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ABSTRACT
Year : 2019  |  Volume : 14  |  Issue : 6  |  Page : 99-249

Abstract


Date of Web Publication18-Dec-2019

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How to cite this article:
. Abstract. Indian J Rheumatol 2019;14, Suppl S2:99-249

How to cite this URL:
. Abstract. Indian J Rheumatol [serial online] 2019 [cited 2020 Apr 7];14, Suppl S2:99-249. Available from: http://www.indianjrheumatol.com/text.asp?2019/14/6/99/273470




  OB0001: Rethinking safety profile of drugs for rheumatoid arthritis Top


Gurvisha Sandhu, B K Thelma; Dewpartment of Genetics, University of Delhi South Campus, New Delhi, India

Background: Current treatment for Rheumatoid Arthritis (RA) comprises synthetic disease-modifying anti-rheumatic drugs and/or biologics, guided by disease activity assessment. Both drug categories exhibit drawbacks, namely limited efficacy, contraindications and side effects. Discovery of small molecules is presently a major thrust area but adverse drug reactions due to unintended activity at off-targets are a leading cause for attrition in clinical trials of such candidates or withdrawal of marketed drugs. Therefore, there is an unmet need for early safety checks in novel drug discovery.

Objectives: Using genetic and cellular network data, Mitogen-activated protein kinase kinase kinase 8 (MAP3K8) was previously prioritized in the laboratory as a potential drug target for RA. This study aims to develop a rational prediction tool for uncovering off-target liabilities of novel inhibitors designed in this study against MAP3K8, to identify 'safer' molecules for pre-clinical development.

Methodology: Firstly, we built a chemo-centric probabilistic model based on high-throughput screening of 2-D chemical similarities among 1.7 million ligand pairs from a Background dataset of >50,000 unique preclinical and clinical small molecule hits, covering 400 kinases. We then calculated Z-scores and expectation values (E-values) for ligand sets of any size, such that the Background fits an extreme value distribution [Figure 1]. E-value <10-4 for query drug against a particular protein was interpreted as statistically significant signal/noise discriminator.
Figure 1: Cheminformatics schema for target profiling

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Results: On assessing off-targets of FDA-approved drugs to validate the model, E-value predictions matched rationally with their in vitro molecular activity profiles. Of the 13 novel MAP3K8-based small molecule inhibitors, six were 'predicted as specific' with no significant off-targets. In the seven remaining, central scaffold overlapped with a drug candidate for a clinically validated target in cancer and psoriasis, warranting additional investigations.

Conclusions: The cheminformatics tool developed seems robust to identify off-target liabilities for individual chemical agents and is notably applicable universally across human kinome.


  OB0002: Evaluation of the effect of human platelet lysate versus fetal bovine serum on human osteoarthritic cartilage derived chondroprogenitors Top


Upasana Kachroo, Shikha Zachariah, Abel Livingston1, Boopalan Ramasamy2, Elizabeth Vinod13; Departments of Physiology and1Orthopaedics,3Center for Stem Cell Research, CMC, Vellore, Tamil Nadu, India,2Department of Orthopaedics, Royal Darwin Hospital, Tiwi, Australia

Background and Objectives: Chondroprogenitors, considered as a mesenchymal stem cell, maybe a contender for cell-based therapy for cartilage repair due to inherent chondrogenesis and good proliferative capacity. Fetal bovine serum (FBS) is a widely used culture additive for expansion and differentiation of chondroprogenitor cells (CPs). However due to concerns related to potential transmission of zoonoses and possibility of transplant rejections following cell implantation, there is a need for a human derived alternative. Human Platelet Lysate (HPL) has been extensively for expanding various cell lines but data related to its use with chondroprogenitors has not been reported. We therefore aimed to culture human chondroprogenitors in HPL and compare them to those grown in FBS.

Methods: CPs were isolated from osteoarthritic knee joints (n=3) following differential fibronectin adhesion assay. Passage 0 cells were considered for assessment of trilineage differentiation, cell cycle analysis, galactosidase senescence assay, CD marker expression and RT-PCR. Cumulative population doubling (CPD) was assessed up to passage 3.

Results: and Conclusion: Results: obtained showed that CD marker expression, number of senescent cells were comparable between the two groups whereas cellular proliferation was significantly higher with HPL (p<0.05). Observations from trilineage differentiation showed that cells in both groups demonstrated capacity for adipogenic and chondrogenic potential, whereas ability for osteogenic differentiation was notably higher with HPL group. Similar Results: were noted in RT-PCR where cells grown in FBS showed lower expression of markers of hypertrophy.

This was the first in vitro study to establish xeno free conditions for expansion and characterization of human CPs. Although Results: presented in the study identify HPL as an effective medium supplement for growth of human articular cartilage derived chondroprogenitors, further evaluation is required for consideration of HPL as an additive in cellular differentiation towards osteogenic lineage for bone regeneration.

Keywords: Chondroprogenitors, FBS, human platelet lysate


  OB0003: P-gp and HDAC2 may regulates steroid responsiveness in childhood nephrotic syndrome Top


Harshit Singh, Narayan Prasad1, Akhilesh Jaiswal1, Durga P. Misra, Ravi Mishra, Vikas Agarwal; Departments of Clinical Immunology and1Nephrology, SGPGIMS, Lucknow, Uttar Pradesh, India

Background: P-glycoprotein (P-gp) over expression in peripheral blood mononuclear cells (PBMCs) has been reported in patients with steroid resistant nephrotic syndrome (NS). Glucocorticoids suppress NFκB-associated co-activator activity by deacetylation of histone by enzyme histone deacetylase (HDAC)-2.Interaction between HDAC2 activity and P-gp expression in childhood NS patients is not clear.

Aim: To evaluate the role of HDAC2 and P-gp expression on PBMCs and steroid responsiveness in patients with childhood NS.

Materials and Methods: 31 patients were recruited at baseline (n = 31) (before initiating steroid therapy);after six weeks of steroid therapy, 24 patients achieved remission (SSNS n=24 mean age, 7.96±3.90), whereas seven patients were resistant to steroids (SRNS, n=7, mean age 10.00±3.55).mRNA expression of HDAC2 and P-gp/MRP-1 and functional analysis of P-gp as well as enzymatic activity of HDAC2 were analyzed at baseline, at 6 weeks of steroid treatment and at the time of relapse, respectively.

Results: The expression of P-gp mRNA was significantly lower in subjects (n=24) who achieved remission at 6-weeks of steroid therapy as compared to baseline and those who were resistant (n=7) to steroids (P<0.005). Similarly, expression of HDAC2 mRNA was significantly higher at baseline and at remission following 6-weeks of steroid therapy as compared to their expression in those who were resistant to steroids (P<0.005).The function of P-gp was significantly lower in NS patients who achieved remission after 6-weeks of steroid therapy as compared to baseline (p<0.005) and those who were resistant to steroid therapy (p<0.005).The enzymatic activity of HDAC2 was significantly higher in SSNS patients as compared SRNS patients at 6-weeks of steroid therapy (p<0.005).

Conclusion: The expression and function of P-gp and HDAC2 may affect steroid response in NS patients. Combined therapy of steroids with P-gp inhibitor and/or HDAC2 inducers may have rationale in the management of steroid resistant NS patients.


  OB0004: A peptide vaccination approach to ablate auto reactive B-cells as a novel strategy to treat autoantibody-mediated diseases Top


Ram Raj Singh; University of California, Los Angeles, CA, USA

We previously demonstrated that the heavy chain variable regions of anti-DNA antibodies contain epitopes that can bind MHC class I molecules. Vaccination of lupus-prone mice with plasmid DNA vectors carrying minigenes that encode such epitopes induced CD8+ cytotoxic T lymphocytes (CTL) that killed anti-DNA antibody-producing B cells, reduced serum anti-DNA antibody levels, retarded the development of nephritis, and improved survival (Fan G and Singh RR, J Exp Med 2002). Treatment with peptides alone did not induce such CD8+ T-cells, as we and others have reported impaired CD8+ regulatory and CTL responses in mice and humans (Singh RR, et al, J Immunol 2002; Stohl W, et al, Lupus 1999). Here, we asked if we could overcome such impairment using synthetic oligodeoxynuleotides containing unmethylated cytidine-phosphate-guanosine dinucletides (CpG-ODN) that can enhance innate and adaptive immunity.

We immunized lupus-prone (NZB/NZW F1) mice that develop lupus that mimics human lupus with CpG-ODN or a control ODN, and assessed CTL responses. We then treated these mice with CpG-ODN conjugated to MHC class I-binding epitopes that we identified using bioinformatic and cellular binding approaches. We monitored lupus mice for proteinuria, serum anti-dsDNA antibodies, and survival.

Immunization with CpG-ODN corrected the impairment in peptide-specific CTL responses in lupus-prone mice. The CpG-ODN conjugated with an MHC class I-binding, anti-DNA Ab VH-derived epitope induced strong peptide-specific CTL responses against B cells from diseased lupus-prone mice, reduced anti-DNA Ab production, inhibited disease development, and improved survival.

Such peptide vaccine-mediated induction of CTLs that ablate autoreactive B cells represents a novel approach to treat autoantibody-mediated diseases.


  OB0005: Methanolic extract of choerospondias axillaris fruit decreases inflammatory response in rheumatoid arthritis fibroblast like synoviocytes and collagen-induced arthritis model Top


Sonia Mann, Ashish Sarkar, Sagarika Biswas*; CSIR-Institute of Genomics and Integrative Biology, New Delhi, India

Background: Rheumatoid arthritis is an autoimmune, systemic disease which mainly affects joints. Recently the focus has been diverted on traditional medicinal plants for treatment of arthritis. Choerospondias axillaris, Lupsi/Lapsi, is an underutilized and edible fruit of family Anacardiaceae possessing many health benefits.

Objective: Initially Phytochemical analysis of methanolic extract of Choerospondias axillaris has indicated the presence of phytochemicals such as phenols, flavonoids, flavonols, alkaloids etc. As these compounds are of pharmacological interest, coupled with the use of this plant in traditional medicine, encouraged us to check in vitro and in vivo anti-inflammatory activity of Choerospondias axillaris fruit extract.

Methods: The antioxidant in vitro activity was measured by means of the free radicals scavenging assays of the extract. Bioactive compounds of Choerospondias axillaris were identified via LC-MS/MS analysis. The anti-inflammatory effect of methanolic extract was investigated in Rheumatoid arthritis (RA) and Osteo arthritis (OA) primary cells. The study was further extended in collagen-induced arthritis (CIA) rat paw edema model. Further, the medicinal influence of anti-inflammatory bioactive compound of Choerospondias axillaris methanolic extract, were supported by docking studies of ascorbic acid, salicylic acid, quercetin and diclofenac with TNF-α and IL-6.

Results: Both in-vitro and in-vivo studies showed significant decrease in the inflammation. Also, to check the medicinal efficacy of anti-inflammatory bioactive compound of Choerospondias axillaris methanolic extract, docking studies of ascorbic acid, salicylic acid, quercetin and diclofenac with TNF-α and IL-6 was carried out. Docking analysis indicated that salicylic acid inhibits TNF-α with -7.58 kcal/mol binding energy and 2.78 μM inhibitory constant whereas quercetin inhibits IL-6 with -6.36 kcal/mol and 21.9 μM inhibitory constant.

Conclusion: Observed Results: suggest the possible use of Choerospondias axillaris fruit for reducing the inflammatory indicators and symptoms in case of inflammatory diseases such as RA.


  OB0006: Synovial cell-derived micro particles as source of auto antigens and their in vitro effects on synoviocytes in rheumatoid arthritis Top


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

Background: The role of Cell-derived micro particles (MPs) in the pathogenesis of Rheumatoid arthritis is not clearly delineated. The present study investigated the hypothesis that MPs may be a source of auto antigens and drive disease progression in the synovium.

Methods: Five ml of synovial fluid (SF) was collected from knee joints of 41 DMARD naïve patients with RA and 30 patients with OA. Cell-free synovial samples were stained with Annexin-V-APC with either anti-vimentin Alexa Fluor-488 or anti-Glucose regulated protein-78 (GRP78) Dylight-488 for flow cytometry analysis. RA and OA fibroblast-like synoviocytes (FLS) (Cell applications, USA) were co-cultured with respective synovial fluid-derived MPs in vitro for 24 hours. The supernatant was assayed for inflammatory analytes by multiplex assays.

Results: Elevated proportion of AnnexinV+Vimentin+ MPs were present in RA [0.8(1.30)] [median (IQR)] compared to OA [0.3(0.33); p<0.001]. Elevated proportion of AnnexinV+GRP78+ MPs were also found in RA [0.3(0.28)] compared to OA [0.1(0.2); p<0.01]. Among 16 cytokines studied, RA synovial fluid-derived MPs stimulated RA FLS to produce CXCL1, CXCL2, CXCL5, CXCL6, CCL2, CCL8, CCL15, IL8, TNFa, RANTES, and BAFF at high concentrations compared to unstimulated FLS. CXCL6, CCL2, CCL8, CCL15, RANTES, IL6, VEGF, and BAFF were released at higher levels when RA FLS were stimulated with RA synovial fluid-derived MPs compared to OA FLS stimulated with OA synovial fluid-derived MPs. CXCL6, CCL8, and RANTES were higher when RA FLS were stimulated with RA Synovial fluid-derived MP as opposed to MP free SF. However, the stimulatory effects of MPs were lesser than that observed with positive controls (Il-1beta and LPS) [Figure 1].
Figure 1: Levels of CXCL6, CCL2, CCL8, CCL15, RANTES, IL6, VEGF, and OA FLS cell stimulation as-says (a-h). Cells were either left unstimulated (negative control) or stimulated with synovial fluid MP, MP free synovial fluid (2.5 μl), IL-1β (125 pg/well) (thermoflished scientific, USA) (positive control), and lipopolysaccharides from  Escherichia More Details coli) O26;B6 [LPS (1 μg/well) (sigma asdrice, USA) positive control] for 24 h. The data are expressed as mean ± standard deviation of three independent experiments (n = 0). The variation between two independent groups was statistically assessed by unpaired t-test with Wekh's correction. Horizontal lined indicates standard deviation, CXCL, (C-X-C) motif ligand; CCL, (C-C) motif ligand; IL6: Interleukin-6, RANTES: Regulated on activation, normal T-cell expressed and secreted, VEGF: Vascular endothelial grouth factor, BAFF: B-cell activating factor, NV: Negative control (Unstimulated cells), MP: Microparticle, SF: Synovial fluid, LPS: Lipopolysaccharides, RA FLS: Rheumatoid arthritis fibroblast-like synoviocytes, OA FLS: Osteoarthritis fibroblast-like synoviocytes, *P < 0.05, **P < 0.01, ***P < 0.001

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Conclusions: MPs are a source of auto antigens in RA synovium. MPs may stimulate synoviocytes and contribute to local production of autoantibodies, neovascular angiogenesis, and activation of autoreactive cells in RA.


  OB0007: Accumulation of deleterious mitochondrial DNA in peripheral blood mononuclear cells of rheumatoid arthritis patients Top


Kumar Sagar Jaiswal, Shweta Khanna, Arup Ghosh, Prasanta Padhan, Sunil K Raghav, Bhawna Gupta; School of Biotechnology, Kalinga Institute of Industrial Technology, Bhubaneswar, Odisha, India

Background: Due to absence of a defined etiology Rheumatoid Arthritis has become a systemic, debilitating and chronic inflammatory disorder affecting 1% of the total world population. Studies report blend of mitochondrial, environmental, genetic and epigenetic factors responsible for disease progression. Mitochondria are involved in cellular functions and are a deciding factor for fate of immune cells. Mutations in mitochondrial DNA (mtDNA) may alter cellular functions, thus resulting in inflammation. Finding “one for all” cure is difficult due to genetic variations among populations; thus deciphering genetic, epigenetic information and difference in regulatory network between populations can lead us to design a cure based on the principles of personalized medicine.

Objectives: This study aims to identify single nucleotide polymorphisms (SNPs), copy number variation due to deletion or duplication and heteroplasmy in mtDNA hampering overall function of mitochondria.

Methods: mtDNA were sequenced using NGS from 23 RA patients and 17 healthy controls (HC). Further, bioinformatics analyses were used to identify SNPs, changes in heteroplasmy, copy number variations in RA patients and HC along with haplogroup analysis to find any association between mutated mtDNA and RA.

Results: A total of 382 single nucleotide variants were observed in mtDNA; among which 91 (23.82%) of mutations were present in hypervariable region and 291 (76.18%) in coding region of patients and controls. A non-synonymous heteroplasmy in ND1 gene was found at a single position 3533 in increased number of RA patients as compared to HC. Upon analysing copy number variations we could observe a significant increase of duplications in RA mtDNA samples.

Conclusion: The findings of this study indicate altered mtDNA of immune cells and possible role of mitochondria of immune cells in development of RA. The variants identified can be used as prognostic marker for individuals at high risk of developing RA.


  OB0008: Role of altered CD14 expression triggering the fibrotic pathophysiology in the patients with systemic sclerosis Top


Dipanjan Bhattacharjee1, Aniruddha Bagchi1,2, Deepak Rath1, Ayindrila Saha1, Sanchaita Misra1, Sudipta Chatterjee1, Sulagna Chatterjee1, Pradyot Sinhamahapatra1, Alakendu Ghosh3; 1Institute of Post Graduate Medical Education and Research/SSKM Hospital, Departments of3Rheumatology and2Pharmacology, Institute of Post Graduate Medical Education and Research/SSKM Hospital, Kolkata, West Bengal, India

Background: Fibrocytes, the cells of leukocyte lineage, after activation, express markers of fibroblasts [CD90, α-smooth muscle actin (αSMA), Collagen-1(Col1)] by reducing leukocytes markers (CD34, CD45, CD14). Fibrocyte activation and transdifferentiation may trigger the fibrotic pathophysiology.

Objectives: Isolation and characterization of blood-borne fibrocytes and its involvement in scleroderma pathophysiology.

Methods: Bloods were collected from 10 diffused cutaneous SSc (dSSC) patients [ACR, 2013] and 10 age-sex matched healthy controls. Characterization of fibrocytes was identified with specific antibodies by FACS.

Results: Two subpopulation of: CD14high and CD14Low have been found within the CD45+ cells. The percentage of these two subpopulations are inversely represented in scleroderma patients (CD14high: 12 % and CD14Low: 84%) with respect to healthy control (CD14high: 80% and CD14Low: 20%). The expression of Col-I, αSMA and CD90 are significantly higher (p= 0.0004, p=0.001, p=0.02 respectively) in the CD14high population of patients than controls. Patients have an increased expression of Col-I, αSMA and CD90 (p< 0.0001, p=0.006, p=0.001 respectively) in the CD14high population compared to the CD14Low population. There is a clear indication that the patients have higher amount of CD90 expressing cells among the total CD14 population than healthy controls. Percent of CD14high cell population is not correlated (r2 =0.09, p=0.05) with the disease duration, rather showing a positive significant correlation with mRDSS (r2 =0.2, p=0.02).

Discussion: patients' having altered expression of CD14+ cells with increased number of CD45+ / CD14high population which significantly express high amount of Col1.The increased number of CD90+/αSMA+ population of cells in patients showing the involvement of activated circulating fibroblasts that might be a potential inducer of uncontrolled fibrosis in Scleroderma. The higher expression of Col-1 and activation markers of fibroblasts by CD45+ / CD14high subpopulation indicating that this predominant subpopulation in patients might acts as a potent contributor of fibrosis.


  OB0009: Local, organ-specific mechanisms may explain the heterogeneity of SLE Top


Ram Raj Singh1, 2, 3, 4, Anna U Eriksson1, Peter J Kim1, Darshan Randhawa1, Rachael L Philips1, Jennifer King1, Miguel-Angel Gutierrez1,2; 1Department of Medicine, Division of Rheumatology, Autoimmunity and Tolerance Laboratory,2Molecular, Cellular, and Integrative Physiology Graduate Program,3Molecular Toxicology Interdepartmental Program,4Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles, California, USA

Background: Current treatments for lupus involve suppressing systemic immunity, thus increasing the risk of adverse effects. Identifying organ-specific mechanisms and targets may lead to organ-specific treatments. Skin harbors specialized subsets of dendritic cells (DC), including Langerhans cells (LC) in the epidermis, and LangdDC in the dermis. These DCs take antigens from skin and deliver them to skin-draining lymph nodes in order to induce immunity (in case of skin infection) or tolerance (to avoid autoimmunity against skin). We previously reported that the migration of LC is impaired in lupus (Eriksson and Singh, J Immunol 2008), and that this defective migration played a role in the loss of tolerance to skin antigens in lupus (King J…Singh RR, J Immunol 2015). Skin also harbors specialized T-cells called dendritic epidermal γδ T-cells (DETC). Here, we investigated the interplay between LCs and DETCs in the pathogenesis of lupus dermatitis using the MRL model.

Methods: To be able to track and selectively deplete skin-DC in vivo, we generated langerin-driven eGFP gene knock-in and diphtheria toxin receptor knock-in mice, respectively, in genetically lupus-prone MRL model.

Results: DETCs directly modulated LC migration via direct cell-to-cell interaction. Lupus-prone mice had reduced DETCs and reduced LC-migration; both these defects were corrected by glycolipid-treatment that ameliorates lupus dermatitis. Finally, selective depletion of LC accelerated cutaneous lupus. LC-depletion or glycolipid-treatment did not affect anti-DNA antibodies or lupus nephritis.

Conclusion: Skin-DETCs regulate the migration of skin-DCs, which is impaired in lupus leading to autoimmune dermatitis. Glycolipid treatment that corrects this defect improves lupus dermatitis but does not affect disease in other organs.

Implications: We identified a novel mechanism whereby interplay between tissue-resident specialized immune cells regulates local immunity in an organ. Such specialized local regulation of autoimmunity at the tissue level can be targeted to develop tissue-specific treatment without affecting systemic immunity.


  OB0010: Effect of curcumin on pro-inflammatory cytokines in primary sjögren's syndrome Top


Jayakanthan Kabeerdoss, Pulukkol Sandhya, M Hindhumathi, Debashish Danda; Department of Clinical Immunology and Rheumatology, Christian Medical College, Vellore, Tamil Nadu, India

Introduction: Curcumin reduces disease severity and ameliorates lupus-like/ Sjögren's Syndrome-like disease in mice model. The immunological basis of these effects is unknown. This study examined the effect of curcumin on pro-inflammatory cytokines secreted by salivary gland in patients with primary Sjögren's syndrome (pSS).

Methods: Minor salivary gland (MSG) tissue was collected after obtaining written consent from patients undergoing biopsy as a part of evaluation for suspected pSS . The tissue was treated with phytohemagglutinin (PHA) alone as well as PHA with curcumin (30μM) and cultured in RPMI 1640 medium for 48 hours at 37°C in CO2 incubator. After the incubation period, culture supernatant and tissue were stored in the ultra-deep freezer(-80 °C). IL-6 levels were measured in supernatant by commercially available ELISA kits. RNA was extracted from tissue using Tri reagent method. Expression of pro-inflammatory cytokines, IL-6, IL-8, TNF-α, IL-1β, IL-4, IL-21, IL10 and IFN-γ was done by qPCR. Differences between groups were tested by Student's t-test.

Results: Forty-seven patients were recruited. Eight patients satisfied ACR/EULAR criteria for pSS. Seven patients with absent glandular inflammation and negative serologies constituted controls. In pSS group, but not in controls, median IL-6 levels in supernatant was less in curcumin treated as compared to PHA alone (5.5 (0.7 -1.3) vs 18.3 (12-32) ng/ml;p=0.04). mRNA expression levels of IL-6 and IL-1β were lower in curcumin treated groups as compared to PHA alone in both cases and controls (p= 0.0009 & p=0.04 respectively). There was no difference in other cytokine levels between the treatment groups. mRNA expression levels of IL-4, IL-21 and IL10 were below detectable range.

Conclusion: Curcumin reduces secretion of IL-6 levels in salivary gland tissue of patients with pSS. Curcumin suppressed PHA induced mRNA expression levels of IL-6 and IL-1β in MSG tissue of pSS and sicca controls.


