal fibula. Synovial fluid aspiration revealed slightly turbid fluid with WBC 52000/cmm and 70% neutrophils. There was no coagulum formation. Synovial fluid for RA was negative. Synovial fluid was sent for GeneXpert MTB RIF Assay to rule out tuberculosis. It was reported negative as MTB was not detected. Patient started on broad spectrum antibiotics with a possibility of septic arthritis. On day 3 of his admission in our ward, he developed right ankle swelling associated with pain. Patient was subjected to MRI right ankle and HLA B27 to rule out Ankylosing spondylitis. MRI right ankle showed marrow edema, joint effusion with tenosynovitis. HLA B27 was positive. Synovial fluid which was sent for culture and sensitivity at the time of admission was negative. At this point diagnosis of Pseudo-septic arthritis in Ankylosing spondylitis was made.

Intervention: Patient was started on steroids, NSAIDS and oral DMARDS. Subsequently patient received biological agent.

Outcome: At 2.5 month of his follow up, patient was asymptomatic. Patient responded well to the treatment.

Conclusion: Clinicians should have high index of suspicion for Pseudoseptic arthritis when managing with culture negative inflammatory monoarthritis. Pseudo-septic arthritis of knee can be the first presenting feature of ankylosing spondylitis.


  PR237 Lupus APS Sjogren's Syndrome Top


Profile of neuropsychiatric lupus at a tertiary care hospital in North India

Kishore Kunal, Hegde Arun; Department of Rheumatology and Clinical Immunology Army Hospital R and R, New Delhi, Indai

Background: Cerebral manifestations of SLE are common, appearing with a wide range of neurological and psychiatric features. The commonest presentation of primary neuropsychiatric SLE (NPSLE) is an acute or subacute confusional state, followed by other features like psychosis, epilepsy, dementia, stroke, movement disorders, and cranial and peripheral neuropathies.

Case Series: Our case series comprises of 8 patients with SLE (1 male, 7 female) who presented to our hospital with varied manifestations of NPSLE. Case 1 is a 23 year old lady who presented with refractory intestinal vasculitis and later developed features of posterior reversible encephalopathy (PRES) during her stay in hospital. Case 2 is a 30 year old man who presented with pleuropericardial effusion associated with seizures and detected to have bilateral cerebral vasculitic haemorrhages. Case 3 is a 23 years old lady who presented with acute onset paraparesis with sensory loss below umbilicus and urinary retention. She was found to have longitudinally extensive transverse myelitis (LETM) on MRI spine with features of Neuromyelitis optica spectrum disorder (NMOSD). Case 4 is a 12 year old girl who presented with severe headache of one week and pachymeningitis on MRI brain. Case 5 was a 27 year old girl who presented with altered sensorium and features of heart failure. Her MRI brain later revealed acute infarct in Lt parietal lobe and midbrain. Case 6 is a 28 years old lady who presented with acute onset motor aphasia associated with proteinuria and malar rash. Her MRI brain revealed acute ischemic infarct in Lt parietal and temporal lobe. Case 7 is a 35 year old lady who presented with DLE rash, bad obstetric history and features of heart failure. She later developed areflexic quadriparesis and was found to have AIDP (AMAN variant) on Nerve conduction studies. Case 8 is a 20 year old girl who presented with polyarthralgia and acute onset paraesthesia over both upper limbs and back. Her MRI revealed LETM in cervical spine.

Conclusion: This series of patients with NPSLE manifestations highlight the myriad clinical presentation of the disease. Early diagnosis and treatment is the key to successful outcome.


  PR238 Lupus APS Sjogren's Syndrome Top


Pyoderma gangrenosus and APLA syndrome: rare combination .

Anuj Singhal, Arnab Ghosh, Preema Sinha1, V A Arun, V K Sashindran; Departments of Internal Medicine and 1Dermatology, Armed Forces Medical College, Pune, Maharashtra, India

Introduction: Pyoderma gangrenosum (PG), an uncommon entity, is mostly associated with arthritis, inflammatory bowel disease and some hematologic disorders. Increased incidence of PG with autoimmune disorders suggests the immunological mechanism of PG, especially IL-8 and IL-23. However, association of PG with anti-phospholipid antibody (APLA) syndrome has rarely been reported.

Methodology: 46 yr lady presented with rash, for 1 month, initially as red raised lesion behind pinna, later spread to other parts of body, enlarged, turned to black crusted painful ulcer and healed after 15-20 days with scarring. Recurrent similar rashes emerged later with no pruritus or photosensitivity. She had a third trimester pregnancy loss 20 yrs back and underwent bowel resection for mesenteric ischaemia due to superior mesenteric artery thrombosis. Examination revealed level IV right cervical and axillary lymphadenopathy, multiple, tender, plaques over lower limbs with central ulceration and necrosis suggestive of pyoderma gangrenosum [Figure 1]. Biopsy showed subcorneal neutrophilic infiltration with underlying granulation tissue. Further investigations revealed anaemia, 1+ proteinuria, Mantoux test yielded ulceration. Urine Bence Jones proteins, Coomb's test (DCT/ICT), D-dimer, VDRL, both anti-neutrophilic cytoplasmic antibody (cANCA and pANCA), anti-neutrophil antibody (ANA) and Rheumatoid factor (RF) were negative. Serum complements level was normal. Systemic lupus erythematosus (SLE) and polyarteritis nodosa (PAN) were ruled out as they were not meeting diagnostic criteria. Lupus anticoagulant was strongly positive. Colored Doppler flow imaging (CDFI) of mesenteric vessels showed portal cavernoma with porto-splenic collaterals. CDFI of bilateral lower limbs, Upper GI endoscopy, Computerised Tomography of chest/abdomen, and colonoscopy were normal. Fine needle aspiration cytology (FNAC) from axillary lymph nodes showed granulomatous lymphadenitis and no caseation or AFB. She was diagnosed as anti-phospholipid antibody syndrome with pyoderma gangrenosum and managed with oral steroid, hydroxychloroquine, azathioprine as steroid sparing therapy, anticoagulation and topical tacrolimus ointment. In view of strong positive Mantoux test, lymphadenopathy, pyoderma gangrenosum along with thrombosis and steroid therapy isoniazid prophylaxis was given. The overall therapies improved her skin lesions and she is clinically better.



Conclusion: Presence of PG in APLA is a rare combination in this case. Such case report will add to the limited.


  PR239 Vasculitis Top


Association of IgA vasculitis with SLE and GPA

Prijesh Janardanan, Remesh Bhasi1; Departments of General Medicine and 1Rheumatology, Aster MIMS, Calicut, Kerala, India

Background: Henoch-Schonlein purpura (HSP), also known as immunoglobulin A vasculitis (IgAV) is an immune mediated, self limiting systemic vasculitis usually seen in children. It is characterized by tetrad of palpable purpura, arthritis/arthralgia, abdominal pain and renal disease. The characteristic finding of HSP (IgAV) is leukocytoclastic vasculitis accompanied by neutrophil rich inflammatory infiltrate and IgA immune complexes within the affected organs.