  OB0011: NMR-based serum, urine and muscle metabolomics in inflammatory myositis for diagnosis and activity assessment: Serum metabolomics can differentiate active from inactive myositis Top


Latika Gupta, Dinesh Kumar1, Umesh Kumar1, Anupam Guleria1, Ritu Raj1, Ramnath Misra*; Department of Clinical Immunology,1Centre of Biomedical Research, SGPGIMS, Lucknow, Uttar Pradesh, India

Objective: To identify changes in metabolomics profiles in serum, urine and muscle of patients with myositis with active and inactive disease.

Methods: Sera (n=116), urine (n=114) and muscle (n=11) from patients classifiable as myositis by the ACR-EULAR criteria [34 years (23.5 - 50.5 IQR), M/F 28:88] were compared with healthy controls [n=18 and 12 respectively, age= 44 (35-50) years, M/F-8:10]. For the muscle biopsies, two disease controls were used for comparison. To study effect of fasting state on urine metabolomics, 50 paired urine samples obtained in fasting and non-fasting states were analyzed.

Metabolic profiles were obtained at 800 MHZ NMR spectrometer and compared using multivariate partial least-squares discriminant analysis (PLS-DA). The discriminatory metabolites were identified based on variable importance in projection (VIP) statistics (p-value <0.05). Paired T tests were done for metabolites in urine and muscle after normalizing for creatinine. MDAAT>=1 was used to define active myositis.

Results: and discussion: Metabolomics profiles in IIM were distinct from healthy controls [Figure 1]a.Patients with active myositis exhibited differential clustering from those with inactive myositis [Figure 1]b with lower amino-acid metabolites [Figure 1]c elevated phenylalanine-to-tyrosine ratio and glutamate to glutamine ratio [Figure 1]d and low creatinine [Figure 1]e with significant discriminatory potential [Figure 1]f.
Figure 1: Serum metabolomics (a) three dimensional score plot obtained from PCA analysis of 1D 1H CPMG NMR based serum metabolic profiles showing clustering and separation between healthy control and inflammatory myositis patients (b) two dimensional score plot after PCA analysis of sera from myositis patients with active and inactive disease (c) screening of metabolites responsible form the group separation in the score plot identified based on variable importance in projection score > 2.0 (d) variable importance in projection projection suggesting elevated phenylalanine-to-tyrosine ratio (PTR) and glutamate to glutamine ration (EQR) (e) variable importance in projection projection suggesting elevated significantly decreased in the sera of active myositis patients (f) representative ROC curves of discriminatory metabolites

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Urine in IIM exhibited higher creatine and lower urea and creatine than healthy controls [Figure 2]a and [Figure 2]b. However, urine metabolomics exhibited similar clustering in active and inactive disease [Figure 2]c. In urine, paired samples in fasting and non-fasting state exhibited overlapping clustering [Figure 2]d apart from higher creatine and creatinine [Figure 2]e.
Figure 2: Urine metabolomics (a) three dimensional score plot obtained from PCA analysis of 1D 1H CPMG NMR based urine metabolic profiles showing clustering and separation between healthy control and inflammatory myositis patients (b) variable importance in projection projection suggesting elevated creatinine, and low urea and creatine in myositis versus healthy controls. (c) PCA suggests no differential clustering between active and inactive myositis. (d) In urine, paired samples obtained in fasting versus nonfasting state were fairly similar, (e) apart from differences in creatine and creatinine

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Muscle in IIM exhibited almost absent levels of sucrose, mannose, uridine, histamine, inosine and carnitine compared with infection associated myositis.

Conclusion: Serum metabolomic profiling using NMR has the potential to discriminate active from inactive myositis patients. Muscle metabolites holds potential to distinguish inflammatory myositis from infectious polymyositis.


  OB0012: Peripheral T helper subset profiling is different in various subsets of idiopathic inflammatory myositis Top


Anamika Kumari Anuja, Harshit Singh, Durga P Misra, Vikas Agarwal, Latika Gupta; Departemnt of Clinical Immunology, SGPGI, Lucknow, Uttar Pradesh, India

Introduction: There is dearth of biomarkers in Idiopathic Inflammatory Myositis(IIM) to identify ongoing inflammation in the muscle and distinguish it from inactivity or damage.

Objective: Since myositis is autoantibody mediated and tertiary lymphoid organogenesis(TLO) reported in the diseased muscles, we looked for peripheral blood T helper subset profiling as a reflection of ongoing muscle inflammation.

Methods: Twenty-six patients of IIM(ACR EULAR criteria) were compared with 15 healthy controls(HC) and 21 patients with sarcoidosis. Peripheral blood mononuclear cells were stained with combinations of antibodies to identify Th1, Th17, Th17.1 and Treg cells after stimulation assays (BD Biosciences). Myositis Specific and Associated autoantibodies were tested by the line immunoassay(Euroimmune, Germany).

Results: All T helper subsets were higher in myositis as compared with healthy controls [Figure 1]a, [Figure 1]B, [Figure 1]C, [Figure 1]d. Between various IIM subsets, polymyositis had higher Th1 and Treg cells [Figure 2]b and [Figure 2]c while Th17 and Th17.1 cells(c) were higher in Overlap Myositis [Figure 2]a and [Figure 2]d as compared with healthy controls. Patients with sarcoidosis had similar subset profiling as myositis [Figure 5]a, [Figure 5]b, [Figure 5]c, [Figure 5]d, [Figure 5]e, [Figure 5]f.
Figure 1

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Figure 2

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Figure 5

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Patients who were had either arthritis or were positive for myositis specific autoantibodies had higher Th17.1 cells [Figure 3]a(iii) and [Figure 3]b(iii) than those negative for MSA. There was no difference in T cell profile between autoantibody subsets [Figure 6]a, [Figure 6]b, [Figure 6]c, [Figure 6]d.
Figure 6

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There was no difference in subsets between active and inactive disease although active disease had lower Th1/Treg, Th17/Treg and Th17.1/Treg ratios.

Conclusion: T Helper cell subsets are distinct from HC but similar to sarcoidosis patients. However, they differ in various types of myositis, suggesting that possibly T cell populations(Th1, Th17) might be migrating to muscle tissue, hence, lesser in peripheral blood. Autoantibody positivity is associated with elevated Th17.1 population suggesting plasticity in TLO which needs to be explored further. However,T cell profiling cannot distinguish active from inactive disease limited predictive potential as a biomarker.


  OC0001: Comparison of methotrexate and glucosamine in primary knee OA Top


Biswadip Ghosh, Subhankar Hadar, Meghna Saha; Department of Rheumatology, IPGME and R and SSKM Hospital, Kolkata, West Bengal, India

Background: Osteoarthritis (OA) is most common rheumatologic disease but it attracts little attention in comparison from rheumatologists. Many patients with osteoarthritis show some features of inflammation, though the traditional thinking is that, OA is a degenerative disease.

Objective: To compare effect of methotrexate and glucosamine in primary Inflammatory knee OA.

Methods: 344 patients (40-70 years of age) of primary knee OA were examined. 249 patients with local clinical inflammation were recruited, subdivided in systemic Non-Inflammatory group (77) and Inflammatory group (172) based on serum levels of ESR and CRP. Those 172 patients of inflammatory group were randomly allocated to Methotrexate (15-20 mg/wk) or Glucosamine (1500mg/day) group and observed for three months. WOMAC was calculated before and after treatment. 137 patients completed the study of which 59 patients received glucosamine and 78 patients received methotrexate. MRI of knee (3 tesla) was done in every patient.

Results and Analysis: No significant difference in WOMAC, ESR or CRP was found in glucosamine group after three months. Methotrexate produced significant changes in WOMAC, ESR and CRP after three months in primary knee OA patients. There is decrease in swelling of knees in nearly all patients and improvement in pain in most of them after three months of taking methotrexate.

Conclusion: Large number of patients of primary knee OA show signs of local inflammation. Many of them have raised acute phase reactants like ESR and CRP, signifying systemic inflammation. These patients may be benefitted with methotrexate administration.

Funding: Department of Science and technology, Government of West Bengal, India.


  OC0002: Utility of testing conventional and non-conventional anti-phospholipid antibodies in suspected obstetric anti-phospholipid syndrome Top


G Arvind, K Jayakanthan, Aswin Nair, Ashsih J Mathew, Ruchika Goel, John Mathew, John Anthony Jude Prakash1, Sukesh Nair2, Debashish Danda; Departments of Clinical Immunology and Rheumatology,1Microbiology and2Immuno-haematology and Transfusion Medicine, CMC, Vellore, Tamil Nadu, India

Background: Anti-phospholipid syndrome (APS) is an important cause for recurrent pregnancy losses (RPL). Conventional APS antibodies (aPLs) like lupus anti-coagulant (LA), anti-cardiolipin( ACL) and anti-beta 2 glycoprotein I ( anti-β2 GP I) are not present in significant number of obstetric APS(OAPS) patients, leading to a state described as “ sero-negative” OAPS ( SNOAPS). Recent literature shows non-conventional aPLs like Anti phosphatidylserine-prothrombin complex (Anti-PSPT) and Anti-Annexin V (Anti-Ann V) can be positive in up to 50% of SNOAPS patients.

Objectives: Testing performance of conventional and non-conventional aPLs in suspected OAPS patients (obstetric events as defined in the Sydney classification criteria for APS)

Methods: We performed a retrospective chart review of 84 patients who underwent combined testing for non-conventional aPLs for suspected OAPS from May 2015 to August 2019 at our department. Patients were categorized into OAPS cases (n=42, median age 31 years) and controls (n=42, median age 31.5 years) based on their fulfilment of clinical definition of OAPS events defined by Sydney criteria. Conventional aPLs were tested by Methods: adapted in Sydney criteria and Anti PSPT /Anti Ann V were tested by commercial ELISA.

Results: 31 cases (73.8%) were 'sero-positive' & 11 cases (26.2%) were truly 'sero-negative' for conventional aPLs. Four (36.4%) of the SNOAPS patients were positive for Ant-PSPT and/or Anti AnnV antibodies. Performance of the various aPLs in suspected OAPS is displayed in [Table 1].
Table 1: The performance of the various conventional and nonconventional anti-phospholipid syndrome antibodies in suspected obstetric anti-phospholipid syndrome cases

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Conclusion: In a delicate situation like RPL, performance of non-conventional aPLs on their own, though not as sensitive as conventional aPLs, still demonstrate better specificity. The real value of testing Anti PSPT & Anti Ann V in RPL, is combined testing with conventional aPLs wherein they improve the sensitivity and accuracy of diagnosis of OAPS by 10% & 3.5% with only 2.4% drop in specificity. Non-conventional aPLs should be tested in SNOAPS.




  OC0003: Demographic, clinical, laboratory and genetic studies of patients with Palindromic rheumatism: a single center experience Top


Padmanabha Shenoy, Sanjana Joseph, Sreelakshmi Sreenath, Ansu Abu Alex, Sageer Babu, Manjesh V Pai; Care, Centre for Arthritis and Rheumatism Excellence, Cochin, Kerala, India

Introduction: Palindromic rheumatism (PR) is characterized by multiple, episodic and recurrent attacks of arthritis, without residual joint damage occurring at irregular intervals.

Aim: To do a comprehensive demographic, clinical laboratory and genetic studies on patients diagnosed with PR.

Patients and Methods: 350 patients diagnosed with PR based on PR classification criteria from 2014-2018 were included in the study. Whole exome sequencing was done in PR and RA cohorts with the help of Medgenome.

Results: Most of the patients were under the age of 49yrs (n=183) and had female predominance of 72% (n=255). 83 patients showed disease symptoms for greater than 10 years. The major symptoms were redness (54% of patients) and swelling (40% cases). 47% of patients showed Rheumatoid factor positivity (n=334), 60% were ACPA positive (n=328) and 137 cases were both positive. Majority of the patients were on hydroxychloroquine (n=322) and colchicine (n=302) . Prednisolone was given in 83 patients who was tapered from average dose of 3.5mg to 1.84mg and stopped in 18 patients. Methotrexate, Sulfasalazine and leflunomide were also used as second line agents.

Whole exome sequencing was done to find variants that are associated with prevention of persistent inflammation in PR patients (protective variants) in contrast to RA patients (n=10 each).The frequency of IL10RA, IL10RB and HLADRB1 were more in PR. And there were 18 alleles unique to PR and 20 alleles unique to RA and 7 in common. Detailed analysis has to be done further to elucidate the mechanisms unique to PR.

Conclusion: This study is a large case series of patients with PR from a single center looking at comprehensive demographic, clinical and laboratory parameters. More studies on genes involved in inflammation and immune response has to be studied further to identify the molecular and genetic mechanisms in PR.


  OC0004: Assessing the risk of maculopathy in Indian patients using hydroxychloroquine for rheumatic complaints: A cross sectional observational study Top


Arindam Nandy Roy, Yarram Ashok Kumar, Syeda Sana Fatima; Department of Rheumatology, Yashoda Hospital, Secunderabad, Telangana, India

Background: Hydroxychloroquine (HCQ) has shown benefits in treating rheumatic diseases such as SLE , RA and scleroderma. The risk of developing irreversible maculopathy and consequent vision loss is a possible serious complication with HCQ use. Modern day screening Methods: can detect retinopathy early in such patients.

Objective: Primary aim was to assess the prevalence of HCQ maculopathy in Indian patients with rheumatic diseases by modern day screening Methods: Secondary aim was to look for the risk factors of HCQ maculopathy.

Methods: This cross-sectional study was carried out in the Dept of Rheumatology,Yashoda Hospital, Secunderabad between July 2017 to March 2019. 984 subjects having different rheumatic diseases, who had used HCQ for 1 year and beyond and evaluated with at least Humphrey Visual Fields were included. Retinopathies other than HCQ were excluded. Informed consent was taken.

All data regarding Age, Gender, BMI, Diagnosis, Comorbidities, Daily dosage and Duration of HCQ use and Screening Methods: (Humphrey Visual Fields, Spectral Domain Ocular Coherence Tomography, Fundus Auto Fluorescence, Multifocal Electroretinogram, Fundus Fluorescein Angiography) were noted.

Chi Square test was used for statistical analysis and p<0.05 was considered significant.

Results: Maculopathy was found in 13.5% patients on HCQ beyond 1 year No statistical association was seen between HCQ maculopathy and Gender (p= 0.189), BMI (p=0.289), diagnosis (p=0.865), Comorbidities and daily dosage of HCQ (p=0.171).

However, a significant correlation was found between HCQ maculopathy and Age {8.4 %< 30 years VS 20.3%>60 years (p=0.033)}, Weight {8.7 %<50kg VS 15.1%>60kg(p=0.045)}, HCQ duration {11.7%<5 years VS 21.1%>5 years (p=0.002)} and Cumulative dose {283.79 g VS 231.33 g (p=0.006)}.

The detection rates of maculopathy by different Methods: were HVF (13.5%), SDOCT (13.3%), mfERG (12.8%), FAF (12.1%), FFA (11.8%), HVF+SDOCT (13.3%), HVF+mfERG (12.8%) and HVF+FAF (12.1%)

Conclusion: Hydroxychloroquine maculopathy is not infrequently seen in rheumatic disease patients in India.


  OC0005: Infliximab usage in kawasaki disease: Sharing an experience over 3.5 years Top


Priyankar Pal, Mandira Roy, Jigna Bathia, Alolika Mondal, Mallar Mukherjee, Anil Kr Singhi; Pediatric Rheumatology and Cardiology Unit, Institute of Child Health, Kolkata, West Bengal, India

Introduction: Tumor Necrosis Factor (TNF-α) blocker Infliximab (IFX) is emerging as an important drug in management of Kawasaki Disease (KD). This study was undertaken to evaluate the effectiveness of Infliximab in severe KD on 33 children over a period of 3.5 years.

Objectives:

1. Determine effectiveness in IVIg resistant KD.

2. Evaluate it's response if any on developing/ increasing coronary artery aneurysms (CAAs).

Methodology: Study was carried out in IPD of Rheumatology Unit of Institute of Child Health, Kolkata. 33 children aged between 6 weeks to 7 years with KD were included who received IFX after 1-2 doses of IVIG due to persistent fever/ increasing CAA or developing new CAA. Patients were analyzed for response in terms of achievement of defervescence in days, normalization of CRP and improvement in echocardiographical findings specially CAAs.

Results: IFX was used in 1) IVIG resistant fever(18/33) 2) increasing CAAs post IVIg) (10/33). Overlapping indications ie. IVIG resistance with increasing CAAs was also present (7/33). 3 patients received IVIG twice before receiving Infliximab. IFX was given at 5mg/kg and defervescence was achieved within 24 hours of administration in 23 of the 25 resistant cases along with normalization/ drastic fall in CRP. 2 children whose fever persisted were later diagnosed as SOJIA.

17 children with severe CAAs were followed up by echocardiography weekly till 6 weeks, monthly till 3 months, and then at 6 months and 1year.15 of them had significant reduction in size, giant aneurysms being converted to medium or small sized aneurysms over 6 to 18 months.

Conclusion: Infliximab showed 100% success in IVIG resistant patients. Its beneficial effect on the CAAs seems promising, but further follow-up is required for validation.


  OC0006: Short and long-term outcome of immunosuppressive therapy among Indian juvenile lupus nephritis patients Top


Rudra Prosad Goswami, Parasar Ghosh; Department of Rheumatology, IPGME and R, Kolkata, West Bengal, India

Background: Juvenile-onset systemic lupus erythematosus (JSLE) is more severe than adult-onset disease, with higher proportion of lupus nephritis (LN). There is a paucity of long-term outcome data of LN in JSLE from India.

Objective: To describe the short and long-term renal response and flare rates in JSLE LN patients.

Methods: Single centre longitudinal observational study including JSLE LN patients (fulfilling ≥4 American College of Rheumatology criteria, age of onset ≤16 years, biopsy proven LN (classes III, IV or V)) receiving induction with intravenous cyclophosphamide (CyC, n=25, median dose 5400mg, inter-quartile range (IQR): 5000-5400) or mycophenolate mofetil (MMF, n=19, median dose 2gm/day, IQR: 2-2.63) and maintenance azathioprine (n=19, 75mg/day, IQR: 75-100) or MMF (n=21, 1.5gm/day, IQR 1.5-2).

Results: We analysed the Results: of 44 JSLE LN patients [short-term and long-term renal responses in [Figure 1]. Induction failure-rate was 9.1% (4/44, 95% confidence interval (CI): 3.1-21.7). They were treated with either cyclophosphamide (n=1, failed with MMF) or rituximab (n=3, failed with cyclophosphamide). Maintenance failure-rate was 25% (10/40, 95%-CI: 14.2-40.2). Among maintenance failure, 7/10 were early (occurred ≤2 years of FU). Time to maintenance failure was 24 months (IQR: 24-42). In univariate analysis early maintenance failure was associated with lower prednisolone dose in the second year (3.6±1.8 mg/day vs 5.9 ± 3.9 mg/day, p=0.049); prednisolone ≤ 5mg/day from 1st year FU (66.7%, 2/3 vs 13.5%, 5/37, p=0.02) and prednisolone ≤ 5mg/day from 2nd year FU (37.5%, 3/8 vs 10%, 3/30, p=0.05). Patients receiving MMF maintenance experienced numerically higher early maintenance failure (23.8%, 5/21 vs 10.5%, 2/19, p=0.27). In multivariate logistic regression receipt of prednisolone ≤5mg from first year of FU predicted early maintenance failure (Odd's ratio 15.5, 95%-CI:1.13-212, p=0.04).
Figure 1: Renal responses and flare rates of juvenile lupus nephritis patients over 5 years

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Conclusion: Both short-term and long-term outcomes of JSLE LN were good. Majority of renal flares were early and linked to premature prednisolone dose reduction.


  OC0007: Low dose prednisolone plus tacrolimus therapy is more effective in Pla2r-Ve membranous glomerulonephritis patients Top


Akhilesh Jaiswal, Narayan Prasad, Vikas Agarwal, Vinita Agrawal, Harshit Singh; Department of1Nephrology,2Clinical Immunology and3Pathology, SGPGIMS, Lucknow, Uttar Pradesh, India

Background: Idiopathic Membranous Nephropathy (IMN); an autoimmune concomitant nephrotic syndrome of the adult is mainly associated with PLA2R antibody expressed on podocytes. The lack of exact mechanisms involved in the pathogenesis of IMN is transmitted to its therapeutic management. We propose the efficacy of low dose Tacrolimus (Tac) plus prednisolone and associated changes in anti-PLA2R in adult IMN.

Methods: Total 101 membranous nephropathy patients were treated with combination of prednisolone 1mg/kg alt-day) and Tac 0.1mg/kg/day (trough 6-10 ng/ml first 6M and 4-6 ng/ml for next 3M) then both taper by 1/3 every month up to 12M. Out of 101 patients; 15 diabetic; 7 lupus; 1HBV and 1 ankylosing spondylitis patients were excluded. Finally; 77 Patients were followed and evaluated for the anti-PLA2R level at baseline; 3M; 6M; 12M and end of follow-up (17-61; median 38 months). CR; PR; relapse; and side-effects were recorded. Of the 77 patients; at 3M 60(77.92%; CR-37; PR-23); at 6M 61(79.22%; CR-53; PR-8); at 12M 53(68.86%; CR-47; PR-6) achieved remission. At end of follow-up; out of 54 responsive patients 37(68.51%; CR-36; PR-1) remained in remission and 17(31.48%) patients relapsed.

Results: Out of 77 patients; 51 (66.3%) were anti-PLA2R positive. Remission rate was significantly low in PLA2R+ve than PLA2R-ve (36/51 vs 24/26; p=0.03) at 3M; (36/51 vs 25/26; p=0.009) at 6M and (31/51 vs 22/26; p=0.03) at 12M. There were significant correlations between PLA2R level and 24h proteinuria at baseline; 3M and at 6M. During therapy 4 patients develop cutaneous tenia; 1 osteonecrosis of the femur head; 1 corpus tunnel syndrome; 4 onset diabetes; 3 tremor; and 14 patients experienced GI symptoms. To note; 4 females had pregnancy and successful delivery in our cohort of patients.

Conclusion: S PLA2R+ve patients showed poor response compare to PLA2R-ve patients. Remission with Tacrolimus and prednisolone therapy is comparable to historical Ponticelli regimen


  OC0008: Prevalence of musculoskeletal pain and its risk factors among adolescent school children: A cross sectional survey Top


Maumita Kanjilal, Uma Kumar, Ravi Kant Arya1, Gajendra Kumar Gupta1, Deepika Agrawal1; Department of Rheumatology, AIIMS, New Delhi,1Department of Community Medicine, Santosh University, Ghaziabad, Uttar Pradesh, India

Background: The lack of physical activity, sedentary behaviour and increase in the use of visual display unit is an alarming concern among adolescent school children which is leading to increased musculoskeletal pain.

Objectives: The aim of the study is to find prevalence of musculoskeletal pain and its association with duration of physical activity and the screen time spent on smart phone and TV in adolescent school children.

Methods: The cross-sectional study was conducted at Government schools in Delhi. Children in the age group 10-19 years without any known musculoskeletal disorders or any disease participated in the study. The demographic details, Nordic Muscular Questionnaire, the level of sedentary lifestyle and physical activity were assessed through the questionnaire consisting of the time spent on smart phone/TV and International physical activity questionnaire.

Results: 1600 children participated in the study with 855 (53 %) boys and 745 (47 %) girls in the age group 10-19 years participated in the study. The prevalence of musculoskeletal pain was found to be 63% (95% CI :55-70) in past 12 months and recent 7 days period as per Nordic musculoskeletal Questionnaire. The data analysis (χ2) suggested that musculoskeletal pain was significantly associated with female gender (p=<0.0001), late adolescence age (p=<0.0001), duration of physical activity less than 60 minutes per day (p=<0.0001), duration of watching TV (p=0.033) and smart phone usage(p=<0.0001) more than 2 hours per day.

Conclusion: 63% adolescents had musculoskeletal pain. Use of mobile phone/watching TV more than the recommended screen time duration of more than 2 hours per day, moderate physical activity of less than 60 mins per day among adolescent is a major risk factor for the development of musculoskeletal pain. Significant association of pain with late adolescent and girls further endorses this observation.