Objectives: To study the relation between IgA vasculitis and development of other autoimmune diseases.

Methods: 2 cases under follow-up for IgA vasculitis developing SLE and GPA at a later point of time.

Results: Case 1: 19 year old male with a diagnosis of biopsy proven IgA vasculitis on follow-up developed severe abdominal pain and upon evaluation was found to have high anti dsDNA titer and found to have SLE. Case 2: 30 year old male with a diagnosis of biopsy proven IgA nephropathy on follow-up developed fever and productive cough, which on further evaluation showed multiple cavities in CT. He had very high values of c-ANCA and lung biopsy was suggestive of GPA.

Conclusion: 2 cases in which IgA deposition was demonstrated in the tissue developed a second autoimmune disorder, which prompts the question of immune activation after protein deposition. It is an area of potential research that might have an insight into the pathogenesis of SLE and GPA.


  PR240 Asculitis Top


Eosinophilic granulomatosis with polyangitis: Case vignette

Nachiket Kulkarni*, Naisar Nahar**; *Jehangir Hospital, Pune, **Arham Rheumatology Centre, Nashik, Maharashtra, India

Background: Eosinophilic Granulomatosis With Polyangiitis (EGPA) is a rare disease. Limited information about Indian experience is noted in literature.

Methods: We could identify 5 cases from clinic based database. The study period was Jan 2015-May 2017. The diagnosis of EGPA was clinical. Case details (clinical and laboratory) and treatment outcomes were recorded.

Results: A total of 5 cases identified. Male to female ratio was 4:1. Eosinophilia was noted in all. Case 1: Male. Onset age- 55 years and clinical feature was Asthma. Over two years had involvement of Cardiomyopathy, Ischemic stroke, peripheral neuropathy, polyarthralgia and cutaneous rash(biopsy proven. Positive for P-ANCA. Treatment with Cyclophosphamide and Glucocorticoids. Case2: Male. Onset age-54 years and clinical feature was probable Hypersensitive pneumonitis. Over 1 year had features of Palpable purpura, glomerulonephritis (biopsy proven), multiple intracranial lacunar infarcts, constitutional features. Positive for P-ANCA. Treatment with Rituximab and Glucocorticoids. Case 3: Female. Onset Age 62 years and clinical feature was Asthma. Over 6 months had evolution of mononeuritis multiplex and glomerulonephritis. Positive for P-ANCA. Treatment with Mycophenolate Mofetil and Glucocorticoids. Case 4: Male. Onset age 28 years and clinical feature was Asthma. Over few months had an acute ischemic stroke and cutaneous rash. Positive for P-ANCA. Initiated treatment with Azathioprine and Glucocorticoids. Case 5: Male. Onset age 32. Presenting feature was mononeuritis multiplex. Developed cardiomyopathy, interstitial lung disease, asthma and cutaneous rash within 3 months of onset of initial symptom. Positive for P-ANCA. Initiated on Azathioprine and glucocorticoids. The treatment plan was decided based of patient preference. The response was satisfactory. Case 4 was lost to follow up. Supportive treatment was also ongoing in every case.

Conclusion: In current study pulmonary involvement was the most common presenting feature. Neuropathy and P-ANCA positivity was noted in all. Cutaneous, cardiac and renal involvement had variable involvement. Treatment response was satisfactory.


  PR241 Vasculitis Top


Takayasu's arteritis with malignant hypertension

Rohit Singh, Sandesh Raykar, Sukdev Manna, Saswata Saha; KGMU, Lucknow

Introduction: Takayasu's arteritis (TA) is a form of vasculitis which chiefly affects the aorta and its main branches. Common symptoms and signs include headache, visual symptoms, fever, malaise, weight loss, hypertension, asymmetric pulses and bruit over large vessels. Hypertensive encephalopathy as a complication of renovascular hypertension is a rare manifestation of the disease.

Materials and Methods: A 20 year female, presented with fever, headache and altered sensorium for 3 days. There was no history of seizures or abnormal behavior prior to altered sensorium. At admission her GCS score was 8/15 (E2V2M4),she was afebrile, pulse 96/min in right radial artery with weak pulse in left brachial and radial artery, all peripheral pulses were present, blood pressure in right upper limb was 210/140 mmHg and left upper limb 140/100 mmHg in supine position. Fundus examination revealed papilledema in bilateral eyes. On auscultation, a bruit was heard over the left carotid artery. Rest of the cardiovascular examination did not reveal any abnormality.

Observation: On investigation, her Hb was 12.5mg/dl, Total leucocyte count 15,100(N87,L10,M2,E1), Platelet 1.9lakhs, Creatinine 1.4, Urea 26, Na+ 132.9, K+ 4.1, SGOT/SGPT 46/23, ALP 65, Total protein/Albumin 5.7/3.3, ESR 76, CRP 44, Calcium 8.7, Urine and CSF examination were normal. CT Angiography showed luminal narrowing involving left subclavian artery, descending thoracic and abdominal aorta and its branches (celiac trunk, superior mesenteric artery and bilateral renal arteries) with bilateral renal artery ostial narrowing, suggestive of Type V TA. MRI Brain and MR Brain angiography revealed no abnormality. The patient was treated with antihypertensive and regained consciousness in 2 days of control of blood pressure and oral steroid was started.

Conclusion: This report describes an example of how a common condition in clinical practice, hypertension, may have an unusual presentation as a hypertensive emergency with an even more unusual causeâ€"reno¬vascular disease with Takayasu arteritis. The correct diagnosis at the acute phase requires a thorough and open-minded evaluation of all clinical signs, symptoms, and findings, with realization that an unrecognized hy¬pertensive emergency can lead to permanent damage.


  PR242 Scleroderma, Myositis and Overlap Top


Peripheral gangrene in a rare scleroderma overlap syndrome: A case report

R Ramadoss, V Arya, A Sharma, R S Taneja, R Rana; Department of Medicine, PGIMER and Dr. Ram Manohar Lohia Hospital, New Delhi, India

Background: Overlap syndrome is a well-known entity among rheumatological diseases. Scleroderma overlap syndrome can present with other connective tissue disorders like polymyositis, systemic lupus erythematosus and rheumatoid arthritis etc. But very few cases of scleroderma overlap syndrome with Takayasu arteritis were reported in the literature. We are reporting a case of scleroderma- Takayasu arteritis overlap syndrome presented with peripheral gangrene.

Case Report: A 48 years old woman who was a known case of systemic sclerosis and interstitial lung disease presented with complaints of pain and blackish discoloration of toes in both feet for 1 month. There was no history of fever, muscle pain, weakness, skin rash, joint pain or bleeding from any site. On examination, the patient had a pulse rate of 90/min which was regular and its volume was low in left brachial and radial arteries. Dorsalis pedis pulse was not palpable in both sides. There was a difference of 30mmHg between right and left arm systolic blood pressures. MSK examination showed auto amputated fingers over both hands, telangiectasia, calcinosis cutis and skin thickening. Dry gangrene was there in both feet (Right > Left). Investigation showed a rise in ESR and CRP and positive report of ANA and anti-centromere antibodies. Arterial Doppler of both lower limbs was reported as features of small vessel disease. CT aorta angiogram revealed focal stenosis of branches of the aorta which was suggestive of type IV Takayasu arteritis. The patient was treated with pulse methyl prednisolone followed by oral steroid because of extensive gangrene in both feet. Since there was no response even after a month, she was given cyclophosphamide pulse. But patient succumbed to a respiratory infection happened after 3 weeks.



Conclusion: Scleroderma overlap syndrome with Takayasu arteritis is very rare. Peripheral gangrene with this entity is also less reported. Both Scleroderma and Takayasu arteritis could present with gangrene as described in the literature. Since our patient had progressive gangrene, active vasculitis process was thought of. So the patient was treated with higher immunosuppressive therapy. This case report is unique because it describes a rare combination of two rheumatological diseases with unusual presentation.


  PR243 Scleroderma, Myositis and Overlap Top


Scleromyxedema: Case report

Vishal Haripara, Jyotsna Oak; Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Mumbai, Maharashtra, India

Scleromyxedema (SM) is a variant of Lichen or Papular Mucinosis in which lichenoid papules are present along with scleroderma like features. It was first described by Arndt and Gottron (1954) and then by Rongioletti and Rebora (2001). It is characterised by scleroderma type increased fibroblast proliferation, sclerodermatoid eruptions and mucin deposition. It is associated with paraproteinemia mainly of the immunoglobulin G lamda type monoclonal gammopathy in the absence of thyroid disorder. Patient with scleromyxedema have clinical resemblance to systemic sclerosis and is mistaken due to its multisystemic affection like pulmonary, gastrointestinal, cardiovascular and renal involvement like scleroderma. Scleromyxedema affects both genders at middle age. The standard treatment of SM does not exist as the disease is rare. Until 2009 only 150 cases of SM were reported. The skin of SM patients is elephant like and has linear folds. Histological examination reveals mucin deposits and sclerosis in the form of thick collagen fibres. The mucin deposits are subtle in these lesions. 37 years old male was admitted with complaints of 1 -2 mm waxy, firm, pink colored globular, papular eruptions over forehead, neck, face and both shoulders progressing over 15-20 days and leonine facies. He slowly developed swelling and pain in joints of hands and was unable to do fine movements. He complained of severe periorbital swelling and altered facial features due to thickening of skin over forehead, cheeks and submental swelling. He also had itching of skin. However he did not give any history of Raynaud’s phenomenon, dysphagia, dyspnea on exertion or chest pain. On examination he had coarse features due to severe periorbital swelling. [Figure 1]. His pulse rate was 84 per minute, BP was 130/90 and he had no hepatosplenomegaly or lymphadenopathy. His cardiovascular and respiratory systems were normal. His investigations revealed Hb of 15.7 gm/dl, WBC 17000/cmm.Platelet count 3.8 lacs/cmm. His liver functions and renal functions were normal. Thyroid profile and urine examination was within normal limit. The serum protein electrophoresis was normal and no M band was observed. ANA, Anti Scl Antibody was negative. CPK 32.8 U/l (10 to 171 U/L) Radiological and ultrasonographic examinations were performed to exclude any systemic involvement of the disease but they were normal. Skin biopsy showed increased fibroblast and increased space between collagen bundles due to mucin deposition [Figure 2]. Based on clinical features, histopathological and laboratory data the diagnosis of scleromyxedema without paraproteinemia was obtained. The treatment with intravenous gammaglobulins 20gm/ day was started and was given for 5 days along with systemic corticosteroids.



Discussion: The pathogenesis of scleromyxoedema is uncertain. The majority of patients of SM have concomitant monoclonal gammopathy of unknown significance. The serum of these patients has been shown to produce fibroblast proliferation in vitro as well as an increased production of hyluronic Acid and prostaglandin E. However the immunoglobulins alone does not reduce these effects. SM typically presents with symmetric sheets of linearly arranged 2-3 mm waxy,firm flesh coloured papules most commonly involving face,neck,forearms and dorsum of hands. Scleromyxoedma (SM) is a rare cutaneous disorder characterised by progressive cutaneous mucinosis. Its etiology is unknown. Since SM is rare, therapeutic trials do not exist. SM can be associated with paraproteinemia and other systemic disorders. But in our patient there was no evidence of gammopathy and his serum protein electrophoresis was normal. Due to severity of the disease it requires aggressive treatment and long term maintenance therapy which is essential in most of the cases. Our patient was initially treated with intravenous immunoglobulin and then maintained on oral regime of lenalidomide. He showed remarkable improvement after ivIg [Figure 2]. The swelling of fingers and cutaneous eruptions as well as periorbital oedema showed regression. IvIg is considered as the best therapeutic option for SM as it has few side effects. Plasmapheresis has short term efficacy and often leads to relapse. With IvIg our patient's skin eruptions and scerodermoid lesions became less visible. In a therapeutic study of 30 patients it was observed that the mean age of diagnosis was 19 years and monoclonal gammopathy was dectected in 27 patients. Intravenous Immunoglobulin had induced a complete remmission in 4 and partial remission in 9 patients with treatment duration of 2 years.21 patients had followed for 33.5 months. Our patient was then maintained on lenalidomide. A few case reports are available with long term efficacy of lenalidomide which is an oral immunomodulatory agent showing positive response in patients with multiple myeloma. This drug is an analogue of thalidomide with better tolerability profile, less somnolence, constipation and neuropathy. Thus allowing long term administration. Lenalidomide has immunomodulatory effect due to stimulation of NK cell activity and the increased IL-2 production which enhances cell activity. These properties make Lenalidomide a good maintenance treatment even though the sideeffects such as cytopenia and thromboembolic events have been reported. The recent introduction of highly effective antiplasma cell therapy such as Bortezomib, Thalidomide and Lenalidomide provide a higher likelihood of achieving a complete response. The favourable toxicity profile of these agents makes it more reasonable treatment even in patients without manifestations of multiple myeloma.




  PR244 Scleroderma, Myositis and Overlap Top


Psoriasis and dermatomyositis: Unusual coexistence

Asmita More; Dr. Vasantrao Pawar Medical College and Research Centre, Adgoan, Nashik, Maharashtra, India

Background: Psoriasis is an autoimmune chronic inflammatory skin disease that is common in India. In spite of the high prevalence of psoriasis in the general population, its association with dermatomyositis only occasionally been reported.