  OC0009: Assessment of liver fibrosis by transient elastography in patients with rheumatoid arthritis on long-term methotrexate therapy Top


Prashant Bafna, Anupam Wakhlu, Sumit Rungta1, Puneet Kumar, Urmila Dhakad; Departments of Clinical Immunology and Rheumatology and1Medical Gastroenterology, King George's Medical University, Lucknow, Uttar Pradesh, India

Background: Although methotrexate (MTX) is widely available effective DMARD in rheumatoid arthritis (RA), long term therapy may be associated with increased risk of liver fibrosis. A noninvasive method like transient elastography (TE) is used for assessing hepatic fibrosis, as monitoring with liver enzymes and albumin alone are unreliable for assessing liver dysfunction in liver fibrosis.

Objectives: Primary: To study the prevalence of liver fibrosis in patients with long term methotrexate therapy for RA

Secondary: To correlate duration and cumulative dose of methotrexate use with the risk of developing liver fibrosis

Methods: In this cross-sectional study, 40 patients fulfilling the 2010 ACR/EULAR criteria of RA on methotrexate therapy for > 3years were assessed. Ethical approval was obtained. The median duration and cumulative dose of MTX use were calculated from their previous records. Hepatic stiffness by TE method (by FibroScan) was determined for the included patients. The duration and cumulative dose of MTX with liver fibrosis was correlated using Spearman correlation formula.

Results: The mean age of patients was 49±9.4years. There were 37 females and 3 males. The median duration of MTX use was 336weeks (IQR 240-432 weeks). Nine(22.5%) patients were on MTX for >10years. The median cumulative dose of MTX was 5.6gm (IQR 4.6gm-7.3gm). Thirty(75%) patients had a cumulative dose between 5 to 10 gm and 4(10%) had >10gm. Mean hepatic stiffness was 5.26kPa±2.16kPa. Six(15%) patients had liver stiffness between 7.5 to 9.5kPa. Two(5%) patients had liver stiffness of 11.6kPa and 12.2kPa with their corresponding cumulative dose of 7.6gm and 13.8gm respectively. Their stiffness improved to < 7.5kPa by 6 months after discontinuation of methotrexate. No correlation was found between the dose and duration of methotrexate use with the liver fibrosis (p=0.08, r=0.06).

Conclusion: Methotrexate, when conventionally prescribed in low-dose weekly schedule is possibly safe for long-term use in rheumatoid arthritis


  OC0010: Cardiovascular risk assessment in Indian rheumatoid arthritis patients: Comparison of risk algorithms and carotid intima media thickness Top


Hafis Muhammed, Durga P Misra, Sujata Ganguly, Sarit Sekhar Pattanaik, Saurabh Chaturvedi, Harshit Singh, Mohit K Rai, Anamika Anuja, Namita Mohindra1, Neeraj Jain1, Sudeep Kumar2, Vikas Agarwal; Departments of Clinical Immunology and Rheumatology,1Radiodiagnosis and2Cardiology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Rheumatoid arthritis(RA) patients have increased cardiovascular(CV) risk with no data on CV risk scores in Indian patients. We aimed at studying CV risk in RA patients.

Methods: Patients fulfilling 2010 ACR/EULAR criteria for RA were included. Presence of CV risk factors were recorded. 10-year-CV risk was predicted using Framingham Risk scoring using lipids(FRS-Lipids), Framingham Risk scoring using body mass index(FRS- BMI), QRISK-2, SCORE and the algorithm recommended by ACC/AHA (ASCVD) in patient who were 40 years or older. Carotid Intima Media Thickness(CIMT) was measured on far-wall of the common carotid artery. Subclinical atherosclerosis was defined as CIMT >0.90 mm or presence of plaque.

Results: 334 patients (M: F = 49:285, Mean age =47.16±12.57 years) were enrolled. 6% were smokers, 12% had diabetes mellitus(DM) and 21% had hypertension. Mean CIMT was 0.70±0.15 mm. In univariate analysis mean CIMT significantly differed according to gender and presence or absence of erosions, extra articular manifestation (EAM), DM and hypertension. CIMT correlated significantly with age, disease duration, systolic blood pressure and total cholesterol. Multiple regression analysis showed age, EAM and male gender as independent predictors of CIMT (r2=0.431 for final regression model). All risk scores had moderate correlation with CIMT with maximum for QRISK (r=0.570). Percentage of patients with predicted >10% risk varied from17.6% to 41.9% between scores. Agreement between scores in predicting risk was moderate in general with maximum agreement between QRISK2 and FRS-Lipids (weighted kappa:0.790). ASCVD and QRISK-2 showed maximum sensitivity when subclinical atherosclerosis assessed by CIMT was taken as standard (AUC: 0.822 and 0.806 respectively) [Figure 1].
Figure 1: ROC curve

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Conclusions: Prevalence of risk factors and subclinical atherosclerosis were calculated. Age, EAM and male gender independently predicted CIMT. While further validation is required in terms of clinical end points, our findings suggest that risk algorithms cannot be used indiscriminately in RA.


  OC0011: Subclinical atherosclerosis in Indian patients with spondyloarthropathy: Clinical and serological associations Top


Mohit Kumar Rai, Mantabya Singh, Harshit Singh, Saurabh Chaturvedi, Anamika Anuja, Kritika Singh, Namita Mohindra, Neeraj Jain, Sudeep Kumar, Vikas Agarwal, Durga Prasanna Misra; Departments of Clinical Immunology and Rheumatology, Nephrology, Radiodiagnosis and Cardiology, SGPGIMS, Lucknow, Uttar Pradesh, India

Background:

We evaluated traditional and novel risk factors associated with subclinical atherosclerosis (SCA) in spondyloarthritis, in a cross-sectional study.

Objectives:

  • To compare mean carotid intima-medial thickness (CIMT) between patients with spondyloarthropathy (n=105) fulfilling ASAS 2010 criteria, and healthy controls (n=55).
  • To compare traditional and novel biomarkers (microparticles, endothelial microparticles, inflammatory cytokines associated with increased cardiovascular risk, serum osteoprotegerin, serum apolipoprotein A1) between patients with spondyloarthritis and healthy controls.
  • To identify factors associated with SCA in spondyloarthropathy.


Methods: SCA was defined by carotid artery plaques, or increased CIMT >2 standard deviations compared with Indian reference standards for age and sex. Total microparticles (TMP) were measured in plasma after ultracentrifugation using microbeads of 7 mm size (TMP were of size 0.1-1 mm); of these, microparticles positive for CD31 and CD146 were taken as endothelial microparticles (EMP). Serum cytokines (IL-1b, IL-6, TNF-a, IL-17, IL-27, IL-33), osteoprotegerin and apolipoprotein A1 were measured by ELISA using manufacturer instructions. Univariate analysis compared patients with spondyloarthropathy with healthy controls. Linear regression was used to identify the determinants of CIMT in spondyloarthropathy. Binomial logistic regression was used to identify factors associated with SCA in spondyloarthopathy.

Results: Despite lower BMI, total cholesterol, triglycerides and higher HDL cholesterol, CIMT was significantly higher in patients with spondyloarthropathy [Table 1]; on linear regression, body mass index (BMI, p=0.026), total cholesterol (p=0.031), endothelial microparticles (0.006), serum IL-1b (p=0.001) and IL-27 (p=0.040) were independent predictors of CIMT in spondyloarthropathy. Fourty- two (40%) patients had SCA; 15 (14.3%) had carotid plaques. Spondyloarthropathy patients with SCA had higher age, disease duration, BMI, waist-hip ratio, LDL cholesterol and serum IL-1b; binomial logistic regression identified independent association of age and LDL cholesterol with SCA.
Table 1: Comparison between patients with spondyloarthritis and healthy controls

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Conclusions: Both traditional and novel cardiovascular risk factors (endothelial microparticles, IL-1b, IL-27) associate with high prevalence of SCA in Indian spondyloarthropathy patients.




  OC0012: Scoring system based on inflammatory parameters to screen for infection and disease flare and bacterial versus viral infections in the background of inflammatory rheumatic disease Top


S Chandrashekara, P Renuka, K R Anupama, Devaraj, J Prakruti, R Veena; ChanRe Rheumatology and Immunology Centre and Research,1ChanRe Diagnostic Centre, Bengaluru, Karnataka, India

Background: In patients with autoimmune rheumatic disease, differentiating infections (viral or bacterial) from disease exacerbation is a challenge. The leucocyte response, CRP, NLR and pro-calcitonin behave differently and could be used to differentiate them.

Objective: To develop and validate two clinical risk scores for the screening of infections and disease flare and in infection to distinguish bacterial or viral in AIRD patients.

Materials and Methods: The retrospective cohort study included 115 consecutive AIRD patients (January-December 2018) for derivation and 90 patients (January-July 2019) for internal validation. The clinical risk score was derived by assigning points based on multivariate logistic regression co-efficient. The points were summed to derive the risk score for each patient.

Results: Of the 115 patients, 77 (66.96%) had infections and 38 (33.04%) had disease flare. Corresponding bacterial and viral infections noted was 44 (57.14%) and 33 (42.86%). The final infection and flare risk score included total leucocyte count, NLR, CRP and procalcitonin. Bacterial vs viral infection risk score included CRP, NLR and procalcitonin. ROC curve obtained for both models showed fair discrimination (AUC= 0.7). The categorization of patients for infection vs. flare risk score was as follows: >9 high probability of infection, 5 to 9 moderate possibility of infection present and <5 infection less likely. Patients with bacterial vs. viral risk were scored as: >8 risk of bacterial infection, 5 to 8 borderline risk of bacterial infection and <5 viral infection likely. In validation cohort, proportion of patients noted with risk score ≥5 was 79.71% for infection and 78.43% for bacterial infection.

Conclusion: We derived 2 simple risk scores to aid in screening and appropriate treatment selection for AIRD patients in hospital setting. Clinical features and other markers may improve classification of patients. External validation has to be performed to evaluate generalizability of risk score.


  PB0001: Alteration of serum biomarkers in primary knee OA Top


Meghna Saha, Subhankar Hadar, Biswadip Ghosh; Department of Rheumatology, IPGME and R and SSKM Hospital, Kolkata, West Bengal, India

Background: Osteoarthritis (OA) is a leading cause of pain and disability. 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 knee OA.

Methods: 249 patients with knee OA with local clinical inflammation were recruited, subdivided in systemic Non-Inflammatory group (77) and Inflammatory group (172) based on serum levels of ESR and CRP. WOMAC of these patients were measured. Then OPG, RANKL, MPO, Fibrin, Human COLL2-1 NO2 from the serum were measured by ELISA and compared with 48 healthy controls.

Results: The serum level of RANKL is significantly higher in all patients of knee OA, when compared with healthy control group and serum level of OPG is significantly lower in both Non-Inflammatory group and Inflammatory group, when compared with healthy control group. Serum level of MPO was found to be significantly lowered in both the inflammatory and Non-inflammatory patient groups. However, fibrin and Human COLL2-1 NO2 showed no significant change in both the patient groups when compared with healthy control group.

Conclusions: Increased serum level of OPG and decreased level of RANKL indicate abnormality in bone. COLL2-1 NO2, a marker of cartilage damage is not altered in OA patients in comparison to healthy controls. MPO, a marker of inflammation, is significantly lowered in all patients of Knee OA. Serum level of Fibrin, an acute phase reactant, showed no significant change in patients with primary OA. So, it seems that the pathogenesis in knee OA is localised inflammation and role of bone may be no less than cartilage. It is time to shift our attention outside cartilage in knee OA, particularly in patients with systemic inflammation, as evidenced by raised ESR/CRP in many patients.


  PB0002: Methotrexate treatment is associated with reduction of neutrophil reactive oxygen species and CD177 in RA patients Top


Varun Dhir, Urvashi Kaundal, Aastha Khullar, Biman Saikia; PGIMER, Chandigarh, India

Background: Methotrexate (MTX) is the gold-standard DMARD in rheumatoid arthritis, and may work through inhibiting neutrophils. This may involve changes in ROS production and neutrophil activation.

Objectives: To compare ROS production and activation markers CD177, CD11b and CD64 in neutrophils of naïve RA and methotrexate treated RA patients.

Methods: This was a single-center cross-sectional study, which recruited rheumatoid arthritis (RA) patients and healthy controls. Both naïve and MTX treated RA patients (>=15 mg/week x 6 mos) recruited. Neutrophils were separated by using Hetasep and density-gradient centrifugation. Reactive oxygen species (ROS) were detected at baseline and after PMA (1.62 ug/ml) stimulation using dihydrorhodamine assay on FACS. ROS by Luminol detected after adding PMA (0.4 ug/ml) and Luminol (50 uM) by luminescence detector. Activation markers CD177, CD11b and CD64 were assessed on FACS.

Results: This study included total 121 subjects, which included 53 (F:M= 43:10) RA on methotrexate (MTX-RA), 47 (F:M=39:8) naïve (naïve-RA) and 21 healthy controls. Mean DAS28-3 was significantly higher in naïve-RA than MTX-RA (6.2, 4.9, p<0.001). At baseline, neutrophils of naive-RA compared to MTX-RA showed higher MFI on DHR assay (11049, 7301, p=0.009). This remained higher even after PMA stimulation. There was no significant difference between MTX-RA and healthy controls after PMA. Even on luminol assay, after PMA stimulation, there was higher ROS production in naïve-RA than MTX-RA. CD177 had higher expression in naïve-RA than MTX-RA patients (MFI: 46620, 34475, p=0.02) and healthy controls (46620, 26855, p= 0.001). No significant difference in CD11b and CD64 levels between naïve RA and MTX-RA.

Conclusions: MTX treated RA patients had reduced (similar to healthy controls) levels of ROS production in neutrophils. CD177 expression was also significantly reduced in MTX- treated patients. One of the ways MTX acts in RA may be through reducing neutrophil activation and ROS.


  PB0003: Female sex hormone mediated protein regulations in rheumatoid arthritis Top


Debolina Chakraborty, Uma Kumar1, Sagarika Biswas; CSIR-Institute of Genomics and Integrative Biology,1AIIMS, New Delhi, India

Background: Female prevalence in certain diseases more than male, rise several questions which is still unclear and need to be revealed. Rheumatoid Arthritis (RA) which is a persistent systemic disease associated with inflammation of joints leading to joint cartilage destruction is one such autoimmune disease with female to male ratio 4-5:1 (age<50) and 2-3:1 in later ages. Controversies whether female sex hormone has positive or negative influence on RA progression emerge an interest to search for the pathways regulated by sex hormones in RA.

Objectives: For diminishing this controversy, this study will deal with the proteins which are differentially expressed with hormonal (Estrogen: Most important female sex hormone regulating RA) regulations in RA patients leading to their in-depth study for understanding the mechanism of estrogen regulated disease associated proteins.

Methods: Expression level of pro-inflammatory cytokines TNFα, IL6 and IL17 were measured before and after estrogen induction in RA and OA primary fibroblast like synoviocytes compared with LPS induced and normal SW982 synovial cells. Differential protein profiling will be studied.

Results: Inflammatory status of RA FLS compared to OA and secondary cell lines by estrogen induction is deduced. Differentially proteins expression which links estrogen with inflammation can reveal a potential reason for the prevalence of women affected by RA.

Conclusions: Expression changes of specific identified proteins can directly link estrogen mediated pathways in regulating disease pathogenesis. This study will provide an idea for analyzing the reason for different RA conditions in complete female life cycle and RA remission in pregnancy period as well as will provide the instances for increase in RA severity during nulliparity. These proteins can also become potential targets for therapy and can provide an instant for different ways of treatment for different sexes in RA.


  PB0004: Major histocompatibility antigen HLA-DQB1*0601 is associated with rheumatoid arthritis among Indians in a replication study: Evidence of gene-environment interaction with LPG stove use Top


Able Lawrence, Anshul Dhar, Swayam Prakash, Suvrat Arya, Amita Aggarwal, Suraksha Agarwal; Sanjay Gandhi Postgraduate Institute of Medical Science, Lucknow, Uttar Pradesh, India

Background: Shared Epitope interaction with smoking does not fully explain MHC association in Rheumatoid Arthritis (RA). We previously showed HLA-DQB1*0601 with restricted Asian distribution as susceptibility allele in India. Pristane Induced arthritis susceptibility is determined by rat DQ ortholog RT1-B.

Objective: To confirm DQB1*0601 association with RA and explore interactions with smoking and household fuel use.

Methods: Patients with RA (ACR 2010 criteria) and healthy controls of similar ethnic Background were included. Data on environmental exposure like tobacco use, active and passive smoking and exposure to household smoke during cooking including biomass fuels (wood, coal, cow-dung cake) and hydrocarbon fuels LPG and kerosene was collected. Patients sex, age of onset, duration of exposure, birth year, RF , anti-CCP were also recorded. HLA-DQB1*0601 was genotyped using Sequence-specific PCR. The association of Chi-squared test was used to test association. Gene environment interaction was studied using Cox regression after stratifying by genotype.

Results: 175 patients (21 males) and 263 healthy controls were included. The median age of onset of RA was 37(range 17-67 years) and median duration of disease was 9 years (range 2mo to 35 years). DQB1*0601 was present in 45/175 (25.7%) of patients and 47/263 of controls (17.9%), OR 1.59 (P<0.005) confirming association with RA. LPG exposure was associated with earlier onset of RA (median 40 years versus 44 years) only in the presence of DQ6.1 with a hazard ratio of 2.5 (CI 1.2-5, P=0.014).

Conclusions: This replication study confirms the association of DQB1*0601 with RA. There is signal of gene-environment interaction between DQ6.1 and exposure to LPG stoves.
Figure 1: Earlier onset of rheumatoid arthritis among LPG stove exposed only in the presence of DQB1*0601: Evidence of gene-environment interaction. Kaplan Maier hazard function amone DQB1*0601 negative (N = 128) and DQB1*0601 positive subjects (N = 45) LPG use is associated with a hazard ratio of 2.85 (1.23–6.63) P = 0.015 only among DQB1*0601 positive subjects

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Reference

  1. Lawrence A, Prakash S, Bharadwaj U, Aggarwal A, Misra R, Agrawal S. Major histocompatibility antigen HLA-DQ6.1 (DQA1*0103/DQB1*0601) increases rheumatoid arthritis risk independent of shared epitope among Indians. Arthritis Rheumatol 2016;68 Suppl 10.



  PB0005: Correlation of calcium pyrophosphate dihydrate crystals with varying grades of osteoarthritis Top


Elizabeth Vinod1,2, Tephilla Epsibha Jefferson1, Soosai Manickam Amirtham1, Neetu Prince1, Upasana Kachroo1, Boopalan Ramasamy3; 1Department of Physiology, CMC,2Centre for Stem Cell Research, CMC, Vellore, Tamil Nadu, India,3Department of Orthopaedics, Royal Darwin Hospital, Tiwi, Australia

Background: Accurate diagnosis of osteoarthritis (OA) is the first important step in ensuring appropriate management of the disease. A multitude of tests involving assessment of biomarkers help in assessment of severity and grading of osteoarthritic damage. However, most of these tests are time consuming and are limited by the paucity in synovial fluid volume. In majority of OA effusions, calcium pyrophosphate dihydrate and basic calcium phosphate crystals are found. Therefore, the aim of our study was to evaluate whether a correlation existed between the amount of calcium containing crystals present in synovial fluid and severity scoring of osteoarthritis to propose a quick and inexpensive technique for disease assessment.

Materials and Methods: 12 Adult male NZW rabbits were used to create low-and high-grade OA (n=6 each) using monosodium-iodoacetate. At 16 weeks, synovial fluid and joints were harvested for histopathological analysis (H&E, Safranin O and Collagen II). OA grading was established based on OARSI scoring and the calcium containing crystals in synovial fluid (5μl) were assessed by light microscopy following staining with Alizarin red on a wet mount preparation (three independent blinded investigators). Presence of calcium containing crystals was confirmed using Fluo-4 AM staining. For statistical analysis, Mann Whitney U test was used to compare low- and high-grade OA groups, whereas Pearson correlation was used to assess the relation between number of calcium crystal clumps vs the grade of OA.

Results: and Conclusion: The clumps counted in low-grade OA were significantly lower than high-grade OA, in addition to showing a positive correlation (coefficient: 0.65; P=0.02) between positively stained clumps vs OA severity. A positive correlation between the amount of calcium containing crystals and severity scoring of OA thus enables us to consider Alizarin red staining of synovial fluid crystals as an effective and rapid, bed-side method for detecting progression of the disease.


  PB0006: Immunomodulation of CD8+ T-cells from patients with rheumatoid arthritis by lactobacillus rhamnosus Top


Archana Tripathy, Prasanta Padhan, Sunil Raghav and Bhawna Gupta; School of Biotechnology, Kalinga Institute of Industrial Technology, Bhubaneswar, Odisha, India

Background: Rheumatoid arthritis (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. Microbes are also considered and often discussed as potential triggers for initiation and perpetuation of RA. Alterations in gut microbiota create an imbalance between pro-inflammatory and anti-inflammatory immune responses and trigger the development of RA.

Objective: Identify the role of Lactobacillus rhamnosus (L. rhamnosus) in modulating CD8+Tcell response in RA patients and determine the effect of Escherichia coli (E. coli) towards acute inflammation in RA.

Methods: Hundred RA patients and hundred healthy donors participated in this study. Clinical variations like disease duration, number of inflamed joints, type of bones deformities, CRP, RF, Anti-CCP, ESR were recorded for each patient and DAS 28 scores were calculated with the help of the clinician. Different cytokines and transcription factors level was checked after stimulation of CD8+T cells with E. coli and L. rhamnosus by flow cytometry. We analyzed the TLRs profiling and the proliferation rate of CD8+Tcells upon bacterial infection.

Results: Significant increased of different inflammatory cytokines in patients with RA compared to healthy donors after stimulation with E. coli (p<0.005). In CD8+ T cells, L. rhamnosus treatment decreased the expression level of proinflammatory mediators which leads to down regulate Th1 response in CD4+T cell (p<0.05). L. rhamnosus infection significantly increased the mRNA expression of TLR2 which enhances the proliferation capacity of CD8+ T cells (p<0.005) where as E. coli significantly upregulated the transcript of TLR1 and 4.

Conclusion: In summary, our data suggest that co-culture of L. rhamnosus with CD8+T cells can effectively suppress CD8+T cell mediated inflammation by simultaneous down regulation of Th1 response which may leads to suppression of disease symptoms.


  PB0007: TLR7 mediated activation of cd8+ t cells leads to downregulation of inflammatory mediators in patients with rheumatoid arthritis Top


Nitish Swain, Archana Tripathy, Prasanta Padhan, Sunil Raghav, Bhawna Gupta; School of Biotechnology, Kalinga Institute of Industrial Technology, Bhubaneswar, Odisha, India

Background: Rheumatoid arthritis (RA) is a systemic inflammatory, autoimmune disease of unknown origin characterized by aberrant immune responses towards self peptides. The disease is thought to be induced by environmental or genetic factors. Cytokine mediated destruction of the synovial joints is the signature symptom of the progression of this disease. Toll- like Receptors (TLRs) are one of the most prominent innate immune receptors which recognize an array of components ranging from microbial compounds to nucleic acids. Research has shown the presence of TLRs in the CD8+ T cells in case of RA patients and this study focuses on finding the role of TLR7 in the pathogenesis of RA.

Objective: To determine the role of TLR7 in CD8+ T cells of patients with RA.

Methods: 40 RA patients and 35 healthy control (HC) participated in this study. Clinical variations like disease duration, number of actively inflamed joints, bone deformities, CRP, RF, Anti-CCP, ESR were recorded for each patient and DAS 28 scores were calculated. We analyzed the expression of different cytokine transcripts by real-time PCR and protein level expression by flow cytometry as well as immunoblotting after stimulation of TLR7 present on CD8+T cells by its agonist imiquimod.

Results: We found that TLR7 ligand responsiveness significantly increased the mRNA expression of different inflammatory mediators in CD8+T cells of RA patients compared to healthy individuals. Flow cytometry analyses revealed that there was no significant increase in the level of the cytokines that were found at the mRNA level. Significant up regulation of Tristetraprolin (TTP) in treated CD8+ T cells might be the explanation for translation inhibition and mRNA decay of inflammatory mediators (p< 0.05).

Conclusion: Our data suggests that activation of CD8+ T cells via TLR7 agonist decreased expression of effector molecules to a similar level as in HC.


  PB0008: Serum IL-36γ levels are elevated in enthesitis related arthritis category of juvenile idiopathic arthitis Top


Sanjukta Majumder, Amita Aggarwal; Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: IL-36, is a newly identified cytokine belonging to the IL-1 family and have been shown to be increased in serum and local tissue in Spondyloarthropathies (SpA) like psoriasis and IBD. IL-36R antagonist is being tried in psoriasis. JIA-ERA resembles SpA however, no data is available in JIA-ERA regarding IL-36.

Objective: To identify role of IL-36 in JIA-ERA by studying PBMC, serum and synovial fluid (SF) levels of IL-36.

Methods: JIA-ERA patients (ILAR criteria) were enrolled in the study. Forty-three SF samples and 37 paired serum samples from patients and 16 serum samples from healthy subjects were collected after obtaining informed consent. The study was approved by ethics committee. IL-36α and IL-36γ was measured by ELISA (R&D Systems) following manufacturers recommendations. In PBMCs of 11 patients mRNA levels of IL-36 were also measured. All Results: are denoted as Mean±SD.

Results: Among 43 patients 41 were boys. Their mean age was 15.7 ± 2.8 years and the mean duration of disease was 43.4 ± 37.8 months. All had active disease with mean ESR of 51.2 ± 32.9 mm. Out of 16 healthy control serum IL-36γ was detectable in 1 person while it was present in 15 JIA-ERA patients. The serum levels were higher in patients 64.51 ± 153.1 pg/μL as compared to controls (p<0.05). Among SF it was present in 19 of 43 samples and the mean level was 84.71 ± 271.21 pg/μL. In contrast IL-36α was detectable in only 1 patient's serum and synovial fluid and none in healthy control serum.

Real time qPCR analysis was done on 11 patients with JIA-ERA. Patient PBMCs had higher mRNA fold difference as compared to HC: IL-36αà2.9 ± 5.9 and IL-36γà9.54 ± 14.4 folds.

Conclusion: IL-36 may have a role in inflammation seen in JIA-ERA but its exact role needs to be studied.


  PB0009: Circulatory histidine levels for evaluating disease activity in patients with takayasu arteritis: A targeted NMR based serum metabolomics study Top


Pankti Mehta, Umesh Kumar, Avinash Jain, Anupam Guleria, Durga P Misra, Ramnath Misra, Dinesh Kumar; Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Objective assessment of disease activity is important for management in Takayasu arteritis (TA). The dominance of oxidative stress is the hallmark of active inflammation. Both preclinical and clinical data suggests that histidine has strong anti-oxidative and anti-inflammatory effect. Thus, we hypothesized that circulatory Histidine may serve as a biomarker of activity in TA.

Objective: To perform estimation of circulatory levels of histidine and evaluate its potential in diagnostic screening of active and inactive TA patients.

Methods: Serum samples were collected from 98 TA patients fulfilling American College of Rheumatology (ACR) criteria and 77 normal controls (NC). The 1D 1H CPMG NMR spectra was recorded on each serum sample at 800 MHz NMR spectrometer. Concentrations of Histidine were estimated (with formate as an internal reference) using NMR Suite of software program CHENOMX.

Results: According to Indian Takayasu Clinical Activity Score (ITAS) combined with acute phase reactant–erythrocyte sedimentation rate [ITAS-A (ESR)], 45 patients (46%) were clinically active, whereas 53 patients (54%) patients were inactive. Circulating levels of histidine were significantly decreased in active TA patients compared to both inactive TA patients and NC, whereas, there was no statistically significant difference between Inactive TA and NC. Further, the receiver operating characteristic (ROC) curve analysis was performed to assess the diagnostic potential of Histidine and yielded satisfactory sensitivity and specificity with AUROC equal to 0.65 [95% CI=0.54-0.76]. The circulatory levels of Histidine correlated well the erythrocyte sedimentation rate (r= - 0.19, p< 0.075) and with the C-reactive protein level (r= -0.26, p< 0.01).

Conclusion: The circulatory levels of histidine may serve as a useful biomarker for the assessment of disease activity and guiding treatment in TA patients. However, its use in clinical settings will require future studies on large patient cohorts in a longitudinal manner improve accuracy.
Figure 1: (a) Box plot showing comparison of circulatory histidine levels in active and inactive TA patients with respect to normal healthy control. (b) Receiver operating characteristic curve analysis performed form evaluating the diagnostic potential of histidine. The area under the receiver operating characteristic curve is shown in red to highlight the diagnostic value of circulatory histidine for differentiating active and inactive TA patients. (c and d) Graphs showing pearson-correlation form histidine with erythrocyte sedimentation rate and C-reactive protein levels

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  PB0010: Altered systemic expression of micro RNAs miR-125a, miR-125b and miR-200a in patients with Rheumatoid arthritis Top


Sudipta Chatterjee, Dipanjan Bhattacharyya, Sanchaita Misra, Nitai Bhattacharyya1, Alakendu Ghosh; Department of Rheumatology, Institute of Post Graduate Medical Education and Research, Crystallography and Molecular Biology Division, Saha Institute of Nuclear Physics, Kolkata, West Bengal, India

Background: Regulatory biomolecules, such as long non-coding RNA are being globally investigated to explain the pathogenesis of Rheumatoid arthritis (RA).

Objective: To study the systemic expression levels of micro RNAs miR-125a, miR-125b and miR-200a in patients with RA and comparative analysis of the same with healthy control group.

Methods: Thirty-eight patients with only RA and 15 healthy individuals were recruited. Blood samples were collected. Serum and PBMCs were separated. DAS28-CRP and HAQ-DI scorings were done at the time of recruitment. Laboratory markers for RA were measured from sera. RNA was extracted from the PBMCs, reverse transcribed to c-DNA using universal stem loop primer and qPCR was performed with specific primers for miR-125a, miR-125b and miR-200a using SYBR Green.

Results: Serum TNF- α and CRP was high inpatients (p<0.05). Increased expression of miR-125a was observed in the PBMCs of the patient group compared to healthy controls. On the contrary, miR-125b and miR-200a was found to be under expressed in RA patients. The fold change of the three miRNAs (calculated by 2^-ΔΔCt) were found to be mutually positively correlated.

Conclusion: The over expression of mir-125a suggests its excitatory function in RA pathogenesis. The under expression of mir-125b and mir-200a indicate their inhibitory role in RA pathogenic pathways. The mutual correlation of the micro RNAs indicate their common regulatory mechanism. The specific target molecules of these micro RNAs and their functional implication in context of RA needs to be investigated hence.


  PB0011: Hypomethylation of the promoter region of TLR2 and TLR4 gene in patients with rheumatoid arthritis and periodontitis: a comparative study Top


Sudipta Chatterjee, Dipanjan Bhattacharyya, Ankur Rao1, Arghya Chattopadhyay2, Alakendu Ghosh; Department of Rheumatology, Institute of Post Graduate Medical Education and Research, St. Xaviers College, Kolkata, West Bengal, Department of Rheumatology, Postgraduate Institute of Medical Education and Research, Puducherry, India

Background: Association of Rheumatoid arthritis (RA) and Periodontitis (PD) has long been implicated in several studies. Epigenetic modifications are being recently explored to explain such associations, DNA methylation being one such mechanism that regulate gene expressions.

Objective: To study the effect chronic generalized periodontitis on systemic methylation of TLR2 and TLR4 genes and compare that with only RA and RA with PD patients.

Methods: Fifty-three RA patients, among which 21 patients had chronic generalized PD, 20 patients with only PD and 15 healthy individuals recruited. Blood samples were collected. Serum and PBMCs were separated. DNA was isolated from PBMCs, then were first bisulphite converted and then methylation specific PCR were performed using primers for methylated and um-methylated promoters of TLR2 and TLR4. The DNA amplifications were checked in horizontal gel electrophoresis. The methylation signatures were verified by DNA sequencing (Sanger) of the amplified products.

Results: The anti-CCP, DAS-CRP and HAQ DI were higher in patients with both RA and PD (220+40, 5.7+0.2, 1.5+0.1 respectively, p<0.05). Serum TNFα, IL8 and IL-17 were highest in patients with both RA and PD (p<0.05). Control samples had shown amplification bands for methylated primers TLR2 and TLR4 but not for un-methylated primers. However, only RA, only PD and RA with PD samples, had shown amplification for un-methylated primers and not for methylated primers. These Results: together with DNA sequencing indicated that 2 CpG islands in TLR2 promoter and six CpG sites in the promoter of TLR4 genes were hypo-methylated in the PBMCs of patients whereas those remain methylated in healthy individuals.

Conclusion: The observations indicated systemic involvement of TLR mediated pathways in only PD patients which is similar to those in RA. However, further validation in larger cohort and down-stream signalling molecules in vitro needs to be studied.


  PB0013: Increased expression of long non-coding RNAs NEAT1, MALAT1 and MEG3 in patients with lupus nephritis and their impact on disease activity Top


Sulagna Chatterjee, Dipanjan Bhattacharjee, Sudipta Chatterjee, Ayindrila Saha, Sanchaita Misra, Dipendranath Ghosh, Nitai P Bhattacharyya, Alakendu Ghosh; Department of Rheumatology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

Background: Deregulations of long non-coding RNAs (lncRNA) recently implicated in rheumatic diseases including Systemic Lupus Erythematosus (SLE). Although, their specific roles in pathogenesis of disease and clinical impact in lupus nephritis remain largely unknown.

Objectives:

  1. Comparative quantification of expression of regulatory long non coding RNAs (NEAT1, MALAT1 and MEG3).
  2. Association between the lncRNAs and the serological markers (C3, C4, Anti-dsDNA antibody) in patients with lupus nephritis.


Methods: 10 Lupus patients with nephritis (Class III & Class IV) and 9 healthy controls (age-sex matched) were recruited. Peripheral blood was collected from each study participants and the PBMCs were respectively isolated. RNA was isolated from the PBMCs of individual samples and expression of the lncRNAs was measured by Real time PCR. C3, C4 and Anti-dsDNA were quantified by Nephelometry and ELISA respectively for every participant.

Results: Expression of lncRNAs (NEAT1, MALAT1, MEG3) increased significantly (p=0.0002, p=0.0003, p=0.01 respectively) in Patients. Significant negative correlation found between NEAT1 and C3(r=-0.75, p=0.01) as well as with C4(r=-0.82, p=0.004). Positive correlation found between NEAT 1 and Anti-dsDNA antibody(r=0.42, p=0.2) and with SLEDAI score (r=0.63, p=0.05). No significant correlation found between MALAT1 and MEG3 with C3 {(r=-0.01, p=0.9), (r=-0.22,p=0.5) respectively}, C4{(r=0.07,p=0.83),( r=-0.29,p=0.4) respectively}. Negative correlation found between MALAT1 and Anti-dsDNA antibody (r=-0.7, p=0.02), while no correlation found between MEG3 and Anti-dsDNA antibody ( r=-0.006,p=1). No significant correlation was found between MALAT1 and SLEDAI score (r=-0.17, p=0.63) and between MEG3 with SLEDAI score (r=0.34, p=0.33).

Conclusion: Increased expression of NEAT1 and its correlation with the serological markers (C3, C4, Anti-dsDNA antibody) and SLEDAI score well reflect the disease activity compared to MALAT1 and MEG3 which indicates the potentiality of NEAT1 as a probable additional marker of disease activity in patients with lupus nephritis. However validation of this finding in large sample is necessary.


  PB0014: Polymorphisms in genes involved in methotrexate pathway: Predictor of response to methotrexate therapy in Indian rheumatoid arthritis patients Top


Ankita Singh, G Harikrishnan, Vikas Gupta, Pradepto Sekhar Patro, Ramnath Misra, Amita Aggarwal; Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute, Lucknow, Uttar Pradesh, India

Introduction: Methotrexate (MTX) is first line therapy to treat rheumatoid arthritis (RA) Variable response to MTX therapy(50-60%) is observed in patients with RA.The variability can be explained by single nucleotide polymorphism (SNP) of genes involved in MTX metabolic or adenosine pathway. Hence, we studied association of seven genes polymorphism [rs2236225 (MTHFD1 1958G>A), rs17602729 (AMPD1 C>T), rs1127354 (ITPA C>A), rs1431131 (TGFBR2 A>T), rs2372536 (ATIC C>G), rs11188513 (ENTPD1 C>T), rs5751876 (ADORA2A T>C)] with response to MTX therapy in RA patients.

Methods: Patients satisfying EULAR/ACR 2010 criteria,DMARD naïve with active RA (DAS28) >3.2 were enrolled. Genotyping was done by TaqMan 5' nuclease assay. Patients were treated with MTX monotherapy ,gradually escalating the dose to maximum of 25 mg/week or maximal tolerated dose.Based on EULAR response criteria, patients were classified into responder and non-responder groups at 4 months. Using Chi-squared test, SNPs were associated with response to MTX therapy.

Results: 207 patients (85.5% females; median-age 40 [17] years); median disease duration 24 (40) months; median DAS28-CRP 4.64 (1.39) were enrolled. At 4 months, based on EULAR response 172 patients were classified as responders and 35 as non-responders. The G allele(p=0.009) and GG genotype(p=0.006) of ATIC gene and C allele(p=0.00005) and CC genotype(p=0.006) of ITPA gene associated with response to MTX therapy.On binary logistic regression analysis including clinical measures:DAS28 ESR,TJC,SJC, age and ATIC, ENTPD1and ITPA genes polymorphism; CC genotype of ITPA and GG genotype of ATIC gene in additive(p=0.006,p=0.002respectively)and recessive model(p=0.019,p=0.002 respectively) were independent predictors of response to MTX

Conclusion: Two genes polymorphism i.e. ATIC C>G and ITPA C>T were associated with response to MTX therapy in Indian RA patients. In a model comprising of clinical variables along with three genes polymorphism, GG genotype of ATIC C>G and CC genotype of ITPA C>T gene had an independent association with response to MTX.


  PB0015: Urinary renalase as a biomarker in lupus nephritis Top


Ranjan Gupta, Akhilesh Yadav1, Amita Aggarwal1; Department of Rheumatology, All India Institute of Medical Sciences, New Delhi1Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Renalase is a renal hormone secreted chiefly by tubular epithelial cells and degrades catecholamines. Serum renalase levels have been shown to correlate with markers of renal disease activity in patients with proliferative Lupus Nephritis (LN).

Objective: To test urinary Renalase in a cross-sectional and longitudinal study as a biomarker of renal disease activity of LN

Methods: Forty-Seven SLE patients with active nephritis (AN), 20 with active disease without nephritis (active non-renal; ANR) and 20 with inactive disease (ID) were enrolled. Patients in AN group were treated according to the ACR 2012 guidelines and followed up every 6 months for 1 year. At baseline, urine samples were collected from all and serum samples from 10 AN patients. Follow-up samples were collected every 6 months only in AN group. Urine samples from 10 healthy subjects (HC) served as controls. All urinary values were normalized for creatinine excretion. Variables are expressed as median (range) and non-parametric tests were used. A p-value<0.05 was considered significant.

Results: Baseline normalized urinary renalase (mcg/mg of creatinine) was significantly higher (p<0.001) in AN group [100.03 (0.45 – 22178.34)] as compared to HC [2.36 (0 – 104.47)]. However, the levels were not different as compared to ID [113.46 (8.04 – 1494.56] and ANR [57.32 (5.36 – 615.25)] groups. Urinary renalase did not show any correlation with serum Renalase [78993.5 (40206 – 293915) mcg/ml] which was tested in 10 patients with AN.

On follow-up of AN patients, with reduction in disease activity, urinary Renalase also decreased significantly at 6 [15.55 (0.64 – 266.88)] and 12 [5.68 (0.16 – 235.2)] months visits as compared to baseline (p<0.001).

Conclusion: Urinary Renalase is a poor biomarker of LN. However, it may be useful in assessing the response to therapy as its levels decrease with the fall in disease activity with treatment.


  PB0016: Urinary MRP8/14, an endogenous toll-like receptor 4 ligand, reflects renal disease activity in lupus nephritis: A cross-sectional and longitudinal assessment Top


Ranjan Gupta, Dipendra K Mitra1, Sonam Rani

Departments of Rheumatology and1Transplant Immunology and Immunogenetics, All India Institute of Medical Sciences, New Delhi, India

Background: In Lupus Nephritis (LN), monocytes/macrophages are the most abundant cells infiltrating the glomeruli and have a relative abundance of Toll-Like Receptor 4 (TLR4).

Objective: To test MRP8/14 (Calprotectin), an endogenous TLR4 ligand, as a urinary biomarker of renal disease activity of LN.

Methods: SLE patients with active nephritis (AN), active disease without nephritis (active non-renal; ANR) and inactive disease (ID) were enrolled. Patients in AN group were treated according to the ACR 2012 guidelines and followed up every 3 months for 1 year. Urine and serum samples were collected at baseline for all and every 3 months in AN group. Urine samples from 10 healthy subjects (HC) and 10 patients of rheumatoid arthritis (RA) served as controls. Variables are expressed as median (range) and non-parametric tests were used for analysis. A p-value<0.05 was considered significant.

Results: Baseline normalized uMRP8/14 (ng/mg of creatinine) was significantly higher in AN group [n=32; 4464 (569-128894)] as compared to ANR [n=10; 1167 (4-18357)], ID [n=10; 438 (147-4320)], HC [1348 (3-2726)] and RA [339 (6-11519)] (p<0.05) groups. It also showed good correlation with urinary protein:creatinine ratio (r=0.55; p<0.001), rSLEDAI (r=0.5, p<0.001) and SLEDAI (r=0.3, p<0.001) but not with sMRP8/14 (r=0.25). Baseline uMRP8/14 but not sMRP8/14 could differentiate between AN and ANR groups and performed better on Receiver Operator Curve (ROC) analysis to differentiate between the two groups.

On follow-up of AN patients, with reduction in disease activity, uMRP8/14 also decreased significantly at 6, 9 and 12 months visits as compared to baseline (p<0.05). uMRP8/14 also showed a rise before conventional markers in 6 patients who relapsed within 1 year.

Conclusion: uMRP8/14 is a potential biomarker of LN disease activity. In patients with active SLE, it helps differentiate between patients with and without renal involvement and has the potential to predict relapse of LN.


  PB0017: CD-14 (C-159 T) variants are associated with SLE and lupus nephritis: Plasma levels of sCD14 is a promising biomarker of SLE disease activity Top


Saumya Ranjan Tripathy, Aditya Kumar Panda1, Rina Tripathy, Manoj Kumar Parida, Bidyut Kumar Das; SCB Medical College, Cuttack,1Khallikote University, Berhampur, Odisha, India

Background: Cluster of differentiation-14 (CD-14) is a co-receptor for TLRs (2, 4, 7 and 9) signaling pathways and believed to plays an important role in innate immune system. Host innate receptor TLR7 and 9 recognizes nucleic acids as their ligands and produce type-I interferon which has been demonstrated in pathogenesis of SLE. Since TLR7 and 9 require CD14 for effective signaling, we hypothesized that CD-14 would correlate with disease activity and pathogenesis of SLE.

Objectives: To evaluate association of C159-T polymorphism of CD14 with SLE and its disease activity; to evaluate association of soluble CD14 (sCD14) with SLE disease activity.

Methods: In the present hospital-based case-control study, SLE patients (diagnosed based on SLICC-criteria) (n=200 females), and 200 age and sex-matched healthy controls were enrolled. CD14 (C-159T) polymorphism was genotyped by PCR-RFLP. Based on availability of samples (78 SLE patients and 46 healthy controls), plasma sCD14, TNF-α, IFN-α levels were quantified by ELISA. Clinical, serological and other markers of disease activity (C3, C4 and anti-dsDNA) were measured by standard laboratory procedures.

Results: Mutant genotypes (CT and TT) and allele T for CD 14 (C-159T) were significantly associated with predisposition to development of SLE and lupus nephritis. Mutants (CT and TT) for CD14(C-159T) polymorphism was associated with higher plasma sCD14, IFN-α and TNF-α compared to wild type CC.

Plasma sCD14 levels were significantly high in SLE patients compared to healthy controls (P<0.001). Plasma levels of sCD14 correlated with SLEDAI-2K scores (P=0.002, r=0.34), proteinuria (P=0.001, r=0.34) and negatively correlated with C3 (P=0.003, r=-0.33) and C4 (P<0.0001, r=-0.50). Patients with lupus nephritis displayed higher plasma levels of sCD14, TNF-α and IFN-α.

Conclusions: CD14 (C-159T) polymorphism is associated with susceptibility to SLE and lupus nephritis. Plasma sCD14 is a promising marker in the assessment of SLE disease activity.


  PB0018: Interferon gene signature in Indian SLE patients is not correlated with disease activity and clinical manifestations Top


Vineeta Shobha*, Anu Mohan*, Shailesh Dudhgaonkar#, Preethi Karunanithi#; *St. Johns Academy of Medical Sciences, Bengaluru, Karnataka, India

Background: SLE is an autoimmune disease characterized by inflammation and tissue damage. Despite the availability of effective therapies, resistance to treatment and premature mortality remain major concerns. Interferon (IFN) signature genes are highly expressed in peripheral blood of patients with systemic lupus erythematosus (SLE), especially in the presence of active disease. However, the alteration in the expression of this gene signature upon treatment has not been fully elucidated in Indian SLE patients.

Objective: To identify the IFN gene signature in Indian patients and understand its correlation with clinical manifestations of disease and its treatment.

Methods: We characterized 54 Indian SLE patients for the IFN signature, and its correlation / concordance with clinical manifestations and SLEDAI score. Patients with mild, moderate and severe disease with SLEDAI ≤ 4, 5 to 12 and ≥13 respectively were recruited. All patients were on treatment with steroids in combination with other immunosuppressants like hydroxycloroquin, cyclophosphamide, MMF, azathioprine and methotrexate.

Results: Multiple IFN related pathways were enriched with significant increase in IFN gene signature in SLE patients as compared to normal heathy volunteers. These gene signatures did not correlate with SLEDAI score or clinical manifestations. Based on these signatures, we were able to stratify patients into two groups - i.e. patients with high IFN signature and those with low IFN signature [ref [Figure 1]]. Furthermore, we noted that IFN-high and IFN-low groups were composed of patients from across disease severity.
Figure 1

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Conclusion: These findings indicate that more studies are required to understand if specific immune cell types such as pDCs, monocytes, contribute to the IFN gene signature in different aspects of SLE disease biology and their clinical correlations. An an in-depth characterization of variants within the IFN pathway may provide guidance for personalized therapies.


  PB0019: Lymphocyturia is a good and cheap biomarker for active lupus nephritis and is sensitive to change Top


Sarit Sekhar Pattanaik, Ankita Singh, Shilpa Venkataraman1, Ramnath Misra, Vinita Agarwal1, Amita Aggarwal; Departments of Clinical Immunology and Rheumatology and1Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Introduction: Multiple urinary biomarkers have been described for lupus nephritis, however none has reached the clinic. Recently, urinary CD4 and CD8 T cells have shown high specificity for active lupus nephritis(LN). Thus we studied whether urinary lymphocyte count by simple staining of sediment or T cell count by flowcytometry can serve as a simple and cheap biomarker.

Methods: Patients with active LN(ALN) defined as renal SLEDAI(rSLEDAI) >4, active lupus without nephritis(ANR) defined as SLEDAI >4, inactive nephritis(IN) defined as rSLEDAI ≤4 and SLEDAI <4, and age matched healthy controls(HC)were included. Lymphocytes in the urine were stained using Kovak stain and reported as number of cells/high power field. CD3 T cells were counted by FACS using anti-CD3 antibody and DAPI for live gate. Immunohistochemistry was done on renal biopsy for CD3 cells.

Results: Among 74 patients, 44 had ALN, 10 had ANR disease and 20 patients had IN. The median (IQR) urinary Lymphocyte count was 4(7) in ALN as compared to ANR[2(3.5),p=0.1], IN(0, p<0.001) and HC(0,p<0.001). The median CD3 count was 2793(8982) in ALN as compared to ANR[209(539.2), p=0.001],IN[86(303),p<0.001] .There was moderate correlation between lymphocyturia and urinary T cell number(r=0.59, p<0.001).

Lymphocyturia and urinary CD3 cell count had moderate correlation with SLEDAI(r=0.59, p<0.001;r=0.47,p<0.001) and rSLEDAI(r=0.58, p<0.001;r=0.54, p<0.001).The ROC curve for lymphocyturia to differentiate ALN from IN(AUC: 0.87) and for CD3(AUC: 0.874) [Figure 1].
Figure 1

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In 15 patients of ALN on follow up, there was a excellent correlation between change in SLEDAI and change in lymphocyte count(r=0.845,p<0.001).In 31 patients with renal biopsy, there was a moderate correlation between the CD3 cells by IHC in tubulointerstitial compartment and urinary CD3 cells(r=0.55,p=0.001)

Conclusion: Lymphocyturia measured by simple staining of urinary sediment can be a cheap and effective marker of active LN and can be used to follow up patients with active nephritis in resource poor countries.