Abstract: 58 years Male, diagnosed case of psoriasis 4 months back, presented with difficulty in getting up from squatting position and pain in both knee joints. On enquiry had past history of breathlessness and dry cough. He was diagnosed as Nonspecific Interstitial pneumonitis (NSIP) on HRCT (DEC 2014) and treated with steroids for 18 months, off medications since May 2016.On clinical evaluation had proximal muscle weakness involving both upper and lower limbs with arthritis with synovitis at both knee joints. Further investigations like CPK total, EMG, MRI-THIGH and muscle biopsy were suggestive of inflammatory myositis. Autoimmune workup confirmed diagnosis of Antisynthetase syndrome (ANA, Anti-Jo1 antibody positive). Malignancy workup was negative. We also confirmed diagnosis of psoriasis clinically by two dermatologist and skin biopsy was suggestive of parakeratosis and micro munro abscess. Patient was started on steroids and Methotrexate, on follow up showed marked improvement in proximal muscle power, skin lesions and joint pain. He is on tapering doses of steroids on regular follow up.

Conclusion: NSIP or inflammatory myositis is rarely found in Psoriasis and are pointer for further workup of patient. Coexistence of Psoriasis and connective tissue disorder is rare.


  PR245 Scleroderma, Myositis and Overlap Top


Early onset systemic sclerosis with internal organ involvement

Saumyaleen Roy, Anup Singh, Manaswi Chaubey, Uday Prabhakar Mishra, Chandran Kr Baranwal, Deepa Kumari; Benares Hindu University, Varanasi, Uttar Pradesh, India

Background: Systemic sclerosis (SSc) is an uncommon disease in childhood. Its annual incidence is 1/ million children. In juvenile onset SSC internal organ manifestation and the frequency of autoantibodies are less pronounced than in adult onset SSC. We report a case of 13 yr old female who developed lung involvement within 18 months of the onset of disease. This highlights the fact that rapid progression of internal organ involvement may occur in children and treatment plan must be tailored on a case to case basis.

Objective: Childhood systemic sclerosis along with early involvement of internal organs.

Methods: Patient came with blackish discolouration of the skin along with tightening of the fingers for 18 months and progressively developing contracture of fingers and knees and shortness of breath for the past 1 year mainly on exertion, Raynaud's phenomenon negative. ANA, anti centromere antibody,anti Scl 70, along with PFT and HRCT chest done.

Results: ANA positive, anti Scl-70 positive, HRCT chest b/l ground glass opacification in b/l lower lobes along with cystic spaces which proves it to be a SSC with ILD variant.

Conclusion: Association of early onset systemic sclerosis with internal organ involvement can be seen.


  PR246 Scleroderma, Myositis and Overlap Top


Rapidly progressive ILD in a case of myopathic dermatomyositis

D Patel, H Abdul-Rahim, V Bejarano, L Croot; Barnsley Hospital, Sheffield, England, UK

Introduction: Dermatomyositis (DM) is an autoimmune disease that mainly affects the skin and skeletal muscle. However, a rare form of DM with no accompanying muscle symptoms was designated as amyopathic DM (ADM) by Pearson (1) or Euwer and Sontheimerv. A treatment-resistant interstitial lung disease (ILD) may complicate ADM and result in worse prognosis.

Case Report: A 52 year old male with no comorbidities was referred to Rheumatology department with a 6 weeks history of symmetrical small joint inflammatory arthritis, rash over his MCP/PIP joints, chest wall and with hoarseness of voice. He developed SOB on exertion over few weeks. The skin examination findings were suggestive of dermatomyositis and subsequent skin biopsy confirmed it. He had no muscle weakness and his CK and EMG were normal. A full infection screen and malignancy screen were unremarkable. During his in patient stay his SOB worsened and imaging of his lungs (CT chest, HRCT) done 15 days apart showed significant deterioration with features suggestive of ILD, in spite of rapid administration of high doses methylprednisolone and immunosuppression including cyclophosphamide, IVIG and finally Rituximab. In spite of these, he required high respiratory support for respiratory failure and subsequently demised. Discussion According to Strauss et al, 5-10% of patients with Dermatomyositis have pulmonary disease in the form of interstitial pulmonary fibrosis, respiratory muscle insufficiency, or aspiration pneumonia. Amyopathic Dermatomyositis (AMD) patients have 80% survival in less than a year and drops to 60% if ILD develops. In our patient, the progression of ILD was very rapid and resistant to treatment. The anti-melanoma differentiation-associated gene 5 (anti-MDA-5) antibody is associated with rapidly progressive ILD (RP-ILD), this was not checked in our patient.

Conclusion: Dermatomyositis is rare and the AMD type is even more uncommon, therefore, recognizing it can be a challenge. Appearance of signs and symptoms of interstitial pneumonitis in a patient newly diagnosed with dermatomyositis should be considered a medical emergency (2).

References

  1. Person CM. Polymyositis and dermatomyositis. In: McCarty DJ, editor. Arthritis and Allied Conditions: A Text Book of Rheumatology. 9th ed. Philadelphia: Lea & Febiger; 1979. p. 742-61.
  2. Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkjaer L, Airio A, et al. Frequency of specific cancer types in dermatomyositis and polymyositis: A population-based study. Lancet 2001;357:96-100.



  PR247 OA, Osteoporosis Crystal Arthropathy Top


Clinical presentation and management of gout in post liver transplantation situation: A catch 22 situation

M Harish Kumar, Phanikumar evarasetti, I R Vara Prasad, Liza Rajashekar, Department of Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background and Objective: Post organ transplant patients developed hyperuricemia and gout with calcineurin inhibitors (CNI), more so with renal transplant, and uncommon in liver transplant. Tacrolimus is used along with MMF, sirolimus and prednisolone. The case is presented highlighting the rapidity of erosions and tophus formation (without pretransplant risk factors for hyperuricemia or gout and normal renal function) and the clinical challenge between immunesuppression and drug toxicity.