  PB0020: Clinical utility of Antibodies to C1q sub-component as a biomarker of anti-phospholipid syndrome Top


G Arvind, J Kabeerdoss, A Nair, A Mathew, R Goel, SKumar, J Mathew, D Danda ; Department of Clinical Immunology and Rheumatology, Christian Medical College Hospital, Vellore, Tamil Nadu, India

Background:

Several clinical and experimental animal model studies have emphasised the role of complement activation in anti-phospholipid syndrome (APS). Recently, in a 2016 study by Oku et al published in Oxford rheumatology, antibodies to C1q (Anti C1q) sub-component were present in significantly higher number of primary APS patients compared to controls ( healthy subjects, non-lupus connective tissue disease (CTD) patients and lupus patients negative for anti-phospholipid antibodies (APLs).

Aim: To evaluate the utility of Anti C1q as a biomarker of APS.

Methods: We enrolled 49 APS patients and 341 age-matched controls in a retrospective design, recruited from June 2016 to June 2019. Anti C1q ELISA was performed by commercial ELISA kit (Human Diagnostics, Germany).

Results: Median age of APS patients was 37 years in comparison to 35 years in controls. Among the APS patients,13 were primary and 36 were secondary. Among the controls, 85 were non-lupus CTD patients, 44 were lupus patients with positive APLs and 211 were lupus patients negative for APLs. Anti C1q antibody was positive in 12/49(24%) APS cases in comparison to 114/ 341 (33.4%) controls (p value- 0.1, chi-square test). Anti C1 q was present only 1 out 13 (7.6%) primary APS patients and was positive in 11/36 (30.5%) secondary APS patients. Anti C1q positivity in APS secondary to lupus was 11/36 (30.5%) in comparison in 87/ 211 (41.2%) in lupus controls (p value-0.3, chi-square test)

Conclusion: Anti C1q was not helpful as a biomarker of APS in our study, in both primary as well as secondary APS clinical settings.


  PB0021: NLR reflects the clinical activity in AS and corresponds to BASDAI than other inflammatory parameters Top


Shivputra Ghanti, S Chandrashekara, C Renuka1, K R Anupama; ChanRe Rheumatology and Immunology Centre and Research,1ChanRe Diagnostic Centre, Bengaluru, Karnataka, India

Background: Neutrophil-to-lymphocyte ratio (NLR) has emerged as one of the important markers in inflammatory rheumatic diseases. The present study is intended to analyze the relationship of the NLR with various measurable parameters of AS.

Materiala and Methods: The demographic, clinical and laboratory parameters obtained at the recruitment phase were, age, gender, clinical symptoms for scoring the BASDAI, BASFI and BASMI, total leucocyte count, ESR and CRP. NLR was calculated. The BASDAI was considered as the outcome variable. Patients were classified as those with low (<4) and high disease (≥4) activities. The NLR was considered as a categorized variable with sub groups <2, 2-4 and >4.

Statistics: Mann Whitney test for no normal distribution, chi square test for categorized variables and Spearman's correlation for bivariate linear association were performed. Mountain plots were used to verify agreement of clinical and inflammatory parameters of BASDAI, BASFI, BASMI, NLR and CRP.

Results: The study involved a total of 162 AS patients (112 males and 50 females) with a median age of 39.5 (18-73) yrs. The corresponding values noted were: BASDAI 2.2 (0-8.5), BASMI 1 (0-10), BASFI 2.2 (0-7.7), ESR 29 (2-126), CRP 8.45 (0.3-120) and NLR 2.37 (0.99-12.39). BASDAI <4 was noted in 124 subjects and 38 had ≥4.

NLR was high in BASDAI ≥4 group. BASDAI strongly correlated with BASFI and moderately with BASMI. NLR showed weak correlation with BASFI and BASMI. Mountain plots revealed BASFI had least bias 0.1 (2.5th and 97.5th percentiles were -3.43 and 2.99) with BASDAI, followed by NLR -0.22 (-4.35 and 3.86), BASMI 0.8 (-6 and 4.99) and CRP -7.25 (-91.41 and 2.85).

Conclusion: NLR corresponds with BASDAI and serves as a better marker of inflammation than CRP in AS. NLR correlates weakly with BASFI and BASMI in lower BASDAI score.


  PB0022: Adenosine deaminase-genetic polymorphism and baseline serum level as a biomarker of treatment response to methotrexate in rheumatoid arthritis Top


G Harikrishnan, A Singh, A Aggarwal; Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Despite methotrexate (MTX) being the first line therapy for RA,30% of patients do not respond to MTX.Methotrexate acts by increasing adenosine levels leading to anti-inflammatory effects.Adenosine deaminase (ADA) enzyme converts adenosine to inosine thus reduces levels of adenosine.Thus,ADA levels and single nucleotide polymorphism(SNP) in ADA gene may affect the treatment effect of MTX.

Objective: To study if genetic polymorphism in ADA gene and baseline serum ADA levels in RA patients is associated with MTX response

Methods: 207 DMARD naïve active RA(DAS28 ESR ≥3.2)patients satisfying EULAR/ACR 2010 criteria were enrolled. Genotyping was done in all patients(n=207) by TaqMan 5' nuclease assay.In a subset of patients(n=59) blood sample was collected at baseline and 2 months for ADA estimation by ELISA. MTX was initiated at dose of 15mg/week, escalated 5mg/month till the patients had DAS28 < 2.6 or a maximum dose of 25 mg/week was reached. At 4 months based on EULAR response patients were classified as responders or non-responders.Association of SNPs in ADA gene, baseline clinical parameters and serum ADA levels with EULAR response was studied.

Results: Among 207 patients(177females)mean age was 41.49± 12.14 years and median disease duration 24(40) months.172 patients(83.1%) were responders and 35(16.9%) non responders.47.8% were AA,42.5% AG and 9.7% were GG genotype.Mean DAS 28 CRP was 4.76 ± 1.05 and median DAS 28 ESR was 6.00(1.38). Mean serum baseline ADA was 10.82 ±5.33 pg/dl.The baseline parameters like age, disease duration, ESR,CRP,DAS 28 ESR,DAS 28 CRP were similar between responders and non-responders. SNPs in ADA gene were not associated with EULAR response(p=0.475).Baseline ADA levels(10.52 ± 5.37 vs 12.28 ± 5.14;p=0.34)2month ADA levels(p=0.34) and delta ADA(p=0.55) didnot show any association with MTX response. Mean ADA level was similar between the 3 genotypes of ADA(p=0.736).

Conclusion: ADA gene polymorphism and serum ADA levels do not affect response to MTX


  PB0023: Ficolin H assay in pediatric-onset systemic lupus erythematosus Top


Pratap Kumar Patra, B N Anil Kumar, Amit Rawat, Surjit Singh; Post Graduate Institute of Medical Education and Research, Chandigarh, India

Background: Systemic lupus erythematosus (SLE) is a complex autoimmune disease of childhood with a myriad of presentation. Though the complement pathway plays a key role in the pathogenesis of SLE, the role of ficolin pathway in its pathogenesis is not well studied.

Objectives: To study the role of ficolin pathway by estimating ficolin H levels in children with SLE and to establish a correlation of ficolin H level with the disease activity.

Methods: A cross-sectional study was carried out in the Pediatric Rheumatology Clinic, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India. Thirty-three cases of SLE and 31 controls were included in the study. Ficolin level in both groups was measured by enzyme-linked immunosorbent assay (ELISA) and compared.

Results: There were 21 girls and 12 boys in the study group with a ratio of 1.75:1, whereas the control group had 18 girls and 13 boys, a ratio of 1.8:1 ( p = 0.64 ). A significant difference in the mean ficolin concentration between the groups (cases: 248.35±93.29); (control: 184.71±57.02; p =0.002) was found. A negative correlation between Anti-dsDNA and ficolin level was observed (r -3.72). However, there was no significant difference between the mean ficolin concentration in patients with active disease; 266.76μg/dl as compared to inactive disease; 234.79/μg/dl, (p 0.339).

Conclusion: We observed an increased ficolin level in the study group as compared to control suggesting involvement of ficolin pathway in SLE. A negative correlation was also observed between the disease activity and ficolin H level. However, we did not find any significant difference in ficolin level between active and inactive disease group. Further studies with a larger sample size required to confirm any association between anti-dsDNA titers and ficolin H level in SLE.


  PB0024: Significantly decreased histidine/tyrosine ratio in ANCA†associated vasculitis compared to takayasu's arteritis: A marker for inflammation Top


Avinash Jain, Umesh Kumar1,Gayatri G Ekbote2, Latika Gupta, Ritu Raj1, Anupam Guleria, Rajiva Gupta2, Ramnath Misra1, Dinesh Kumar1; Department of Clinical Immunology and Rheumatology, SGPGIMS,1Centre of Biomedical Research, Lucknow, Uttar Pradesh,2Medanta-The Medicity, Gurgaon, Haryana, India

Introduction: Takayasu arteritis (TA) and Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) predominantly affects large and small vessel respectively. Biomarkers are invaluable tools in clinical medicine that aid in disease diagnosis, understanding disease pathogenesis, predicting prognosis, and response to therapy. The oxidative stress is the hallmark of inflammatory state and studies suggest that histidine has strong anti-oxidative and anti-inflammatory effects whereas tyrosine has a limited role. Based on this, we hypothesized that the circulatory Histidine/Tyrosine ratio (HTR) will be lower in AAV (considering the extent of inflammation) compared to TA.

Objective: The aim is to compare the circulatory HTR values in TA and AAV patients.

Methods: The serum metabolic profiles of 53 AAV and 98 TA patients (satisfying their respective American College of Rheumatology (ACR) classification criteria) and 77 healthy controls (NC) were measured using 1D 1H-CPMG NMR experiments at 800 MHz NMR spectrometer and analyzed using CHENOMX (w.r.t formate as an internal reference) and compared.

Results: The median age in AAV and TA groups was 46.5 years and 27 years respectively. Female to male ratio was 1.79 in the AAV group and was 4.16 in the TA group [Figure 1]a. Circulating HTR levels were significantly decreased in AAV and TA patients compared to NC [Figure 1]b but were lower in AAV when compared with TA. Further, the receiver operating characteristic (ROC) curve analysis showed good sensitivity and specificity with AUROC equal to 0.8 [95% CI=0.72-0.87] [Figure 1]c.
Figure 1: (a) Clinical and demographic characteristics of AAV, TA and NC subjects. (b) Box NC. (c) Receiver operating characteristic curve analysis performed for evaluating the diagnostic potential of circulatory HTR. Highlighting the diagnostic value of circulatory HTR for differenctiating Anca and TA patients

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Conclusion: This proof of the concept study demonstrates that circulatory HTR level has the potential to serve as a surrogate biomarker for differentiating TA from AAV and might aid in screening TA patients with small vessel involvement, understand the pathogenesis and guiding the disease treatment. Limitation – Needs validation and larger cohort.


  PB0025: Correlation of serum calprotectin level with C – reactive protein and jadas – 27 score in children with juvenile idiopathic arthritis Top


F Remthangpuii, Anu Maheshwari, Jagdish Chandra, Sunita Sharma, Deonath Mahto; Lady Hardinge Medical College and Associated SSK and KSC Hospitals, New Delhi, India

Background: Serum Calprotectin is a new inflammatory marker that can be used as a marker for disease activity in patients with JIA. We hypothesized that there is a correlation between Disease Activity Score (JADAS-27) and serum levels of Calprotectin and CRP in children with JIA.

Objectives: To correlate Disease Activity Score (JADAS-27) with serum levels of Calprotectin and quantitative C-Reactive Protein (CRP) in children with Juvenile idiopathic arthritis(JIA).

Methods: This cross-sectional observational study was conducted in Department of Pediatrics, Pediatric Rheumatology and immunology clinic and Department of Pathology, Lady Hardinge Medical College, New Delhi from November 2017–March 2019. Fifty patients with Juvenile Idiopathic Arthritis (JIA) attended in rheumatology and immunology clinic were enrolled. BioVendor Human S100A8/A9 ELISA was used for determination of serum Calprotectin level. CRP Ultra EIA is used for quantitative determination of CRP.

Results: The mean serum Calprotectin level was 42,284.5±14,129.98 ng/ml with minimum and maximum values being 8,560ng/ml and 63,160ng/ml, respectively. The mean Q–CRP was 40.14±37.14 mg/l, and the minimum and maximum values being 0.02 mg/l and 107.4 mg/l, respectively. The mean JADAS-27 score was 13.42±8.12, 0 and 32 being minimum and maximum values, respectively. Spearman correlation demonstrated a positive correlation between serum Calprotectin and JADAS-27 score (r=0.418), and p value was 0.003, which indicated a statically significant association between the two. There was also a positive correlation between Q-CRP and JADAS-27 score (r = 0.619), and the correlation was statistically significant (p=0.000). We also found a positive correlation between serum Calprotectin and Q – CRP (r=0.489), and the association was statistically significant (p=0.000).

Conclusions: Disease Activity Score (JADAS-27) correlate with serum levels of Calprotectin and Q-CRP, and the correlations were statistically significant.


  PB0026: TLR7/9 expression and humoral response against Epstein barr virus in systemic sclerosis Top


Sanghamitra Machhua, Shefali Khanna Sharma2, Yashwant Kumar, Sanjeev Handa2, Surjit Singh3, Shashi Anand, Ranjana W Minz; Departments of Immunopathology,1Internal Medicine,2Dermatology, Venerology and Leprolosy and3Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Objective: To analyze TLR7/9 expression and Humoral response against Epstein Barr virus in Systemic sclerosis

Materials and Methods: EBV viral load detection was done by absolute quantification using Taqman probe based real time PCR. Antibodies of class IgG and IgM against EBV was detected by Immunoblotting (Euroimmun, Germany), With the EUROLINE Anti-EBV Profile 2 (IgG and IgM –Anti VCA gp125,VCA p19, EBNA-1, p22, EA-D) and the Results: were interpreted using EUROLINE Scan software. Gene expression of TLR-7, TLR-9 was analyzed by PCR array based on SYBR green chemistry using real time PCR (Qiagen).

Results: EBV was not detected in peripheral blood of 49 SSc patients out of 50 patients, whereas in 50 age matched controls, 10 were positive. Out of 48 SSc patients 33 had antibody profile suggestive of late phase infection whereas 14 were suggestive of reactivation phase, while in controls 23 had late phase infection but none of them were in reactivation phase. Significant down regulation of EBV receptors, TLR 7 and TLR 9 was observed in SSc patients as compared to controls [Figure 1].
Figure 1: TLR 7 and 9 gene expression

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Conclusion: EBV and systemic sclerosis have a unique relationship. Humoral response to EBV antigens was strong in patients detected with re-activation phase, but the virus was not detected in peripheral blood by Real time PCR .There are however foot prints of the virus in terms of down –regulation of EBV receptor TLR 7 and TLR 9 in peripheral blood cells .We postulate that the virus hides in the niche of skin and endothelial cells where it triggers pro-fibrogenic environment, which is needs to be further explored in near future.


  PB0027: Identifying endothelial dysfunction through circulating endothelial cells in patients with ankylosing spondylitis: a tertiary centre pilot study Top


Ayindrila Saha, Aniruddha Bagchi, Sumantro Mondal, Sanchaita Misra, Dipanjan Bhattacharjee, Sulagna Chatterjee, Sudipta Chatterjee, Parasar Ghosh, Alakendu Ghosh; Department of Rheumatology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

Background: Ankylosing spondylitis(AS) is characterised by inflammatory back pain with evidence of radiographic sacroiliitis. Impaired endothelial function is a harbinger of cardiovascular complications in AS patients. The endothelial cells, lining the entire vascular system, are detached from the vessel wall as an immediate consequence of compromised endothelial integrity and can be detected as circulating endothelial cells (CEC) in the peripheral blood.

Objectives: To investigate the relationship between endothelial function and CECs in AS patients in a cross-sectional study.

Methods: CECs were characterized and quantified sourced from peripheral blood of patients with AS (n=15) and age and sex matched healthy controls (n=10). Flow-mediated dilatation (FMD) was assessed for all subjects. Disease activity was evaluated using Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and functional ability was monitored using Bath Ankylosing Spondylitis Functional Index (BASFI). All subjects were free of any other known traditional CV risk factors.

Results: Frequency of CECs in AS patients was upregulated compared to healthy controls being 2.54(1.640-4.210) vs 0.33(0.07-0.56), p<0.001, along with reduced FMD% being 14(5.9-17.50) vs 18.26(11.86-25.25), p<0.05). Frequency of circulating endothelial cell population was negatively correlated with FMD%, r=-0.56, p<0.05, but positively correlated with disease duration, r=0.7, p<0.05 and & to some extent with disease activity, BASDAI.

Conclusion: A proportionate amount of CECs were found to be present in patients with AS compared to healthy controls, denoted a high degree of endothelial damage which was further supported by reduced FMD in this population. Frequency of CECs were directly proportional with disease duration and BASDAI and inversely proportional with FMD%. These circulating endothelial cells may be sloughed from the vascular lining under conditions of systemic inflammation. Thus elevated frequency of CECs can be a potential bio-marker for early stage of endothelial damage in patients with ankylosing spondylitis.


  PB0028: Urinary IL-36 γ in urine: A new potential marker of active lupus Top


Pratibha Singh, Sanjukta Majumder, Amita Aggarwal; Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India

Background: IL-36 is a new member of the IL-1 family and has inflammatory properties. Recent studies have shown elevated serum levels of IL-36α and IL-36γ in patients with Systemic Lupus Erythematosus (SLE). As no data is available on urinary levels of IL-36α and IL-36γ in SLE, we studied these in patients with SLE including lupus nephritis (LN).

Objective: To identify role of IL- 36 by studying urinary levels of IL-36 in SLE patients by ELISA.

Methods: Urine samples were collected from 106 patients with SLE (active LN (ALN)=68, inactive LN (ILN)=20, active non-renal (ANR)=18) and 10 healthy subjects (HC) after informed consent. Urinary IL-36α and IL-36γ levels were measured by ELISA (R&D Systems) and are expressed as pg/ml and as median (IQR).

Results: Out of 106 patients 94 were females. Their mean (SD) age was (28.3(9.7)] years, duration of disease 26.3(44.1) months and SLE disease activity index (SLEDAI) score 10.3(7.5).

None of the healthy control subjects had detectable IL-36γ levels in urine. Median IL-36γ levels in patient's urine were significantly higher than healthy controls (P<0.01). IL-36γ levels in urine of ALN [4.1 (42.1)] patients were also significantly higher than ILN [0(8.3), P<0.05] and ANR [0(7.7), P<0.05] patients. Urinary IL-36γ level had moderate positive correlation with SLEDAI score (r= 0.31, P<0.002).

Two healthy controls had IL-36α detectable in the urine. Median IL-36α levels in patient's urine were significantly higher than healthy controls (P <0.01). The median IL-36α levels in ALN [3.6 (35.8)] were no different than ILN [10.5(28.4)] and ANR [30.2(75)] patients.

Conclusion: Both IL-36α and IL-36γ levels were higher in SLE, however only IL-36γ correlates with lupus activity. In addition, IL-36γ levels were higher in active nephritis as compared to inactive nephritis patients.


  PB0029: Identification of disease-associated specific protein to understand RA pathogenesis and diagnosis development Top


Monu, Prachi Agnihotri, Uma Kumar1, Sagarika Biswas; CSIR-Institute of Genomics and Integrative Biology,1All India Institute of Medical Sciences, New Delhi, India

Background: Rheumatoid arthritis is a chronic, autoimmune and systemic rheumatic disease with advanced inflammation in or around of one or more joints. RA diagnosis at early stage have potential to increase chance for successful treatment, whereas, delays can alter treatment process at late stage of disease presentation. In spite of availability of diagnostic markers for RA, the seropositive and seronegative patient have been ascended resulting into difficulties in diagnosis conditions



Objectives: Identification of a disease-associated specific differential protein/marker to improve RA diagnosis.

Methods: The plasma proteins profiling of RA was performed using combination of two-dimensional electrophoresis (2-DE) and LC-MS/MS techniques. The plasmatic expression of identified proteins was validated using Western blot and biochemical assay.

Results: Preliminary, 11 protein spots were identified using 2-DE and LC MS/MS analysis. Amongst differentially expressed proteins, a transthyretin (TTR) protein was selected and validated in RA plasma. The Western blot densitometric analysis reveals upregulation of TTR in RA (n=10) plasma compared to healthy control (n=10) with a significant difference of p=0.0112. Furthermore, to determine expression consistency of TTR, ELISA of RA patient's plasma (n=72) and healthy control (n=37) was analysed, indicating the up-regulation of TTR compared to control with an odd ratio of 1.44 at significant difference (p<0.0014).

Conclusions: The distinctive identification and differential expression inflammatory proteins/markers can reflect a late stage of disease, so measuring disease activities at the early stage would be promising to diagnose RA. In our understanding the expression of TTR increased with disease severity and their systematic investigation of these altered proteins may act as a complementing therapeutic tool for RA management.
Table 1: List of few identified plasma proteins from rheumatoid arthritis patients

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  PB0031: Does gut inflammation correlate with disease activity in non-IBD spondyloarthritis? Top


Sakshi Mittal, A K Jain1, Varun Dhir, Sanjay Jain, Shefali K Sharma; Department of Medicine, Clinical Immunology and Rheumatology Wing, PGIMER,1Department of Gastroenterology, PGIMER, Chandigarh, India

Background: Studies have demonstrated subclinical gut inflammation in more than half of Spondyloarthritis (SpA) patients(1). It has been hypothesized that gut inflammation has a role in disease pathogenesis(2). Moreover, gut inflammation seems to co-relate with SpA disease activity and sacro-iliac joint marrow oedema(1,3). Calprotectin is the most abundant protein in neutrophils(4) and faecal calprotectin is a well-established marker of gut inflammation(5,6).

Objectives: To evaluate faecal calprotectin as a marker of disease activity in Spondyloarthritis.

Methods: Axial or peripheral SpA patients without known IBD or loose stools/ blood in stools for last 3 months were enrolled. Faecal calprotectin estimation was done by ELISA (cut off – 43 m/g). BASDAI was calculated at the same visit and ESR and CRP were estimated. NSAID index score was calculated for the last 3 months of NSAID intake as per ASAS recommendations(7).

Results: Faecal calprotectin was tested in 301 patients of axial and peripheral SpA. Disease was active (BASDAI > 4) in 50 (16.6%) patients. Faecal calprotectin was raised in 68 (22.59%) patients. We found a significant association between elevated faecal calprotectin and BASDAI, ESR and CRP. Faecal calprotectin was not affected by age, sex, disease duration, HLA B-27 positivity, previous NSAID intake or sulfasalazine use. Through the ROC analysis faecal calprotectin level of 43 m/g could predict active spondyloarthritis (BASDAI > 4) with a sensitivity of 32% and specificity of 83.5%.

Conclusion: There is a significant correlation between gut inflammation and disease activity in axial and peripheral spondyloarthritis.
Table 1: Association of elevated faecal calprotectin with spondyloarthritis parameters

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  PB0032: In vitro characterization of treg cells isolated from peripheral blood of rheumatoid arthritis patients Top


Vallayyachari Kommoju, K G Chengappa, V S Negi; Department of Clinical Immunology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India

Background: Present study was designed to understand the influence of synovial inflammatory milieu on peripheral blood Treg cells in patients with Rheumatoid Arthritis (RA).

Methods: The Peripheral Blood (PB) and synovial fluid (SF) of RA (n=80) and OA (n=30) patients were analyzed for CD4+T-cell subset frequencies and phenotypes by flow cytometry. Cytokine concentrations in plasma and SF were measured by cytometric bead array. Tregs from 5 RA-PB were isolated and cultured in autologous synovial fluid for 24 hrs. Phenotypic expression of Th1 and Th17 chemokines on the cell surface were analyzed by flow cytometry and expression levels of T-bet, RORγ and FOXP3 in the cultured Treg cells were measured with quantitative real-time PCR (RT-qPCR).

Results: The PB and SF frequencies of Th1, Th17 and Tregs. The pro-inflammatory cytokines were high in the plasma and SF of RA but the anti-Inflammatory cytokines were similar [Figure 1]a and [Figure 1]b. Treg cells isolated from PB and cultured in autologous SF of RA showed increased cell surface expression of CXCR3+ and CCR6+ [Figure 1]c. Gene expression studies showed an increased expression of T-bet, RORγ and decreased expression of Foxp3 after in-vitro culture [Figure 1]d.
Figure 1: (a) comparison of plasma cytokine levels between of RA and OA patients; (b) comparison of synovial fluid between RA and OA patients; (c) surface characerzation of in vitro cultured peripheral blood treg cells of RA patients. C1 and C2 CXCR3 (Th1) snf VVR6 (Th17) chemokines expressing treg cells isoloated from peripheral compartment of RA patients; C3 abd C4 CXCR3 (Th1) and CCR6 (Th 1) and CCR6 (Th17) chemoking expressing treg cells cultured in autologous synovial fluid. Expression of (D1) T-bet, (D2) ROR-g, and (D3) Foxp3. Mamm–Whitney U-test was used to analyze cytokine data, unpaired Student's t-test was used to analyze treg frequency data, paired t-test was used to analyze gene expression data; *P < 0.05 considered significant

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Conclusion: Tregs in RA are converted to Th17 phenotype on exposure to inflammatory cytokine in the synovial fluid, thus losing their regulatory functions. Understanding factors influencing stability of Treg cells may help improve future therapeutics.

https://drive.google.com/file/d/13BD0-E4wRVRcRCw3PvITMd4t250RqVH1/view?usp=sharing


  PC0001: Validation of Hindi version of the Pittsburg sleep quality index Top


Anil Kumar, Rohini Handa, Sundeep Kumar Upadhyaya, Sirinder Jit Gupta, Bindhyachal Kumar Gupta; Department of Rheumatology, Indraprastha Apollo Hospital, New Delhi, India

Objectives: Pittsburg sleep quality index (PSQI) is used to assess subjective sleep quality in different population groups over the last one month. There is lack of suitable instrument to assess sleep quality in Hindi speaking population. We aimed to translate and assess the reliability and validity of the Hindi translated version of the Pittsburgh Sleep Quality Index (PSQI-H).

Methods: We translated and assessed the reliability and validity of the Hindi translated version of the Pittsburgh Sleep Quality Index (PSQI-H). The PSQI-H was developed from PSQI according to the following steps: (a) translation, (b) back-translation, (c) comparison between translation and back-translation, and (d) pre-pilot test in intended population. The PSQI-H was applied to 105 bilingual individuals knowing Hindi and English. The internal consistency of PSQI-H was assessed by Cronbach's alpha. For test-retest reliability assessment, intraclass correlation coefficient (ICC) was measured between PSQI-H at baseline and PSQI after 4 weeks. Pearson's coefficient was used to assess the correlation between the score of the questions and the PSQI-H scores.

Results: The seven components of PSQI-H shows an acceptable level of internal consistency with Cronbach's alpha of 0.776. There is good test-retest reliability between PSQI-H and PSQI as measured by ICC of 0.979. The score of individual items and global scores of PSQI-H were highly correlated with each other (p< 0.001). The mean of the seven individual components score and global scores of PSQI-H at baseline and original PSQI after 4 weeks did not differ significantly.

Conclusion: This study Results demonstrate that PSQI-H is a valid and reliable instrument for the assessment of sleep quality in Hindi speaking population.


  PC0002: A study on estrogen level in systemic lupus erythematosus patients and it's co-relation with the disease activity Top


U Faruque, P Dihingia, A K Jha; Assam Medical College and Hospital, Dibrugarh, Assam, India

Introduction/Background: Systemic Lupus Erythematosus (SLE) is an auto-immune disease. There are evidences of hormone effect like estrogen, genes on X chromosome and epigenetic differences between the genders playing a role. Supporting to the hormonal factor there are studies showing that women using estrogen containing OCPs or hormone replacement therapy have increased risk of developing SLE.

Objectives: This study was undertaken to know the co-relation between serum estrogen level and the disease activity.

Materials and Methods: A total of 123 patients visiting the OPD of AMCH, Dibrugarh were taken up for study after applying the inclusion and exclusion criteria. Their SLEDAI score was calculated and simultaneously estrogen level was also taken. A proforma was filled which also had the menstrual history of the patient.

Results: Male:Female was 1:24. 52% had duration of disease less than 1 year. Most common symptom was malaise seen in 47% patients. 49% patients were in follicular phase of their cycle. 60% patients belonged to moderate disease activity group (SLEDAI 4-12). On looking the overall co-relation between SLEDAI score and estrogen level, with increasing disease activity the estrogen level increased but on taking the mean of each SLEDAI group, there was a dip in the estrogen level in the severe disease activity group.

Conclusion: With increase in estrogen level the disease activity increases but only till the disease activity is of moderate nature. Once severe disease activity sets in there is a decline in the estrogen level. It may be due to the suppression of the hypothalamic-pituitary-gonadal axis suppression during severe disease activity. Also, the aromatase enzyme levels are supressed leading to decreased estrogen synthesis.
Table 1: Mean value of estrogen in various groups

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  PC0003: Development of an Indian brief international classification of functioning, disability and health core set for rheumatoid arthritis: From patients and health care professionals' perspective Top


M Suja Sathya Dharshini, Samuel Kamalesh Kumar, R Aruna, Ronald Thomvic Maradona, Raji Thomas, John Mathew; Christian Medical College, Vellore, Tamil Nadu, India

Background and Objective: An ICF Core Set (ICF-CS) is the assemblage of necessary categories which are relevant to elucidate the functioning of a person with a specific health condition. The comprehensive core set is vast and time consuming to be performed in an outpatient setting. The brief core set can be used in variety of settings but geographic and socio-cultural specific core sets need to be developed for accuracy in assessment and treatment. Therefore, our study was designed to develop a brief “Indian ICF Core Set for RA” from the perspective of patients and health professionals.

Methods: Participants include 106 patients with RA and 46 health care professionals who were providing services to patients with RA. A cross sectional survey was done in our tertiary care teaching hospital. An open questionnaire and ICF-CS based questionnaire were given to patients and health care professionals to identify the most important problems that patients with RA experience.

Results: 32 categories from the open questionnaire and 32 categories from the ICF-CS based questionnaire were obtained. The final brief “Indian ICF core set” was formulated by combining both the approaches which resulted in 41 categories. The majority of categories were from the ICF-CS component - activities and participation (22 categories, 54%).There were dissimilarities observed between the response of patients and health care professionals. Only 15 (37%) categories were reflected in our Indian brief ICF-CS compared to the original brief ICF core set demonstrating the geographic and socio-cultural differences in our population.

Conclusion: A brief ICF core set for patients with RA from the perspectives of patients and health care professionals was developed for Indian population.


  PC0004: Lupus is associated with worse outcomes after hip arthroplasty Top


Jasvinder Singh, John Cleveland; University of Alabama at Birmingham, Birmingham, Alabama, USA

Background/Objective: Hip osteonecrosis and hip osteoarthritis are common causes of severe hip disease in lupus, both treated successfully with a total hip arthroplasty (THA). To our knowledge, comprehensive analyses for specific outcomes such as infection, revision or associated health care utilization after THA are lacking. Therefore, our objective was to assess the risk of specific post-THA outcomes, i.e., infection, transfusion, revision and mortality and associated health care utilization, associated with lupus.

Methods: We used the 1998-2014 U.S. National Inpatient Sample data. Multivariable-adjusted separate Cox proportional hazard regression models assessed the association of lupus with post-operative complications (infection, transfusion, THA revision and mortality) and healthcare utilization outcomes (total hospital charges, discharge to inpatient facility, length of hospital stay) post-THA, adjusting for demographics, underlying diagnosis, comorbidity, insurance payer, and hospital characteristics, using hazard ratios (HR) and 95% confidence intervals (CI).

Results: Among 4, 116, 485 primary THA hospitalizations, 22,557 (0.5%) were in lupus patients. Patients with lupus were younger, more likely to be female, African-American or Hispanic and, have higher comorbidity, Medicaid insurance payer, lower income, or living in the South. In multivariable-adjusted analyses, lupus was associated with a significantly higher risk of infection, transfusion, hospital charges above the median ($37,658) and discharge to inpatient facility, with respective HRs of 1.95 (95% CI, 1.28, 2.97), 1.34 (95% CI, 1.25, 1.43), 1.21 (95% CI, 1.01, 1.44) and 1.38 (95% CI, 1.30, 1.47). Lupus was not significantly associated with the risk of revision, mortality or hospital stay above the median (>3 days), the HRs were 1.10 (95% CI, 0.68, 1.78) and 0.95 (95% CI, 0.61, 1.47) and 1.06 (95% CI, 0.99, 1.13).

Conclusions: Lupus was associated with a higher risk of infection and transfusion and higher hospital charges post-primary THA. Insight into modifiable factors associated with these outcomes may improve outcomes in lupus patients undergoing THA.


  PC0005: Introduction of mini clinical evaluation exercise as a mode of assessment for postgraduate students in medicine for examination of sacroiliac joints Top


Anuj Singhal; AFMC, Pune, Maharashtra, India

Background: Formative and subjective assessment of medical education is the need of the hour today. Mini clinical evaluation exercise (mini- CEX) and directly observed procedural skills (DOPS) are the commonly used workplace-based assessment (WPBA) tools and have been extensively studied in both undergraduate and post graduate setting. In this study we tried to assess the feasibility of mini-CEX and also its acceptability amongst postgraduate students of Internal Medicine for examination of Sacroiliac joints and teaching faculty.

Methodology: This is an interventional study which was carried out in a Government Medical college in Pune, Maharashtra from Jan to Jun 2019. A total of 18 first- and second-year postgraduate students and 9 teaching faculty from the Department of Internal Medicine participated in this study. Each student underwent 5 Mini-CEX evaluation over a period of 6 months under different teaching faculty. Feedback was taken from both the teaching faculty and students regarding the feasibility of Mini-CEX as an assessment tool.

Results: A total of 90 mini-CEX exposures involving 18 post graduate students and 9 faculty were analysed. We found a statistically significant improvement in the domain of medical interviewing (p value< 0.001), physical examination (p value= 0.003), professionalism (p value =0.001), clinical judgement (p value = 0.003), counselling skills (p value<0.001) and organizing efficiency (p value< 0.001). Overall clinical competence improved from a scale of 5(1.7) to 6.7 (0.8).

Conclusion: The Results: of this study proved that overall Mini-CEX is an acceptable and effective assessment tool. However, regular training of assessors through workshops on provision of effective feedback is required. Modification of the assessment form based on the feedbacks provided by teachers and students will further facilitate the implementation of this teaching tool in the curriculum.

Keywords: Medical education, mini clinical evaluation exercise, qualitative study, sacroilitis, workplace based assessment
Figure 1

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  PC0006: Correlation of sex hormones with disease activity in premenopausal females with rheumatoid arthritis Top


Pulin Kumar Gupta, Pankaj Gupta, Saurabh Tyagi, Manita Khatak, Lokesh Sharma; Department of Medicine Subdivision Rheumatology, PGIMER Dr RML Hospital, New Delhi, India

Introduction: Rheumatoid arthritis (RA) is an inflammatory disease involving multiple organ systems and the gonadal system is no exception. The relationship of sex hormones with the pathogenesis of RA is well understood however the same with disease activity is still not clear and hence the present study was planned.

Methods: It was a cross-sectional observational study done in 100 premenopausal females with definite RA. All cases with history of cardiac, renal, hepatic, metabolic disorders, biological or hormonal therapy anytime in the past or corticosteroids intake within last three months were excluded. Cases were subjected to detailed evaluation in early follicular phase of menstrual cycle.

Results: The mean age of the cases was 35.2 ± 5.35 years with mean duration of disease 4.6 ± 1.8 years. The mean DAS-28 was 3.53 ± 1.6. The proportion of cases with mild, moderate and high disease activity were 25%, 29% and 11% respectively and 33% were in remission. 12.5% of cases had RA associated deformities. 36% of females had symptoms of hypogonadism out of which nearly 50% had definite ovarian failure. A significant association was seen between female hypogonadism and exposure to methotrexate for more than three years.

A statistically significant direct correlation was seen between DAS-28 (as a marker of disease activity) as a dependant variable and serum prolactin (<0.002), estradiol (P<0.013), progesterone (P<0.005), FSH (P<0.02), LH (P<0.005) and anti ccp (P<0.005) as independent variables, while serum testosterone (p<0.016) and DHEAS (p<0.008) showed a significant inverse correlation with DAS-28. However higher VAS was numerically seen more in cases with lower levels of serum estradiol and progesterone.

Conclusion: Female hypogonadism is common in patients with RA and disease activity is directly associated with higher levels of gonadotropins and female sex hormones and lower levels of serum testosterone and DHEAS.


  PC0007: Axial spondyloarthritis: MRI inflammation and disease activity in relation to smoking in an Egyptian cohort Top


Fatma Fayed, Mona Helmy, Mohamed Barakat, Abeer Abdelati; Department of Rheumatology and Immunology, Alexandria University, Alexandria, Egypt

Background: literature suggests that smoking is one of the crucial triggering factors of rheumatological diseases. (1) In axial Spondyloarthritis (axSpA), classified into radiographic SpA (AS) and non-radiographic SpA (nrSpA), smoking associated with disease activity and extra-articular manifestation. (2) The relationship between smoking and HLAB-27 as well as MRI inflammation in axSpA patients and the difference between nrSpA and AS regarding smoking have not been studied to date in details.

Objective: to investigate the influence of smoking on disease activity and MRI inflammation in axSpA patients (AS and nrSpA).

Methods: sixty Egyptian patients (42 males and 18 females) with the mean age (31.33 ± 7.02), with early active axial spondyloarthritis (49 AS and 11 non-radiographic SpA) within two years disease duration, diagnosed based on ASAS classification criteria. All clinical indices (BASDI, BASFI, BASMI, ASDAS-CRP) were applied to all patients. HLA-B27 and the inflammatory markers (ESR, CRP) was done. MRI of sacroiliac joints was performed in a standard protocol using short tau inversion recovery and T1 sequences (slice thickness 3-4mm, both semi-coronal and semi-axial orientations), and scored by the Berlin method. Smoking use assessed by smoking pack-year index.

Results: of all 60 patients, 38 smokers and 22 non-smokers. No significant difference regarding smoking packs index between nrSpA and AS. (p=0.822) There was a robust correlation between smoking packing index and the Berlin score of MRI in all axSpA patients (rs=0.631) (p=<0.001). Moreover, there was a significant correlation between smoking and C-reactive protein (rs=0.952) as well as HLA-B27. (rs=0.340) (p<0.001) Furthermore, a significant relationship between smoking and activity indices (BASDI (rs=0.961) and ASDAS-CRP (rs=0.938)). Otherwise, no significant correlation among smoking, BASMI, and BASFI as well as ESR.

Conclusion: Smoking has a significant association with MRI inflammation, disease activity scores as well as HLA b27 postivity


  PC0008: The early effect of non-steroidal anti-inflammatory drugs on progression of newly diagnosed axial spondyloarthritis Top


Fatma Fayed, Mona Helmy, Mohamed Barakat, Abeer Abdelati; Department of Rheumatology and Immunology, Alexandria University, Alexandria, Egypt

Objectives: We aimed to evaluate the early effect of continuous treatment with optimum dose of NSAIDs on disease activity and radiographic progression of newly diagnosed axial spondyloarthritis (axSpA).

Methods: A six-week prospective study on thirty consecutive newly diagnosed active axSpA patients. All patients were assessed at baseline visit, a follow-up visit after 2 weeks, and after 6 weeks of treatment with a continuous optimal dose of NSAID. Assessment included calculation of ASAS NSAIDs index, inflammatory markers, patient's global assessment, physician's global assessment, quality of life index, as well as pain scale. Disease activity was assessed by determining BASDAI and ASDAS, while functional assessment was evaluated by using BASFI. Spinal mobility was assessed by the mean improvement in BASMI. Magnetic Resonance Imaging of sacroiliac joints was taken at baseline and at the end of the study and was evaluated according to Berlin scoring method.

Results: We observed improvement in Berlin MRI score, laboratory markers, as well as other clinical parameters including disease activity, spinal mobility, and pain scale. Furthermore, at week 6, ASDAS clinically important improvement and ASAS40 were achieved in 73.3% and 63.3% of patients, respectively. Additionally, BASDI50 was achieved in 30% and 56.7% of patients at week 2 and week 6, respectively.

Conclusion: A continuous intake of optimal dose of NSAIDs improves not only all clinical indices and inflammatory markers, but also the radiographic signs of activity in axial spondyloarthritis. Moreover, the higher the NSAID index the lower the radiographic progression.


  PC0009: Beliefs about medications in very early rheumatoid arthritis: Indian patients are indifferent about their medication Top


Ankan Patel, Arup Mahapatra, Prasanta Pradhan, Sakir Ahmed; Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India

Background Drug compliance is a major determinant of disease control in rheumatoid arthritis(RA). Various studies have shown that drug compliance depends on patients' beliefs towards medicine. But all of these studies are in established disease where the disease itself could have modified the patients' beliefs!
Table 1: Maximum possible scores, medians (quartiles) and percentage of high scorers obtained from the Belief about Medicines Questionnaire

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Objective: To determine the attitude of patients towards medicines in very early RA(<3 months).

Methods: 250 patients who had developed arthritis within last 3 months and who fulfilled the ACR/EULAR2010 criteria for RA were included. Demographic and clinical data were collected. Patients filled the Belief about Medicines Questionnaire(BMQ). The BMQ has 4 subsets: Specific necessity, Specific concern, General overuse and General harm. For these scores, more than scale mid-point is considered high. Depending on the Specific necessity and Specific concern scores patients can be classified as indifferent, accepting, sceptical or ambivalent.

Results: Mean (±SD) age was 47.6(±13.4) with 88.4%(221) females. 86 were graduates or above, 64 completed higher secondary school, 20 read till high school, 76 had primary education while one had no formal education.

12 (4.8%) had high specific necessity while 31 (12.4%) had high specific concern score. General overuse score high in 248 (99.2%). General harm score was high in 246(98.4%) patients. Thus, 242 (96.8%) patients were classified as indifferent, 4 (1.6%) accepting, 4 (1.6%) sceptical and none as ambivalent!

There were weak correlations (Spearman; r-square<0.1) between the subset scores, and there was no statistically significant correlations of these scores with age, income, educational status or occupation.

Conclusion: Though general harm and overuse scores were high, patients had low scores on the scales specific for RA medication. Thus most of them were classifiable as “indifferent” unlike previous studies in established RA. Further studies are required to assess if these beliefs change with increasing disease duration.


  PC0010: Ultrasound imaging in evaluation of small joints of hand in rheumatoid arthritis Top


P P Amritha, manisha Ashwin Daware, B N Kishore Kumar; Narayana Hrudayalaya, Bengaluru, Karnataka, India

Background: Rheumatoid arthritis (RA) is a common disease in our population which significantly affects the functioning capacity of patients. With early and adequate treatment it is possible to halt disease progression and prevent irreversible joint damage.

Objectives

  • To highlight the role of high frequency gray-scale and power Doppler ultrasound in small joints of hand in patients with RA and correlate with clinical and radiographic findings
  • To determine whether sonography can be used as a reliable and cost effective investigation in grading the severity of RA.


Methods: In this study, 47 early RA patients with disease duration less than 2 years were included. Rheumatoid factor (RF) and ESR of the patients were measured. Bilateral hand joints and wrists were examined by ultrasonography (US) and radiography. Sonographic examination included evaluation of wrists, the second, third, and fifth metacarpophalangeal (MCP) joints; second, third and fifth proximal interphalangeal (PIP) joints. Relationship between laboratory parameters, radiographic and US findings were analyzed.

Results: US was significantly more sensitive in detecting bone erosions compared to radiography. PDUS was positively correlated with ESR(P<0.01), but not correlated with RF and disease duration (P > 0.05). GSUS was positively correlated with RF (P<0.05) but not correlated with ESR and disease duration (P > 0.05). Bone erosion was positively correlated with disease duration, and RF (P < 0.05) and was not correlated with ESR (P>0.05).
Figure 1: Mild synovial thickening in the dorsal aspect of left wrist joint with mild articular margin erosions

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Conclusion: US is very valuable in the diagnosis of early RA in synovitis synovitis, joint effusion, tenosynovitis, and bone erosion. Ultrasonography and clinical and laboratory parameters had a great correlativity. In view of the advantages of low cost and convenience, ultrasound may be a better choice during early RA diagnosis.


  PC0012: Sleep quality in rheumatoid arthritis Top


Anil Kumar, Rohini Handa, Sundeep Kumar Upadhyaya, Sirinder Jit Gupta, Bindhyachal Kumar Gupta; Department of Rheumatology, Indraprastha Apollo Hospital, New Delhi, India

Objective: Poor sleep quality is one of the most common problem in rheumatoid arthritis patients and it significantly negatively affect the HRQoL. No study related to sleep quality in RA has been conducted in India. The aim of this study was to assess the sleep quality and factors influencing sleep quality in Indian RA patients.

Methods: This prospective questionnaire based cross sectional study enrolled, 80 patients of RA with age ≥18 years. A detailed history of basic demographic data was taken. Sleep quality was assessed by Hindi version of Pittsburgh sleep quality index (PSQI). Other patient related outcomes measured were VAS 100 for pain, DAS28 and CDAI for disease activity, HAQ-DI Indian version for function disability and modified Kuppuswamy's Socio-Economic scale 2018 for socioeconomic status. Patients were divided into good sleeper (PSQI was < 5.0) and poor sleeper (PSQI was ≥5.0) and these two groups were compared.

Results: The mean age of study participants was 49.05 ± 12.37 years and mean disease duration was 7.8 ± 6.32 years. 81% of study participants were female. The prevalence of poor sleep quality in present study was 67.50% and the mean global PSQI score was 6.52 ± 3.02. The mean VAS Pain, HAQ-DI and DAS28 were 49.49, 1.275 and 4.02 respectively. Poor sleepers had significantly higher DAS28 (4.48± 1.26 vs 2.79 ± 1.09; p=0.0001) and CDAI (20.41 ± 11.33; p=0.0001). HAQ-DI score and VAS pain score were also significantly higher in poor sleeper. Poor sleep quality was not associated with age, sex, disease duration, drugs used and socioeconomic status.

Conclusion: The majority of Indian RA patients have poor sleep quality. Poor control of disease activity and functional disability are associated with poor sleep.


  PC0013: Clinical profiling of Psoriatic arthritis: An observational study from Karnataka psoriatic arthritis cohort Top


K Chanakya, Chandrashekhar Srikanntiah1, Sharath Kumar2, Vikram Haridas3, Vijay Rao4, Ramesh Jois5, Manisha Daware6, Shweta Singhai7, B G Dharmanad5, Promod Chebbi8, R Subramanian9, Ashwini Kamath10, Uma Karjiigi11, Vikram Raj K Jain12, Chethana Dharmaplaiah13, Shiva Prasad14, C Srinivas15, J Ramya, Benzeeta Pinto, Harshini16, Mahendranath17, Vineeta Shobha; St. John's Medical College Hospital,1Chanre Rheumatology and Immunology Research Centre,2Columbia Asia Hospital,4Manipal Hospitals,5Vikram Hospital,6Narayana Health City,7Sakra Hospital,11Apollo Hospital,13Mahaveer Jain Hospital,14Aster CMI Hospital,15Fortis Hospital,16Sparsh Hospital,17Samarpan Health Centre, Bengaluru,3Arthritis Specialty Clinic, Hubli,8SDM Medical College, Dharwad,9JSS Medical College,12Apollo BGS Hospital, Mysore,10Yenepoya Specialty Hospital, Managlore, Karnataka, India

Introduction: Clinical patterns and disease activity burden of psoriatic arthritis (PsA) varies in different parts of the world. There are limited studies from the Indian subcontinent.

Aims: To study the cutaneous and articular profile of PsA and describe their disease activity with validated outcome measures.

Methods: This is a multicenter, cross-sectional, non-interventional study done in the Karnataka Psoriatic Arthritis Cohort (KPsAC). All consecutive PsA patients defined by CASPAR or expert diagnosis were evaluated using structured CRF over 6 months from 17 Rheumatology centers.