Case Report: 46 yr old male a diagnosed case of end stage liver disease underwent a live donor(LD) transplantation from his sister(10 Dec 16) and immune suppressed with prednisolone 7.5mg, Tacrolimus 2mg,mycophenolic acid 720mg and Everolimus 0.5mg BD. Four weeks post transplantation he developed acute swollen tender Rt midfoot and 1st MTP joint with fever. Investigations [Table 1] revealed thrombocytopenia with transaminitis. Normal foot Xray. Initial USG foot revealed collection with septation. Septic arthritis was considered and exhibited broad spectrum antibiotics but the transaminitis persisted, hence acute rejection was also considered and pulse methyl prednisolone of 1gm (3 doses) was given on 31 Jan 17. Patient had become afebrile joint symptoms persisted. At our rheumatology centre repeat HRUS of the 1st MTP revealed synovial thickening with increased vascularity, hyperechoiec non vascular nodules, focus of erosion with overhanging edges on the metatarsal head with fluid collection. Aspiration showed needle shaped intracellular and extracellular crystals on microscopy. Diagnosed as gouty arthropathy with tophus and exhibited colchicine 0.5mgBD with febuxostat 40mgOD. His swelling resolved but persisted to have transaminitis. Liver biopsy planned to evaluate rejection and colchicine stopped. Transaminitis resolved but pain recurred. The immunesuppression doses could not be reduced due to fear of rejection. Hence a balance was achieved with giving colchicine 0.5mgonce in 3 days by end of May 17, when he became asymptomatic clinically and resolution of transaminitis and improvement of hemogram.



Conclusion: Post liver transplant drug induced gout is unpredictable and can be very aggressive with rapid joint destruction and tophus formation. It needs a very delicate balance between immunesuppression and anti inflammation so as to avoid rejection and prevent joint destruction.


  PR248 Paediatric Rheumatology Top


IgM nephropathy in systemic onset jia: A case report

S Mythili, T N Tamilselvam; Institute of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: IgM Nephropathy is a rare idiopathic autoimmune complex mediated glomerulopathy with deposition of IgM antibody as a single or dominant immunoglobulin in the mesangial regions of the glomeruli. The cause of this rare entity of IgM Nephropathy remains unknown.

Objective: We are reporting a rare case of IgM Nephropathy in Systemic onset JIA.

Methods: A 19 year old male child, a known case of systemic JIA (as per ILAR) of 7 years duration developed proteinuria and renal biopsy was resorted. Biopsy features were consistent with IgM nephropathy. He received low dose steroids, remission was obtained in one month and proteinuria disappeared.

Discussion: IgM NEPHROPATHY has been reported in SLE, Rheumatoid arthritis, Diabetes mellitus, Alports syndrome, paraproteinemia.

Conclusion: IgM Nephropathy is important but neglected pathology responsible for renal morbidity in children in developing countries with prompt response to steroids in 50% and rest requiring immunomodulant drugs.


  PR249 Infection Related Rheumatic Disease Top


Seronegative inflammatory polyarthritis associated with hydatid disease

Sourav Pradhan, Puneet Kumar, Rasmi Ranjan Sahoo, Akhil Pawan Goel, V Hari Krishnan, Anupam Wakhlu; Department of Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India

Hydatid disease caused by Echinococcus granulosus is endemic in India, with liver being the most commonly involved organ. Musculoskeletal involvement is uncommon and usually presents as hydatid cyst of bone1. We report a 50-year old hypothyroid female on treatment, who presented with symmetric small and large joint polyarthritis for 2 years. Her arthritis was not in remission despite treatment with prednisolone 10 mg/day, methotrexate 25 mg/week, sulfasalazine 2 gram/day and hydroxychloroquine 400 mg/day. She also complained of vague abdominal discomfort and occasional loose stools. Examination revealed an inflammatory small and large joint symmetrical polyarthritis and 15 cms, firm, non-tender hepatomegaly. Rest of general and systemic examination revealed no significant abnormality. Erythrocyte sedimentation rate was 60 mm in 1st hour (Westergren) and C-reactive protein was 57.03 mg/l (0-6). Rheumatoid factor, anti-cyclic citrullinated peptide antibody, antinuclear antibody and HLA B27 were negative. X ray hands and pelvis were normal. Colonoscopy revealed no abnormality. Nerve conduction study showed bilateral carpal tunnel syndrome. Ultrasonography of abdomen showed multiloculated cystic lesion in the right lobe of liver, likely hydatid cyst and chlolelithiasis. Computed tomography of abdomen revealed findings suggestive of one hydatid cyst in right lobe (10cm×6.4cm) and one in left lobe (4.7cm×4.5cm) of liver. Serum echinococcal IgG antibody was positive. The patient was started on albendazole 400 mg twice daily for one month. Number and size of cysts subsequently necessitated surgical resection along with cholecystectomy. Her inflammatory arthritis subsided and she went into remission within 6 months of surgery. She is currently in remission on tapering medications with low dose steroid (5mg/day), methotrexate 17.5mg/week. It is possible that the refractoriness of the arthritis in this patient was probably a consequence of the reactive process against Echinococcal antigen1,2. This case exemplifies inflammatory arthritis as a consequence of echinococcal infection.

References

  1. Alım B, Çetinel S, Servi MA, Bostancı F, Bingöl M. The case of reactive arthritis secondary to echinococcus infestation. Case Rep Rheumatol 2017;2017:3293060.
  2. Buskila D, Sukenik S, Klein M, Horowitz J. Polyarthritis associated with hydatid disease (echinococcosis) of the liver. Clin Rheumatol 1992;11:286-7.



  PR250 Infection Related Rheumatic Disease Top


An uncommon case of unilateral Sacroilitis in elderly patient

Kishore Kunal, Hegde Arun, Vasdev Vivek, M N Arjun; Department of rheumatology, Army Hospital Research and Referral, New Delhi, Indai

Background: Infective causes of sacroilitis account for 20-40% of all causes of unilateral sacroilitis. Mycobacterium tuberculosis is the commonest cause of mycobacterial osteomyelitis and arthritis worldwide. Nontuberculous mycobacterial (NTM) soft tissue infections are common but skeletal disease is rare.

Case Report: Our patient is a 77 year old male, who was admitted with history of high grade, intermittent fever with chills and rigors of 15 days duration associated with right buttock pain and significant weight loss over 3 months. He was a known case of Hypothyroidism, osteoarthritis â€" post TKR status and chronic kidney disease (C3 Glomerulonephritis) on conservative management. He had underwent bone marrow examination at a civil hospital in Jan 2017 for evaluation of plasma cell dyscrasias in view of anasarca and proteinuria (24 hr urinary protein â€" 10 g/day). Clinically he had severe local tenderness over Right SI joint with normal systemic examination. Lab investigations revealed anemia, raised acute phase reactants (ESR: 140 mm and CRP: 145 mg/l) and azotemia (BUN/creat: 45/2.6). MRI of SI joints revealed right sacroilitis with T2W hyperintensities in surrounding muscles and soft tissues. His FDG PET scan was suggestive of FDG avid periarticular lytic changes. He was investigated for unilateral sacroilitis and his HLA-B7 was negative. His Quantiferon Gold TB test, GeneXpert for MTB and serology for Brucella was negative. He underwent FNAC of the Rt SI joint under CT guidance. His biopsy sample was tested for NTM PCR and was detected to be positive for rapid growers. He was managed as a case of NTM sacroilitis with parenteral (Inj Imipenem for 8 weeks) and oral antibiotics (Azithromycin/Doxycycline for 6 months) with fair response. His back pain had subsided completely after 15 days of treatment and his oral antibiotics were continued for 6 months.

Conclusion: Nontuberculous mycobacteria (NTM) are distinct group of mycobacteria causing indolent infection of musculoskeletal system. NTM infections are usually the result of direct inoculation occurring due to penetrating trauma or invasive medical procedures. Therapy for NTM osteomyelitis comprises of combination of oral and parenteral antibiotics for minimum 6 months.


  PR251 Miscellaneous Rheumatic and Inflammatory Disease Top


An unusual mimic of vasculitis: Primary pulmonary Amyloidosis

Shiv Kumar Suman, Anu Daber, Sauvik Dasgupta, Sukesh C Nair, Adarsh Kumar, Uma Kumar*; Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, India

Primary pulmonary amyloidosis is a rare form of localized amyloidosis presenting in the 5th -6th decade. Following is an interesting case of a female patient aged 40-year with past history of extra-pulmonary tuberculosis (EPTB), who presented with recurrent haemoptysis and chest pain for 4 years. Haemoptysis (30-40 ml) occurred once every 3-4 months. She was diagnosed as a case of pulmonary TB based on her clinical profile and a bronchoalveolar lavage positive for acid fast bacilli, for which she received anti-tubercular treatment elsewhere. The patient's condition worsened. She was re-evaluated - computed tomography scan of the chest [Figure 1] revealed multiple cavitating nodules without any lymphadenopathy, mild renal dysfunction along with pANCA (IIF) positivity. A possibility of vasculitis was considered and she was started on oral cyclophosphamide, but no clinical improvement was noticed after 3-months and patient was referred to AIIMS, Rheumatology department. Patient was subjected to bronchoscopy with BAL along with other relevant investigations. Bronchoscopy revealed tracheobronchial nodules, which on histopathological examination showed amyloid deposits. Patient was thoroughly screened for systemic amyloidosis which was negative. A diagnosis of primary pulmonary amyloidosis was made and patient was kept under observation, weighing the risk benefit ratio of chemotherapy.



Conclusion: Primary pulmonary amyloidosis is a mimicker of ANCA associated vasculitis and must be considered in the differential diagnosis in appropriate clinical setting.


  PR252 Miscellaneous Rheumatic and Inflammatory Disease Top


Metastatic non small cell carcinoma with Immune related adverse effects secondary to immune checkpoint inhibitors

Prasan Deep Rath, Shaloo Bhasin; Department of Rheumatology Max Super Speciality hospital, New Delhi, India

Background: The recent success of immune checkpoint blockade in cancer therapy illustrates the importance of the inhibitory receptors cytotoxic T-lymphocyte-associated antigen 4 (CTLA4) and programmed cell death protein 1 (PD1) in the regulation of antitumour immune responses. However, blocking signalling by these inhibitory immune checkpoint receptors is also associated with substantial inflammatory effects that can resemble autoimmune responses, which is consistent with the role of these receptors in protecting the host from excessive inflammation. The entire spectrum of side effects induced by these inhibitors is referred to as immune related adverse events (IRAEs). IRAEs with rheumatic phenotypes are increasingly being recognised. Inflammatory arthritis, sicca syndrome, inflammatory myopathy, vasculitis and lupus nephritis have been described as a result of ICIs.

Objective: Early intervention by Rheumatologists can help in determining whether the symptoms experienced by patients represent a true inï¬'ammatory disease or other rheumatic IRAEs and be managed accordingly to prevent longterm mortality and morbidity.

Methods: 49 years old male presented with acute hemiparesis with features of raised ICT. On evaluation found to have left upper lobe mass lesion (biopsy was s/o of non small cell carcinoma) with metastatic brain disease. He was managed with conventional and systemic chemotherapy however disease progressed with e/o spinal cord compression and skeletal metastasis. In view of disease progression was started on Nivolumab. Fourth day post immunotherapy patient developed Maculopapular rash with oral ulcers, redness of eyes and progressive dyspnea. Investigations revealed Transaminitis with HRCT showing new infiltrates in b/l lung fields s/o pneumonitis. Eye examination was s/o inflammatory keratitis. Sterile cultures ruled out infection. There was no improvement despite broad spectrum antibiotics. Patient was treated with parenteral steroids following which his general condition improved. Skin lesions improved, transaminitis settled.

Results and Conclusion: The expertise of rheumatologists in evaluating rheumatic signs and symptoms and treating patients with immunosuppression are critical in ensuring the optimal outcomes for patients with rheumatic IRAE. Wake up call for rheumatologists to familiarise themselves with these agents and the evolving literature and be poised to identify and manage such complications.


  PR253 Miscellaneous Rheumatic and Inflammatory Disease Top


Sarcoidsis and Sjogren's syndrome: A diagnostic dilemma

Nahar Naisar, Nahar Prachi; MGMHIS, Nasik, Maharashtra

Sarcoidosis and Sjogren's syndrome (SS) are both multisystemic inflammatory diseases of unknown etiology. Although lungs and the tracheobronchial tree are the most common involvement sites in sarcoidosis it rarely affects the salivary and lacrimal glands. By contrast, SS mainly affects the exocrine glands, but it may also have systemic manifestations. We would like to report a case of a 48 year old female patient presented with dryness of mouth and dry eyes since past 3 months. She also complaints of exertional dysponea. There were no symptoms of arthralgia, myalgia, muscle weakness, rash, photosensitivity, oral ulcers, parotid swelling, or Raynaud phenomenon. Laboratory examinations revealed increased erythrocyte sedimentation rate and normal C-reactive protein. Routine hematological and biochemistry were normal. Rheumatoid factor, antinuclear antibody (ANA), dsDNA and antibodies against SS-A/Ro52, SS-B were negative. Mantoux test and TB gold were negative. Serum angiotensin converting enzyme level was elevated remarkably. HRCT Chest showed bilateral hilar lymphadenopathy. Lip biopsy was perfomed and histopathological examination showed non-caseating granuloma, consistent of sarcoidosis. Patient started on steroid. She improved and on regular follow up.


  PR254 Miscellaneous Rheumatic and Inflammatory Disease Top


Sclerosing mesenteritis post bariatric surgery

D Patel, A Nasta, O Srivastav, B Paunipagar, R Goel; Wockhardt Hospital, Mumbai, Maharashtra, India

OBJECTIVES: Sclerosing mesenteritis is a very uncommon, benign pathological process usually involving the small bowel. As this condition is very rare and lacks typical clinical manifestations, diagnosis can be delayed resulting in poor outcome for the patient. We describe the diagnostic and therapeutic challenges faced in a patient with sclerosing mesenteritis post bariatric surgery and describe a prompt multidisciplinary approach and treatment strategy which resulted in favorable outcome.