Results: Demographics and disease characteristics of 378 patients enrolled is depicted in [Table 1]. In 238 (63.1%) patients, psoriasis preceded PsA while in 52 (13.7%), PsA preceded psoriasis. Mean PASI score was 4.0±7.9, mild (0-5) skin disease in 295(79.3%) and severe (>10) skin disease noted in 41(11.0%). Mean DAPSA was 19.2±16.5. Patients in remission (DAPSA:0-4) being 67(19.3%), low disease activity (5-14) in 106(30.6%), moderate disease activity (14-28) in 86(24.8%) and high disease activity (>28) in 89(25.7%). Mean HAQ-DI is 0.28±0.41 with mild to moderate disability in 48.8%. MDA is achieved in 130(35%) of patients.
Table 1: Demographics and disease characteristics

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Conclusions: Arthritis precedes skin disease in about 14% of patients. Despite mild skin disease in majority, nearly half of the patients have moderate to severe joint activity. Mild to moderate functional disability is noted in almost half of our cohort.


  PC0014: Health assessment questionnaire- disability index in psoriatic arthritis is driven by inflammation: Observational data from Karnataka psoriatic arthritis cohort Top


K Chanakya, Chandrashekhar Srikanntiah1, Sharath Kumar2, Vikram Haridas3, Vijay Rao4, Ramesh Jois5, Manisha Daware6, Shweta Singhai7, B G Dharmanad5, Promod Chebbi8, R Subramanian9, Ashwini Kamath10, Uma Karjiigi11, Vikram Raj K Jain12, Chethana Dharmaplaiah13, Shiva Prasad14, C Srinivas15, J Ramya, Benzeeta Pinto, Harshini16, Mahendranath17, Vineeta Shobha; St. John's Medical College Hospital,1Chanre Rheumatology and Immunology Research Centre,2Columbia Asia Hospital,4Manipal Hospitals,5Vikram Hospital,6Narayana Health City,7Sakra Hospital,11Apollo Hospital,13Mahaveer Jain Hospital,14Aster CMI Hospital,15Fortis Hospital,16Sparsh Hospital,17Samarpan Health Centre,3Arthritis Specialty Clinic, Hubli,8SDM Medical College, Dharwad,9JSS Medical College,12Apollo BGS Hospital, Mysore,10Yenepoya Specialty Hospital, Managlore, Karnataka, India

Introduction: Psoriatic arthritis (PsA) is a chronic inflammatory disease with significant functional impairment. HAQ-DI is a reliable and validated outcome measure for a variety of arthritis including Psoriatic arthritis. Disease characteristics contributing to the functional limitation in PsA have rarely been reported in Indian population.

Objective: Current study aims to evaluate the Indian version of HAQ as an outcome measure in the assessment of patients with psoriatic arthritis (PsA) among those who have received immunosuppressive treatment for 6 months or more in Karnataka Psoriatic Arthritis Cohort (KPsAC).

Methods: The Indian version of HAQ was administered to all consecutive patients enrolled in KPsAC between April and August 2019. Clinical assessments were performed according to a specially designed CRF. The Indian HAQ comprised 12 questions (nine basic and three advanced ADL, on the standard HAQ format) relevant to the Indian population.

Results: From KPsAC cohort of 378 patients, 142(37.4%) were extracted, those on treatment with a rheumatologist for ≥6 months. Mean age of this cohort was 38.3(±15.6) years, half were men, and mean arthritis duration was 6.5(±6.9) years. The mean HAQ score was 0.24(±0.36), mild-moderate (0-1) in 64(46.0%) and moderate-severe (1-2) in 9(6.3%). Parameters contributing to Moderate- High HAQ-DI are depicted in table No.1. HAQ-DI was not different between gender and age groups. Duration of arthritis was not associated with HAQ. Tender joint count (r=0.29, 95%CI=0.13-0.43, p<0.0005), ESR(r=0.22, 95%CI=0.05-0.38, p=0.01) and CRP (r=0.27, 95%CI=0.10-0.42, p=0.001) had a minor correlation with HAQ.

Conclusion: Indian version of HAQ could be efficiently employed to assess outcomes in our cohort. In-spite of being on therapy for at least 6 months, over half of the patients had mild to moderate disability indicating the high burden of inflammation.
Table 1: Associations of Health Assessment Questionnaire

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  PC0015: Prevalence of co-morbidities in psoriatic arthritis and their impact on disease measures: an observational study from Karnataka psoriatic arthritis cohort Top


K Chanakya, Chandrashekhar Srikanntiah1, Sharath Kumar2, Vikram Haridas3, Vijay Rao4, Ramesh Jois5, Manisha Daware6, Shweta Singhai7, B G Dharmanad5, Promod Chebbi8, R Subramanian9, Ashwini Kamath10, Uma Karjiigi11, Vikram Raj K Jain12, Chethana Dharmaplaiah13, Shiva Prasad14, C Srinivas15, J Ramya, Benzeeta Pinto, Harshini16, Mahendranath17, Vineeta Shobha; St. John's Medical College Hospital,1Chanre Rheumatology and Immunology Research Centre,2Columbia Asia Hospital,4Manipal Hospitals,5Vikram Hospital,6Narayana Health City,7Sakra Hospital,11Apollo Hospital,13Mahaveer Jain Hospital,14Aster CMI Hospital,15Fortis Hospital,16Sparsh Hospital,17Samarpan Health Centre, Bengaluru,3Arthritis Specialty Clinic, Hubli,8SDM Medical College, Dharwad,9JSS Medical College,12Apollo BGS Hospital, Mysore.10Yenepoya Specialty Hospital, Managlore, Karnataka, India

Introduction: Co-morbidities frequently accompany Psoriasis and Psoriatic arthritis(PsA) and add to the disease burden. Indian studies on co-morbidities in PsA are sparse.

Aims: To identify the co-morbidity burden in patients with PsA and evaluate its impact on the disease activity measures.

Methods: This is a multicenter, cross-sectional, non-interventional study done in the Karnataka Psoriatic Arthritis Cohort(KPsAC) in which consecutive PsA patients were evaluated through a structured CRF from 17 Rheumatology centers. A comprehensive psoriatic co-morbidity index was calculated for all enrolled patients. Psoriasis Area and Severity Index(PASI), Disease activity in PsA(DAPSA), Health assessment questionnaire-disability index(HAQ-DI) were correlated with co-morbidity index.

Results: The mean age at diagnosis of 378 enrolled patients was 39.2±14.9 years, with male predominance(M:F=13:10.5). Mean duration of PsA in cohort is 5.2(±6.0) years and 150(39.5%) patients had one or more co-morbidities. The mean psoriatic arthritis co-morbidity index was 1.04±2.24. The most prevalent co-morbidities were hypertension(18.7%) followed by type II diabetes(13.9%), smoking(5%), PsA disease severity(3.43%), anxiety(3.16%), IHD & endocrine disease(2.63% each) and depression(2.37%). Others were present in <2% each of cohort. Family history of cardiovascular disease or stroke was present in 29(9.5%). Patients with co-morbidities had significantly longer duration of arthritis than those without co-morbidities(p=0.04). HAQ-DI was significantly more in PsA patients with co-morbidities(p=0.02). Co-morbidity index did not correlate with gender, DAPSA and HAQ-DI. Infections were found in 42(11.2%), of which skin was the commonest site accounting for 50% while other bacterial infections were seen in 14.2%. Seven patients needed hospitalization due to infections.

Conclusion: Nearly 40% of PsA patients have co-morbidities and their presence was associated with greater functional disability. Longer disease duration was associated with presence of co-morbidities. Detection and control of co-morbidities must be an integral part of PsA management.


  PC0016: Prevalence of low bone mineral density in patients of ankylosing spondylitis, correlation with disease activity and serum sclerostin levels Top


Akhil Pawan Goel, Anupam Wakhlu, Puneet Kumar; King George's Medical University, Lucknow, Uttar Pradesh, India

Background: In AS, two bone remodeling processes take place; pathological new bone formation in the cortical zone of vertebrae, the facet joints and excessive loss of trabecular bone in the centre of the vertebral body causing osteoporosis(OP).OP expressed as reduced BMD is a common complication in AS that has been shown to exist in mild and early disease. TNFα is responsible for induction of Dickkopf-1 and sclerostin, which downregulates bone formation by inhibiting Wnt and bone morphogenic proteins.

Objectives:

1) To study the prevalence of low BMD in AS

2) To correlate low BMD with disease activity

3) To correlate serum sclerostin and cytokines-TNFα, IL-17, IL-22 with clinico-radiological and laboratory parameters of disease activity and BMD

Methods: Fifty consecutive patients fulfilling the ASAS 2009 classification criteria for axial spondyloarthritis were enrolled; all other secondary spondyloarthropathies were excluded. An equal number of controls were recruited. Other causes of secondary osteoporosis were excluded. BASDAI, ASDAS-ESR and CRP,mSASSS were calculated. BMD was measured at A-P lumbar spine, neck of femur, lateral lumbar spine using DEXA. Serum sclerostin,TNF-α, IL-17A,1L-22 and PTH levels were measured by commercial ELISA kits according to the manufacturer's protocol.

Results: Mean BMD at various sites was significantly lower in patients. Patients having osteoporosis and low BMD at AP and lateral spine were 38% and 72% respectively; at neck of femur,20% and 68% of patients had osteoporosis and low BMD respectively. BMD at neck of femur had significant positive correlation with BASDAI. Serum sclerostin levels were significantly higher in patients and had significant negative correlation with mSASSS.

Conclusion: Study showed a high prevalence of low BMD using Z-scores and T-scores which was higher than the previous studies; BMD at neck of femur can be used in advanced disease when BASDAI is low; Low sclerostin has a role in formation of syndesmophytes.


  PC0017: Real-world experience of efficacy and patient reported outcome measures of tofacitinib in patients with active rheumatoid arthritis Top


Ramakrishna Rao Uppuluri, Maryam Younis, Archana Rani, Shashikala Arava, Datta Kumar, J Shivanand, C Satyavati; Sri Deepti Rheumatology Centre, Hyderabad, Telangana, India

Background: In Real-world practice, efficacy of the treatment of active rheumatoid arthritis (RA) is analyzed by several measurements such as Disease Activity Score (DAS). It is also essential to measure the Patient Reported Outcome Measures (PROMs) during the treatment.

Objective: The aim of the study is to analyze the efficacy and PROMs of Tofacitinib, a JAKinib (Janus Kinase inhibitor) in patients with active RA not responding to conventional, synthetic disease modifying anti rheumatoid drugs (csDMARDs) in routine clinical practice.

Methods: Data of 50 patients with active RA from a single centre in south India from January 2017 to 2019 was obtained using standardized formats at baseline, 1 and 3 months. Tofacitinib was added 5mg twice daily to the patients with inadequate response to csDMARDs.Mean change from baseline in Tender Joint Count (TJC), Swollen Joint Count (SJC), Patient Global Assessment (PGA) 0-100mm, ESR mm hr, DAS -28 and Health Assessment Questionnaire- disability index( HAQ-DI) were evaluated throughout.

Results: Forty nine (96%) among 50 patients were seropositive for RA (RF/ACPA); 44 (88%) were females, mean (range) age 51.9 (20-77) years with mean disease duration 9.4 (1-25) years. All the patients had active RA with TJC 13 (range 1-28), SJC 6 (1-25), ESR (mm/hr) 64 (8-132) and DAS 6.8 (2.4-8.9). Among the PROMs, PGA was 80 mm (10-100) and HAQ-DI 2.2 (1.3-3) before treatment.All the parameters pertaining to efficacy and PROMs were better with the addition of Tofacitinib seen as early as 1 month and better by 3 months [Figure 1].
Figure 1: TJC, SJC, HAQ-DI and DAS-28 before and after treatment

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Conclusion: Patients with active RA having inadequate response to csDMARDs showed good clinical response with addition of Tofacitinib. DAS and PROMs improved as early as 1 month and became better by 3 months. It needs further studies involving more patients to identify the importance of PROMs in Real-World clinical experience.


  PC0018: To study the correlation of clinical parameters and nerve conduction velocity test findings in rheumatoid arthritis patients with peripheral neuropathy Top


Daisy Dutta, Chitralekha Baruah, Subhajit Das; Department of Medicine, Gauhati Medical College and Hospital, Guwahati, Assam, India

Background: Rheumatoid arthritis (RA) is a chronic multisystem inflammatory illness causing erosive and destructive arthritis of multiple small and large joints with various extra-articular manifestations including peripheral neuropathy which can lead to significant functional limitations. Nerve conduction studies revealed peripheral neuropathy in RA patients without clinical signs and symptoms of neuropathy.

Objectives: 1) To study the prevalence and types of peripheral neuropathy in RA patients and to correlate the clinical parameters with nerve conduction studies. 2) To correlate neuropathy in RA patients with disease severity.

Methodology: 41 cases of RA (diagnosed as per ACR/EULAR criteria 2010) were enrolled in the study and were subjected to detailed clinical examination and electrophysiological test. The demographic and clinical parameters were compiled, and Chi-square test was applied for statistical analysis.

Summary of Results: Of the 41 RA patients, 20 (48.78%) had peripheral neuropathy electro physiologically. Only 9(45%) out of 20 had clinical symptoms of neuropathy while 11(55%) patients had subclinical neuropathy. Sensorimotor neuropathy was the most common form 12(60%) found in our study, followed by motor neuropathy 6(30%) and 1each of mononeuritis multiplex and entrapment neuropathy. Statistically significant association was found between presence of peripheral neuropathy and disease severity (DAS 28), rheumatoid factor positivity and acute phase reactants (ESR and CRP).

Conclusion: Peripheral neuropathy is one of the common extra-articular manifestation of RA. The present study shows high prevalence of subclinical neuropathy in RA patients with high disease activity. Hence, Nerve conduction velocity studies to be undertaken in RA patients when there is high degree of suspicion.


  PC0019: Evolution of mixed connective tissue disease: Results: from a single center - prospective observational study Top


S Karthikeyan, T N Tamilselvam, R Ramesh, S Mythili: Institute of Rheumatology, Rajiv Gandhi Government General Hospital, Madras Medical College, Chennai, Tamil Nadu, India

Background: MCTD is an evolving disease rather than one specific CTD. MCTD would more accurately be termed as undifferentiated autoimmune rheumatic disease.

Objectives: To assess the clinical profile and outcome of MCTD patients.

Methods: This study was conducted at Rajiv Gandhi Government General Hospital over 21 months between November 2017 to July 2019. Alarcon Segovia criteria was applied for diagnosis. Patients with diagnosis of MCTD (including new and old cases) were followed up periodically, based on clinical and immunological profile to assess the phenotypic change in course of the disease.

Results: Study population consists of 30 patients, most of them are females except one male patient and 2 childhood cases. In our study most common clinical presentation was Raynaud's phenomenon (96%) followed by polyarthritis (86%), puffy fingers (86%), PHT (70%), ILD (66%), myositis (60%), gangrene (6%), peripheral neuropathy (3%). None of the patient had renal involvement. PHT was severe in nature in our study. UIP (63%) was the most common ILD pattern followed by NSIP (3%). Among 2 childhood cases one patient presented with Raynaud's phenomenon, myositis, polyarthritis, ILD/PHT, second case presented as PUO. In our study follow up 90% of patients stayed as MCTD without progression to another well-defined connective tissue disease. Among 30 patients, 4 (13%) progressed to another well-defined CTD (2 patients evolved into Limited SSc, one as SS, another one as SLE) after a mean disease duration of 3.4 years for SSc,2.8 years for SS, 6.7 years for SLE.

Conclusion: It is mandatory to have a long term follow up of MCTD patients to know the phenotypic stability of the disease. No need for all MCTD patients to progress to another CTD. There is a possibility that disease can remain as MCTD or even can go into remission.


  PC0020: High disease activity at onset is not associated with chronicity in chikungunya arthritis Top


Benzeeta Pinto, Anu Mohan Desai, Farha Farruqh, Ramya Janardana, Kodishala Chanakya, Vineeta Shobha; Department of Clinical Immunology and Rheumatology and Radiology, St John's Medical College, Bengaluru, Karnataka, India

Introduction: Chikungunya fever is a remerging disease is India and may lead to post chikungunya inflammatory rheumatism.

Objective: To assess the clinical and ultrasound features of chikungunya arthritis in the subacute phase and determine if high disease activity is associated with chronic disability.

Methods: We conducted a prospective observational of all patients who presented with subacute chikungunya arthritis ( joint pains of 2 weeks to 3 months following fever) over 1 year duration. Chikungunya fever was diagnosed by consistent clinical picture with IgM positivity. Clinical and disease activity assessments were done by BP. Ultrasound was done by a radiologist(FF).

Results: Twenty three patients( 18 females ) with a mean age of 44.09±9.9 years were included. he patterns of joint involvement was similar to rheumatoid arthritis, 22 patients had polyarticular disease. Rheumatoid factor was positive in 1, antiCCP was negative in all. Ultrasound revealed synovitis and tenosynovitis both on grey scale and power doppler in most patients.[Table 1] Mean DAS28 score , Indian HAQ and US7 score at presentation were 4.14±1.3, 0.81±0.5 and 17.22± 8.1 respectively. US7 score did not show a good correlation with other parameters of disease activity and HAQ. Twenty patients were treated with a short course of IM/oral steroids, methotrexate and hydroxychloroquine were given in 15 and 3 patients respectively. One year follow up was available in 20 patients. Only 2 patients continued to be on treatment. The HAQ score at follow up was 0.11±0.14. No correlation was found between DAS28, SJC, TJC, ESR, US7 score ,HAQ score or pain NRS at presentation with HAQ and pain NRS at follow up.
Table 1: Clinical features and disease activity parameters in Chikungunya arthritis

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Conclusion: Chikungunya arthritis is a self-limited polyarthritis closely resembling seronegative RA. High disease activity at presentation did not correlate with disability and pain at one year of follow up.


  PC0021: Fever in early lupus: impact on classification and association with clinical features Top


Vineeta Shobha, Asma Chougule, V S Negi, Liza Rajasekhar, Manish Rathi, Ashish Jacob Mathew, Parasar Ghosh, Ranjan Gupta, Bidyut Das, Ramnath Misra, Benzeeta Pinto, Avinash Jain, Murugavangini, Sindhura Gajula, Janany Parathasarthy, Parmeshwar Sandhu, Saumya Ranjan Tripathi, Abhishek Tripathi, Amita Aggarwal; Indian SLE Inception Cohort for Research, India

Background: EULAR/ACR has recently proposed a new set of classification criteria for SLE based on weighted items1. Based on premise that fever is an early manifestation of lupus, and that it assists in classification of early SLE, it has been included as a new component for the first time in lupus classification.

Aim: 1) To understand impact of fever on sensitivity of EULAR/ACR 2018 classification criteria in early SLE using patients recruited in INSPIRE Cohort over last 11 months. 2) To study association of fever with various clinical and immunology domains.

Methods: Indian SLE inception cohort for research (INSPIRE) is a Multi-Institutional Network Program on SLE to understand its diversity. All consecutive patients fulfilling SLICC criteria and symptom duration less than 3 years were included in this Cohort. Association of fever with 6 clinical and 3 immunology domains assessed. Sensitivity of ACR/EULAR classification evaluated in early lupus (onset of symptom <6months).

Results: In cohort of 487 patients, median age 25 + 9.8 years, F:M ratio= 10:1, fever was documented in 338 patients as one of the symptoms. Among these, 145 patients had duration of illness <6 months. Of these 135 patients fulfilled the ACR /EULAR classification criteria (93%). When fever was removed from the scoring, sensitivity was lowered to 89% and an additional 5 patients would not fulfil ACR/EULAR criteria. Fever was associated with minor manifestations of lupus rather than severe lupus such as renal or neuropsychiatric lupus.

Conclusion: Fever in constitutional domain of ACR/EULAR classification criteria adds value to diagnosis in early lupus (< 6months) and is more often associated with non- serious manifestations of lupus.
Table 1: Association of fever with American College of Rheumatology/European League Against Rheumatism

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Reference

  1. Arthritis Rheumatol 2019;1-13. [DOI 10.1002/art.40930].



  PC0022: ANA as obligatory entry criteria decrease sensitivity of ACR/EULAR criteria for lupus classification Top


Vineeta Shobha, Benzeeta Pinto, V S Negi, Liza Rajasekhar, Manish Rathi, Ashish Jacob Mathew, Parasar Ghosh, Ranjan Gupta, Bidyut Das, Ramnath Misra, Avinash Jain, Chengappa, Asma Chougule, Murugavangini, Sindhura Gajula, Janany Parathasarthy, Parmeshwar Sandhu, Saumya Ranjan Tripathi, Amita Aggarwal, Indian SLE Inception Cohort for Research, India

Background: Indian SLE Inception Cohort for Research (INSPIRE) is a Multi-institutional Network Program on SLE to understand its diversity. EULAR/ACR has proposed a new set of classification criteria for SLE based on weighted items and the use of ANA as an entry criterion.[1]

Aim: To evaluate performance EULAR/ACR 2018 classification criteria for SLE using patients recruited in INSPIRE Cohort focusing on ANA as entry criteria.

Methods: All consecutive patients fulfilling SLICC criteria for SLE and disease duration less than 3 years were included from INSPIRE Cohort. EULAR/ACR classification criteria was applied for each patient.

Results: In cohort of 487 patients enrolled so far, median age was 25 + 9.8 years, F:M ratio= 10:1, median disease duration was 9 months. The mean SLICC score was 7.4 and 12 patients were ANA negative. Therefore, ACR/EULAR criteria cannot be applied to 2.5% of SLICC approved and expert physician diagnosed cohort of SLE. Seven of them belonged to early disease subset (duration of illness<12 months). However, all 12 ANA negative patients fulfilled ACR/EULAR criteria independently, and 9 of them were scored in highly specific antibody domain. Furthermore, another 12 did not fulfil ACR/EULAR criteria as the total score was <10. Thus, 24 of 487(5%) could not be classified as SLE using ACR/EULAR criteria.

Conclusion: ACR/EULAR has poor sensitivity of 95% in our cohort. ANA as obligatory entry criteria exclude 2.5% of patients, hence this may need to be revised.


  PC0023: Comparison of results: of line immuno assay with indirect immunofluorescence for detection of antinuclear antibodies in patients with SLE at a tertiary care centre Top


Aishwarya Ramachandran, Rajeswari Sankaralingam, Kennedy Kumar Palraj, Balaji Chilukuri, Saranya Chinnadurai, Vignesh Mantharam, Ramu Ramaswamy, Shanmugesh Selvaraj; Sri Ramachandra Medical College and Research Institute, Chennai, Tamil Nadu, India

Background: Detection of antinuclear antibodies (ANAs) aids in the diagnosis of autoimmune diseases. The Indirect Immunofluorescence assay (IIF) is routinely used as a first line screening test to detect autoantibodies. Line immunoassays (LIA) is further done to detect specific autoantibodies.

Objective: The objective of this study was to compare the two techniques - ANA IIF pattern with LIA autoantibodies in Systemic lupus erythematosus (SLE) patients and to detect a definite correlation between the two methodologies.

Methods: The study included 866 serum samples obtained from patients suspected with SLE in a tertiary care centre over a period of 22 months. Analysis of ANA by IIF was done in all 866 samples.Samples positive for ANA-IIF was subjected to LIA testing.

Results: Out of 866 samples tested, 145 ( 16.7%) were positive for ANA by IIF . On further testing of the ANA-IIF positive samples for specific antibodies by LIA a total of 103 (71.3%) samples were positive for both ANA IIF and LIA and 42 ( 28.9%) showed negativity with line immunoassay.Nucleus speckled pattern was the most common(n=53;51.4%) pattern. The second most common was homogenous (n = 41; 39.9%) pattern followed by Mixed pattern (n = 5; 4.8%) and cytoplasmic pattern (n = 4; 3.8%). In association with these ANA patterns specific autoantibodies were detected by LIA. Correlation with LIA Results: with the following patterns were speckled – RNP/Sm, Sm , PCNA and SSA ( n=46; 86.6%) , homogenous - Ds DNA, histone ,nucleosomes (n = 39; 97.5%) , Mixed pattern - Ds DNA, RNP/Sm ,nucleosomes ( n= 5 ; 100%) and Cytoplasmic pattern- Ribosomal P protein ( n= 4 ; 100%).