Methods: A 32 year old male with BMI of 43, without co-morbidities or previous surgery underwent a standard laparoscopic sleeve gastrectomy. 6 weeks post surgery he presented with upper abdominal pain and bilious vomiting. His vital parameters were stable, abdominal examination revealed tenderness and guarding with a diffuse mass above his naval. The investigations for complete blood count, renal, lipase, hepatic profile were normal. Inflammatory markers were high, CRP 200 (normal <5) ESR 110 (0-20 mm/ hr). Abdominal CT scan (with contrast) demonstrated increased attenuation of omental and mesenteric fat, thickening of peritoneum, loculated fluid collections in interbowel loops, edema of entire small bowel with a left pleural effusion. The features were in favor of polyserositis. A full infection screen was negative including pleural fluid (no evidence of TB or malignancy).A detailed Rheumatology history and investigations (ANA, Dsdna, ENA negative, C3 C4 normal, IgG4 normal) did not suggest an associated autoimmune disease. Based on a MDT approach (comprising of a Bariatic surgeon, Radiologist, Rheumatologist, Infectious disease consultant), a literature review and discussion of radiological features we agreed a diagnosis of sclerosing mesenteritis secondary to recent bariatic surgery. As the patients symptoms were worsening he was initiated on intravenous Methyl prednisolone 500 mg for 5 days and intravenous immunoglobulins 400 mg/kg body weight 5 days given the high disease burden. The patients symptoms settled and was switched to oral prednisolone 1mg/kg body weight with a tapering course. He is commenced on azathioprine as a steroid sparing agent and has responded well. The CT scan post treatment has shown improvement from previous.

Results: We faced multiple challenges in this patient both for diagnosis and treatment. A biopsy of mesentery was considered but not performed as the risk of perforation was too high. The treatment plan was carefully tailored and monitored given high risk of steroids in a recent bariatric surgery patient.

Conclusion: The diagnosis of sclerosing mesenteritis is a challenge to surgeons, radiologist and pathologists. A Rheumatologist needs to be aware of its association with other autoimmune conditions. It has rarely been found as a part of the initial presentation with lupus, relapsing polychondritis, and limited systemic sclerosis. (1-2). The treatment should be individualized based upon symptoms, complications and co morbidities. CT features of this disease are usually highly suggestive and although a histopathologic confirmation of the diagnosis is required, medical management is a safer and more effective treatment modality than surgery in these patients (3-4).

References

  1. Sauvaget F, Piette JC, Galezowski N, Jouanique C, Chapelon C, Blétry O, et al. Relapsing polychondritis and mesenteric panniculitis: Apropos of 2 cases. Rev Med Interne 1993;14:253-6.
  2. Arroyo-Ávila M, Vilá LM. Limited systemic sclerosis initially presenting with mesenteric panniculitis. BMJ Case Rep 2014;2014. pii: bcr2014206961.
  3. Talwar A, Rayner H. The medical mystery of the fatty mesentery. BMJ Case Rep 2012;2012. pii: bcr1220115443.
  4. Ferrari TC, Couto CM, Vilaça TS, Xavier MA, Faria LC. An unusual presentation of mesenteric panniculitis. Clinics (Sao Paulo) 2008;63:843-4.



  PR255 Miscellaneous Rheumatic and Inflammatory Disease Top


Rituximab Induced Serum sickness; 3 case reports

R Padmaja, Nissy Mathew, Mariam Varsha Joseph, Padmanabha Shenoy; Centre for arthritis and Rheumatism Excellence, Cochin, Kerala, India

Rituximab, a chimeric anti-CD20 monoclonal antibody, is widely used in the treatment of autoimmune rheumatic diseases. Being a chimeric molecule, immune system mounts response against rituximab. Although infusion related adverse events are seen in upto 15 % of patients, type 3 hypersensitivity reactions like Serum sickness, has been reported rarely. It has been reported to be more common among patients with autoimmune conditions than hematologic malignancies. Here we report 3 cases of serum sickness associated with Rituximab. Case 1 is a case of Scleroderma with ILD and Case 2 and 3 are Systemic Lupus Erythematosus with Lupus nephritis. In all three cases reported, the patients presented with generalised rash, myalgia, arthralgia within 7-9 days post infusion and responded briskly to steroids. They were initiated on steroids All our patients responded to steroids within 5-7 days and their symptoms resolved completely. RISS (Rituximab Induced Serum Sickness) may mimic exacerbation of various rheumatologic conditions, hence it is important to recognize it clinically. It is seen in about 1-20% of treated patients and occurs as a result of IgG antibodies which forms complex with the Fab fragments of the drug. These complexes get deposited at the tissues and vasculatures. This leads to inflammation, tissue damage and increased vascular permeability. It is typically self-limited however most of the time subsides rapidly with steroids. Further infusions of rituximab should be avoided, as it may provoke more severe symptoms.


  PR256 Miscellaneous Rheumatic and Inflammatory Disease Top


Pituitary macroadenoma masquerading as rheumatologic manifestations

Prachi Patil, Praveen Jadhav, Suit Chandatre; OmKar Heart and Rheumatology Institute, Nashik, Maharashtra, India

Background: Rarely endocrine disease can present to rheumatology clinic with joint disorders. Acromegaly is one such rare disorder. Typical clinical picture comprises of variable manifestations which involve mainly but not exclusively musculoskeletal system and they evolve slowly over person's lifetime and so diagnosing it early can be keystone to reduce morbidity.

Case Report: Illustrating a case of a 48 year old female, who presented with generalised polyarthralgia and persistent fatigue and early morning stiffness for 10 minutes. Symptoms were firstly noted 18 months before for which she took treatment from various health centres and was prescribed DMARDS (RA factor was positive) but her symptoms remained persistent. She presented to us with this scenario. In addition, she had broad spade like fingers, large forehead and coarse facial features and receding hairline. Her laboratory parameters revealed Hb 11.5g/dl, TLC 5000/cumm, platelets were adequate, ESR 38 mm in an hour, RA factor +ve, ACCP –ve. With this features we suspected her of having acromegaly so CT brain and growth hormone levels were advised. CT brain revealed an enlarged sella turcica s/o pituitary macroadenoma and her growth hormone levels were 92 ng/ml (normal 10 ng/ml). She underwent transphenoidal excision of pituitary macroadenoma. Result: Patient had symptomatic improvement which was also evidenced on clinical examination after 3 months.

Conclusion: Clinicians should suspect endocrine disorders as cause of joint manifestations in relevant cases. Acromegaly due to macroadenoma is a curable cause of articular manifestations.


  PR257 Miscellaneous Rheumatic and Inflammatory Disease Top


Central serous chorioretinopathy: An uncommon ocular complication of steroid therapy

Sunilkumar Singh, Ishita Shah; Kokilaben Dhirubhai Ambani hospital and medical research Institute, Mumbai, Maharashtra, India

Background: Central serous chorioretinopathy (CSC) is characterized by serous detachment of the neurosensory retina presenting as visual disturbance. It is caused by several factors including hypercortisolism and psychosocial stress. Patients with inflammatory disorders receiving steroids are at increased risk of developing CSC. It usually resolves post discontinuation or tapering of steroids. We present three patients of rheumatic diseases who developed CSC at various dosages and duration of steroid therapy.

Case Summary: Case 1- A 44 year old female with SLE was started on prednisolone 15 mg/day which was tapered by 5 mg every 2 week. After 6 weeks, she presented with painless blurring of vision in left eye. She also had significant psychological stress. SLE related ocular complication was suspected. However, Ophthalmology examination showed multifocal central serous retinopathy without any inflammatory component. Prednisolone was stopped and Add to dictionary continued. Patient improved gradually and vision was normal in 3 months. Case 2 A 47 year old male with rheumatoid arthritis, on methotrexate 15 mg per week and prednisolone 5mg per day, presented with polyarticular flare. He was given Methyl prednisolone 80mg intramuscular and methotrexate was increased to 20 mg per week. Within 48hours, he developed blurring of vision in left eye. Ophthalmology examination showed multifocal central serous retinopathy. Oral Prednisolone was discontinued. Vision improved and was normal in 2 months. Case 3- 60 year old female, diagnosed case of adult onset Stills disease was on stable dose of prednisolone 7.5mg per day, Methotrexate 20 mg per week and Cyclosporine 200 mg per day since 18 months. She had a traumatic fracture of fibula and hence was psychologically stressed. She complained of dark spots in front of right eye since 5 days. Ophthalmology examination showed multifocal central serous retinopathy. Prednisolone was tapered to 2.5 mg per day. Vision improved over next 2 months.

Conclusion: Central serous chorioretinopathy should be suspected in patients on steroid therapy presenting with visual disturbance. It is independent of dose, duration or formulation of corticosteroids and stressors play equally important role as risk factor. It usually improves post discontinuation of steroid therapy.


  PR258 Miscellaneous Rheumatic and Inflammatory Disease Top


Chronic recurrent multifocal osteomyelitis

Prasanna P V; KIMS Hospitals, Secunderabad, Telangana, India

CRMO is a chronic non infectious osteomyelitis of auto inflammatory origin. It affects predominantly children. It is characterized by recurrent episodes of sterile osteomyelitis involving metaphysis of long bones. We describe clinical presentation, radiographic appearance, bone scintigraphy findings in a four year old boy of CRMO. He had recurrent pains with swelling in his both upper limbs with fever and no other associated features. His radiographs revealed mixed lytic and sclerotic lesions of long bones of upper limb, three phase bone scan evidence of involvement of bilateral humerus, radial bones, and ribs. He responded well to TNF inhibitors and was on maintenance immunosuppression with sulphasalazine. He had no recurrence of symptoms till date. As CRMO is under diagnosed condition, knowledge of this condition is essential for prompt early treatment initiation to prevent associated long term complications of the disease.


  PR259 Miscellaneous Rheumatic and Inflammatory Disease Top


Leflunomide-induced drug rash with eosinophilia and systemic symptoms syndrome

Nahar Naisar, Dube Yatindra; MGMHIS, Nasik, Maharashtra

Drug rash with eosinophilia and systemic symptoms (DRESS) is an uncommon life-threatening syndrome that may appear within 1 to 8 weeks after taking of the eliciting drug, mainly antiepileptics and antibiotics but it is reported with numerous other drugs. A mortality rate of approximately 10% with DRESS syndrome. We report a case of 32 year lady presented to us with exfoliative dermatitis with acute hepatitis secondary to leflunomide. She was managed with steroids and cholestyramine. Rapid diagnosis with withdrawal of offending agent is crucial for survival of these cases. Careful dosing and periodic monitoring of patients treated with leflunomide is recommended.


  PR260 Vasculitis Top


Successful treatment outcomes in pregnant patients with ANCA associated vasculitides- report of two cases and systematic review of literature

Pawan Singh#, Aadhaar Dhooria#1, Manish Rathi2, Ritesh Agarwal, Kusum Sharma3, Ritambhra Nada4, Ranjana Minz5, Vanita Suri6, Sanjay Jain1, Aman Sharma1; Departments of Pulmonary Medicine, 1Internal Medicine, 2Nephrology, 3Microbiology, 4Histopathology, 5Immunopathology and 6Obstetrics and Gynecology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Background: Pregnant patients with active ANCA associated vasculitis (AAV) are a big therapeutic challenge. There is very limited published literature regarding pregnancy in AAV.

Aim: To report the successful use of rituximab and to systematically review the fetal and maternal outcomes in pregnant patients with active AAV.

Methods: We report two cases of successful pregnancy outcomes in patients with active AAV with the use of rituximab. We also conducted a systematic review according to PRISMA statement. PubMed (inception of PubMed till 30th April 2017, English language only) and EmBase databases were searched using "pregnancy" AND "ANCA associated vasculitis" OR "granulomatosis with polyangiitis" OR "eosinophilic granulomatosis with polyangiitis" OR "microscopic polyangiitis" OR "Churg Strauss Syndrome" OR " Wegener's granulomatosis" as the search terms. The primary outcome measure was incidence of flares in pregnancy, pregnancy-related complications and outcome of pregnancy. The secondary outcome was to document the use of different immunosuppressive drugs in pregnancy and its relation to pregnancy outcomes including birth weights.

Results: Initial search yielded 18,125 citations of which 57 studies were finally included in the review. 137 pregnancies were documented in 110 patients of ANCA associated vasculitis. The diagnosis of AAV was established before pregnancy in 69, during pregnancy in 32 and after pregnancy in 9. 20 flares were noted during pregnancy. There were 91 term deliveries, 28 preterm deliveries, 15 abortions. Three cases of transplacental transfer of anti MPO antibody to the newborn have been reported. 78 patients had normal vaginal delivery and 26 underwent caesarian section. The various immunosuppressive drugs used during pregnancy were oral prednisolone (n=38), pulse methylprednisolone (n=8) azathioprine (n=11), IVIG (N=4), cyclophosphamide (n=11), rituximab (n=2) PLEX (n=3). Overall treatment received included steroids (n=94), azathioprine (n=41), cyclophosphamide (n=38), IVIG (n=9), Methotrexate (n=7), mycophenolate mofetil (n=6), rituximab (n=4) cyclosporine (n=2) and PLEX (n=10). There were three maternal deaths.

Conclusions: Successful pregnancy in AAV patients is achievable. Rituximab may be a reasonable remission induction agent in pregnancy.




 

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