Conclusion: Fluorescent patterns of ANA IIF can only predict the presence of antibodies and further confirmation with LIA aid to detect specific antibodies to arrive at a diagnosis of SLE.


  PC0024: Distal forearm and femoral neck as surrogate sites for measurement of spinal BMD in patients with advanced AS with 'bamboo spine' Top


U Aggarwal, A Kumar, A Agarwal, A Sharma, K Gupta; Department of Rheumatology, Fortis Flt Lt Rajan Dhall Hospital, New Delhi, India

Background: Vertebral fracture rates are very high in advanced AS. Spinal BMD measurement by DEXA in 'bamboo spine' usually gives invalid Results. Quantitative CT is not acceptable because of high radiation exposure. Femoral neck has been suggested as a possible surrogate site for measuring spinal BMD in AS. Spinal BMD measurement in AS remains an area of unmet need.

Objective: To study distal forearm and femoral neck as surrogate sites for measurement of spinal BMD using DEXA technique in patients with advanced AS with bamboo spine.

Methods: 25 patients with advanced AS who had bamboo spine (high mSASSS scores) and disease duration more than 10 years were subjected to BMD measurement by DEXA at femoral neck and distal forearm. Patients who were on steroids and had history of disorders of calcium metabolism were excluded.

Results: All 25 patients were males with a median age of 49 years and median disease duration of 13 years. Vertebral morphometric fracture (s) were present in 10 (40%). BMD at femoral neck revealed osteoporosis in 4 patients (16%) and osteopenia in 9 (36%). BMD at forearm showed osteoporosis in 10 patients (40%) and osteopenia in 9 (36%). Two patients had osteoporosis at both sites. Thus, DEXA scan at femoral neck and distal forearm together could detect osteoporosis only in 48% of patients with advanced AS.

Conclusion: DEXA scan at distal forearm was more sensitive than femoral neck in detecting osteoporosis (40% vs 16%) in patients with advanced AS. This also implies that osteoporosis in AS is not caused by immobility of spine alone. There is a systemic component to its pathogenesis.


  PC0025: Long-term outcome of patients with seropositive palindromic rheumatism Top


K Gupta, A Kumar, U Aggarwal, A Sharma, A Agarwal; Department of Rheumatology, Fortis Flt. Lt. Rajan Dhall Hospital, New Delhi, India

Background: Palindromic rheumatism is a well-known syndrome of painful episodes of non-deforming mono/oligoarthritis involving large joints which resolve spontaneously within 48-72 hours. ACPA positivity in these patients has been used to predict its evolution into RA. Data from India on this entity are scant.

Objective: To study long-term outcome of seropositive patients with palindromic rheumatism

Methods: All seropositive (RF and/or ACPA +ve) patients diagnosed with palindromic rheumatism between April 2009 and March 2015 in our rheumatology clinic were recruited and their outcome was documented during the period July-August 2019. Telephonic interviews were carried out when necessary.

Results: Thirty seven patients were identified as per the inclusion criteria. Five were lost to follow up. Clinical details with outcome data were available on 32 patients (F:M = 24:8, median age =46 years). Of these 15 were positive for both RF and ACPA, 12 only for RF and 5 only for ACPA. RA (ACR-EULAR criteria) developed in 25/32 patients (78.1%). Of these, 21 developed RA within 5 years and 4 in 5-10 yrs. The median time to development of RA was 1 year. The development of RA was preceded by a period of 3-6 months of substantially increased frequency of episodes of arthritis. Correlation of serology with progression yielded interesting Results: and will be discussed. Three patients (9.4%) continued to have episodes of palindromic rheumatism. Four patients (12.5%) attained long-term remission.

Conclusion: Most patients with seropositive palindromic rheumatism progressed to RA with 50% doing so in the first year of follow up. Patients should be counselled and closely followed up for development of RA and timely start of DMARDs. At the same time, it is important to avoid starting DMARD therapy prematurely because a subset takes 5-10 years to develop RA.


  PC0026: Clinical profile of granulomatosis with polyangitis in a southern tertiary care centre Top


S Nagmafarheen, P S Arulrajamurugan; Madurai Medical College, Madurai Medical College, India

Background: Granulomatosis with polyangiitis (GPA) formerly known as Wegener's Granulomatosis is an immunologically mediated small vessel vasculitis that is pathologically characterized by inflammatory reaction pattern like necrosis, granulomatous inflammation and vasculitis that occurs in upper and lowerrespiratory tracts and kidneys.

Objective: To study the clinical and laboratory profile of patients with Granulomatous Polyangitis

Methods: A retrospective analysis of the records of patients fulfilling the diagnostic Criteria for Granulomatosis with polyangiitis who attended a Rheumatology clinic over a period of 7 years from 2012 was done. Clinical, hematological, biochemical, immunological and histological profiles wereanalyzed.

Results: 8 patients, 4 males and 4 females with the age range of 22-57 years with mean age of 37 years were included. The duration of symptoms is between 1.5 months to 24 months. Purpura and scleritis were present in 6 patients each followed by arthritis and paranasal sinusitis in 5 each .3 had otitis media. Epistaxis, cough and hemoptysis in 2 each. Proximal muscle weakness was present in 2 patient and 1 had peripheral neuropathy. 1 had fever. 6 were positive for C ANCA. Hematuria, proteinuria, leukocytosis, elevated ESR, CRP and renal parameters were seen in one patient each. 4 had necrotizing granulomas in lung biopsies. Chest X ray revealed cavitation and nodules in 1. CT chest showing nodules in 3 followed by ground glass appearance, cavity, air space consolidation in 2 each.

Conclusion: There is an equal gender preponderance. Eye and skin involvement being the most common manifestations compared to other studies followed by musculoskeletal, upper airway and pulmonary features. C ANCA positivity seen in majority of patients.
Figure 1

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  PC0027: Predicting factors affecting the adherence and non-adherence of methotrexate in rheumatoid arthritis Top


Ravita Thakran, Lubna Khurshid, Anand N Malaviya; Department of Rheumatology, ISIC Superspeciality Hospital, New Delhi, India

Background: Methotrexate (MTX) is the first-line disease-modifying anti-rheumatic drug for the patients with RA . It is important to look for the factors that may positively or adversely influence the adherence so as to modify them, thereby increasing adherence to the anchor drug.

Objectives: 1) To determine the adherence rate to MTX therapy in patients with RA. 2) To identify factors that promote either adherence or non-adherence

Methods: A cross-sectional observational study of 200 patients on MTX for at least 6 months was conducted. The questionnaire comprising demographic details , disease duration, previous treatment , duration of MTX, current dose and number of doses of MTX missed were recorded.Disease activity was measured by CDAI at each visit. Non-adherence was defined as an omission of two or more prescribed doses of MTX in previous 4 weeks. Patients were asked for the factors that motivated their adherence as well as non-adherence to MTX.

Results: Non-adherence was found among 25 % of patients. Those lacking awareness regarding importance and long term need of drug , co-morbidities; lack of affordability, availability at the local pharmacy and family support, social myths were significant factors for non-adherence. Age, gender, level of education, duration of RA, current doses of MTX was not significantly different for adherent and non-adherent patients. Patients who were non-adherent had increased disease activity (CDAI). Good counseling and fear of disability were the strong predictor of adherence among the adherent group.

Conclusion: Various personal and social issues lead to non-adherence, but good patient's counselling can make a difference. As MTX is an anchor drug for the treatment of RA, adherence to it is important for good disease control and a better outcome. Thus, the rheumatologist and the rheumatology nurses can help improve drug adherence by knowing the factor(s) that affect noncompliance


  PC0028: Short-term outcomes in reactive arthritis Top


Manoj Kumar, Prasanta Padhan, Sakir Ahmed; Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India

Introduction: Reactive arthritis(ReA) is a lower limb and large predominant spondyloarthritis that occurs after genitourinary or gastrointestinal infections. Some remit spontaneously while some can even proceed to ankylosing spondylitis. The literature on outcomes of ReA is limited.

Objectives: To find out the short term out comes of ReA.

Methodology: All patients meeting Braun criteria for ReA and having a follow-up of at least 6 months were included. Their current status was determined by telephonic interview and physical review of certain patients as required. Patients with inadequate follow-up data were excluded. Disease activity was measured by Bath Ankylosing Spondylitis Disease Activity Index(BASDAI) while quality of life was ascertained by the Health Assessment Questionnaire-Disability Index(HAQDI).”

Results: 43 patients [8(18.6%) females; median age 26(IQR: 19-34.5years)] were included. Median follow-up duration was 26 (IQR:15-62) months. 27(63%) had resolved, 12(27.9%) had a relapsing-remitting course, 4(9.3%) had persistent arthritis.10(23.8%) were in drug-free remission for more than 6 months. One patient had switched to homeopathy. 33 had received sulfasalazine, 2 methotrexate, and 13 had received at least one intra-articular steroid injection.10 refractory cases received anti-TNF agents of which 3 were continuing, 3 had switched to sulfasalazine and 4 were completely off drugs. 15 were currently on non-steroidal anti-inflammatory drugs, 3 on oral steroids(plus other drugs). In the 33 patients still on drugs, median BASDAI was 0.4(IQR:0-1.6) and median HAQDI was 0(IQR:0-0.15).

Conclusion: In our cohort, around one-fifth attained drug free remission. For the patients continuing drugs, there is good control of disease activity with good quality of life.


  PC0029: Macrophage activation syndrome in SLE and Systemic onset JIA: Similar or dissimilar Top


R Naveen, Hafis Muhammed, Avinash Jain, Latika Gupta, Durga P Misra, Able Lawrence, Vikas Agarwal, Ramnath Misra, Amita Aggarwal; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Macrophage activation syndrome (MAS) is a serious complication in rheumatic disease. Fever and hyperferritinemia are common in systemic onset JIA and cytopenias are common in SLE thus recognizing MAS in them is a challenge. Hence, we compared clinical, laboratory parameters, various classification criteria for MAS, and its outcome in SLE and sJIA.

Methods: Clinical and laboratory data were extracted from clinician diagnosed cases of MAS with SLE/sJIA who were admitted between 2004-2018 at a tertiary care hospital. Percentage of patients satisfying Ravelli, International consensus, HLH 2004 and criteria proposed by parodi et al were calculated.

Results: Among 33 patients (18 females) with MAS 19 had SLE and 14 had sJIA. MAS was more likely to be the presenting manifestation of disease in SLE as compared to sJIA (p<0.05). There were no differences in the clinical features among these two diseases. EBV and CMV were identified in 2 patients each as the trigger for MAS.Patients with SLE had lower baseline TLC and platelet whereas patients with sJIA-MAS had significantly higher median CRP (p = 0.002), fall in TLC (p=0.012) and delta ESR/CRP ratio (p=0.02) and lower fibrinogen level (p=0.006). Neutrophil to lymphocyte ratio, Ferritin/CRP ratio and number of patients with Ferritin/ESR >80 were similar. Bone Marrow hemophagocytosis was seen in only in 30% of patients.Only 6/33 fulfilled HLH criteria but criteria meant for sJIA or SLE performed well for both diseases and majority of patients could be diagnosed using them. Treatment included steroids(100%), cyclosporine(30%), Tacrolimus(21%), cyclophosphamide(21%), etoposide(3%) and thalidomide(12%). Outcome was similar in both groups.

Conclusion: MAS is more likely to be presenting manifestation in SLE compared to sJIA. Though lab parameters are significantly different in MAS associated with SLE &sJIA, criteria meant for MAS in sJIA or SLE MAS performed equally well in both diseases.


  PC0030: Comparison between diarrhoea associated reactive arthritis and urinary tract infection associated reactive arthritis Top


Sidharth Arora, Prasanta Padhan, Sakir Ahmed; Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India

Background: Reactive arthritis(ReA) is a spondyloarthritis that is triggered by gastrointestinal or Urinary tract infection (UTI) infection. It has not been explored if there is a difference between post diarrheal ReA and post Urinary Tract Infection ReA.

Objective: To explore the difference between post diarrheal ReA and post UTI ReA.

Methods: All patients meeting the Braun criteria for ReA were included. They were divided into two groups (post diarrheal ReA and post UTI ReA) based on patient interview. Clinical features, laboratory and necessary laboratory analyses were collected in a preformed proforma. Appropriate statistic tests were used to compare the data. Data is mentioned as median (IQR).

Results: 67 patients [12 (17.9%)females; median age 25 (IQR:19-38) years] with post diarrheal ReA and 22 patients[16 (72.7%); median age 28 (IQR20- 32) years] with post UTI ReA were included. Proportion of females was statistically more in post UTI ReA (p<0.001). Duration of disease between the two were comparable [post diarrheal ReA 3 (IQR1-38) years; post UTI ReA 3 (1-24) years; p=0.76]. There was no difference in educational status. Clinical features were comparable between the two groups. [Table 1]
Table 1

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Conclusion: Post UTI ReA was more common in females. However, in other parameters there was no difference between post diarrheal and post UTI ReA.


  PC0031: Clinical outcome after lowering the dose of TNF alpha blockers among spondyloarthritis study Top


Kattel Vivek, Agrawal Yamuna1, Tilwee Virend, Gupta Neetu2, Malviya Sourabh; Medanta Superspeciality Hospital,3MGM College, Indore, Madhya Pradesh, India,1BP Koirala Institute of Health Sciences Dharan, Nepal

Introduction: TNF alpha blockers are the most effective drugs against Spondyloarthritis (SpA). Recommended dose schedule by ACR and EULAR is associated with better clinical outcome however with higher cost, more risk of latent infection activation and increased likelihood of antibodies development against biological DMARDs (bDMARDs).

Objective: To study the effectiveness of low dose bDMARDs by increasing the gaps between two doses in Indian subcontinent.

Methods: It was a prospective study carried among 50 SpA patients for 52 weeks. SpA was diagnosed according to Assessment in SpondyloArthritis international Society (ASAS) criteria. Initially ACR recommended dose of Adalimumab or Eternacept was given for 12 weeks. Among patients with clinical remission 25% dose of the bDMARDs was reduced after every 12 weeks by increasing gaps between two injections. The dose reduction by 25% was further continued every 12 weeks among patients who had clinical remission.

Results: The median age was 36 years. Clinical presentation as low back pain, peripheral arthritis, enthesitis, dactylitis and uveitis was noted in 92%, 68%, 62%, 18% and 12% respectively. The baseline mean BASDAI, mean sobers and CRP were 7.2, 4.1cm and 29mg/L. By the end of 52 weeks 49 patients were on bDMARDS with mean BASDAI, mean sobers and CRP were 3.6, 6.9cm and 7mg/L. The effectiveness of bDMARDs was maintained among 100%, 98%, 96% and 90% at 24, 36, 48 and 52 weeks respectively despite of lowering the dose by 25% every 3 monthly. Flare ups were observed among 1, 3, 4, 6 and 9 patients at 12, 24, 36, 48 and 52 weeks interval of the treatment respectively.

Conclusion: Increasing gaps between two doses of bDMARDs under clinical supervision among SpA can be a cost effective option.


  PC0032: Experience with the follow-up of patients at a rheumatology-clinic during the early years of practice Top


Anuj Shukla, Priyanka Gaur; Niruj Rheumatology Clinic, Ahmedabad, Gujarat, India

Background: Patient follow-up is important in autoimmune diseases due to relapsing-remitting nature. Follow-up depends upon many factors such as disease severity, duration, patient-doctor communication, patient's financial health etc. Here, we reviewed our initial database to understand the pattern of follow-up and the reasons for the same.

Objective: To share the experience with follow-up of patients seen in a rheumatology clinic.

Methods: Basic information stored in the database was used to contact the patients on phone or were requested personal interview in the clinic. A questionnaire was used to gather information related to the diagnosis and follow-up of the patients. Regular follow up was defined as >6 months.

Results: The total number of patients entered in the database during July'16 to Aug'19 were 4682. Out of which, we contacted 53%(2471) patients while 47%(2211) patients were not contacted due to following reasons; 25%(1200)–degenerative or non-autoimmune diseases (with <3 visits), 9%(439)-missing or wrong contact details, 9%(432)-unanswered calls and 7%(330) having a single clinic visit.Out of 2471 contacted, 83%(2059) were under regular follow-up while 15%(393) stopped follow-up. Among regular follow-up group (n=2059), diagnosis was available for 56%(1171) patients. Out of which, 757-rheumatoid arthritis, 92-spondyloarthritis, 68-systemic lupus erythematosus, 33-idiopathic-inflammatory-myositis, 33-mixed connective tissue disease, 13-primary Sjogren syndrome, 15-primary systemic vasculitis, 11-systemic sclerosis and 32-juvenile autoimmune diseases.Among the lost to follow-up group (n=393): 34%(136) informed got cured (>1year), 16%(63) had no reason, 14%(57) continued medicines but stopped follow-up, 12%(50) were seeing other allopathic doctors, 9%(39) opt for alternative medicines, 8%(34) stopped all medicines although symptomatic, 3%(13) were out of country or financial issues.

Conclusion: This is our initial effort to understand the follow-up of rheumatology related patients. We look forward to further study the factors influencing the follow up of patients. This can help in improved follow-up and better outcome of these patients.


  PC0033: Clinical features, management and follow-up of myositis patients seen at a rheumatology clinic Top


Anuj Shukla, Priyanka Gaur; Niruj Rheumatology Clinic, Ahmedabad, Gujarat, India

Background: Myositis is a rare manifestation of various systemic autoimmune diseases. Its classification and association with myositis specific auto-antibodies (MSA) is evolving in the recent years. Here we share the experience with these patients in our early years of clinical practice.

Objective: To share the experience of patients presenting with the clinical features of myositis.

Methods: A data was collated using clinical notes and personal interviews of the patients seen from July2016 to June2019. Diagnosis of various Idiopathic inflammatory myositis was made based on EULAR-ACR Classification criteria 2017. MSA were tested using Immunodot Myositis12 SAE-IgG 12antigen kit or EUROLINE Autoimmune-Inflammatory-Myopathies 16Ag(IgG) kit in a private diagnostic laboratory.

Results: 41-patients presented with the clinical features of myositis. Mean age at disease onset was 37+14years and the disease duration was 9+4months. Sub-groups of the patients and their MSA profile are shown in [Figure 1]. Among clinical features: 90%(n=37) had muscle weakness, 68%(n=28) skin rash, 56%(n=23) arthritis, 29%(n=12) fever, 14%(n=6) ILD and 19%(n=8) had dysphagia and 21%(n=9) patients had Raynaud phenomenon.Mean follow-up duration was 16+13months. DMARDs used were methotrexate 68%(n=28), azathioprine 21%(n=9) and mycophenolate mofetil 2%(n=1). Rituximab, intravenous immunoglobulin and methylprednisolone pulse were used for induction in 12%(n=5), 4%(n=2) and 12%(n=5) patients respectively.Out of 41 patients, 4(9%) expired, 23(56%) are in remission, 2(4%) has active grumbling disease and 6(14%) patients are on induction therapy. 2 patients had pregnancy during follow up with successful outcome. Complications seen were infections 17%(n=7), calcinosis 9%(n=4), cataract 4%(n=2), hyperpigmentation 4%(n=2) and depression 2%(n=1).
Figure 1: Sub-groups of the myositis patients (N = 41) and myositis specific antibodies

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Conclusion: Thus, we collated a data of patients presented with myositis. The sample size is small for firm Conclusion. But data being from a single center, helped us to collect data uniformly and finely. In future, we look forward to continue collecting the data that might help clearer Conclusion.


  PC0034: Prevalent vertebral fractures incur high risk of future fractures in inflammatory myositis Top


Sujata Ganguly, Able Lawrence, Ramnath Misra, Latika Gupta; Department of Clinical Immunology, SGPGI, Lucknow, Uttar Pradesh, India

Objective: To assess accrual of new vertebral fractures (VF) in patients with inflammatory myositis over 3 years.

Methods: 100 patients previously enrolled for a cross-sectional study on prevalence of asymptomatic VF were requested to review with repeat dorso-lumbar X-ray's and Bone mineral Density evaluation 3 years after the initial assessment and scored using Genant's semi-quantitative technique by two independent observers. Involvement of new vertebra, worsening of prior fracture or new site of fracture were recorded as “fracture event”.Clinical and disease variables were recorded. Statistical analysis was done using SPSS Version 23.

Results: Radiographs from 31 patients (8:23: M:F) of median age 38 years were reviewed. Eighteen of the 31 (58.06%) had VF at baseline.At 91.62 patient years of follow-up, fourteen of 18 (77.78%) patients with previous VF had new fractures, while one without previous fractures had new fractures. Total number of fractures (30 to 51) increased. Most had >1 fracture (14 of 19,73.6 %) although maximum (35 of 51, 68.62%) were grade 1 VF. The increase in lumbar fractures was greater than thoracic. (p=0.032). Previous VF conferred 9 times higher risk of developing a new VF (RR- 9.33(95%CI 1.40-61.95) p-0.02). Patients with old VF accrue fractures at a rate of 22.9 per 100 patient years of follow up. Of the 18 patients with DEXA scans, 3 had osteoporosis and 5 were osteopenic and 2 were below the expected range for age. Age and T scores at L4 level (r=-0.591, p-0.026) and lower third of radius (r- -0.653, p- 0.016) correlated with fracture number. Age, BMI and gender did not differ between fracture progressors and non-progressors nor did change in disease activity and Myositis Damage Index scores. (p-ns)

Conclusion: Patients with inflammatory myositis with prior asymptomatic VF have a high risk of subsequent VF irrespective of disease activity and glucocorticoids.


  PC0035: Angiographic outcome in takayasu arteritis: A bidirectional study from a tertiary care hospital Top


K Spoorthy, D Phanikumar, N Ramakrisha, R Liza; Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Objective: Serial imaging studies recommended in monitoring Takayasu arteritis (TA) patients are not well documented.

Methods: In this bi-directional study, patients fulfilling the ACR 1990 criteria for TA, seen between January 2012 to May 2019 , with a follow up of at least 12 months and comparable CTA/MRA imaging at baseline and follow up were included. Demographic data, clinical features, angiographic and treatment details were noted. Angiographic details of narrowing, stenosis , wall thickening, wall enhancement , aneurysm ,dilatation were noted in 18 arteries in each patient (bilateral subclavian, common carotid, vertebral, right brachiocephalic, ascending aorta , arch ,descending thoracic and abdominal aorta, celiac, superior and inferior mesenteric artery, bilateral renal and common iliac arteries).

Results: Serial CTA / MRA imaging was available in 38 of 129 patients seen during this time. Median (IQR) duration from symptom onset to diagnosis was 18 (4.2-48) months and between two angiographic assessments was 14.5(13-24.2) months. Mean (SD) TADS available for 21 patients was 5(5.2). Establishing disease inactivity in clinically inactive patients was the most common indication for repeat angiogram (30/38).All received steroids. Methotrexate was initiated in 37 patients . During follow-up, no change in treatment noted in 28 patients. Two patients had radiographic progression in spite of clinically inactive disease. Type V was the commonest angiographic subtype (20/38) with abdominal aorta (27/38) and left subclavian artery (24/38) being most commonly involved. A change in type of angiogram was noted in 6 patients and 2 had improvement from type V to type IIa and IIb respectively.Data on 684 arteries assessment is mentioned below .

Conclusion: Wall thickening was the commonest abnormality at baseline and increased during follow-up . There was no significant damage accrual during therapy. Clinical follow-up is a reasonable tool for follow-up in patients with TA on therapy.
Table 1

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  PC0036: Evaluation of wrist joint and hand joint by ultrasonography in patients of rheumatoid arthritis and its clinical comparison Top


S Nelson, N Repaka, A Mittal; NSCB Medical College, Jabalpur, Madhya Pradesh, India

Background: Early diagnosis and treatment of rheumatoid synovitis can turn down the progression of rheumatoid arthritis (RA). However, in the early stages, patients may only have non-specific musculoskeletal symptoms. Ultrasound (US) is increasingly being used to evaluate joint involvement in RA.

Objective: The study aims to evaluate wrist and hand joint involvement in RA by US and its comparison with clinical examination, disease activity and illness duration

Methods: Patients with RA were subjected to detailed clinical examination and laboratory investigations. Ultrasonography of bilateral wrist and hand joint of both hands as well as at the level of carpal tunnel to determine median nerve diameter was performed. The Disease activity score (DAS28) was also calculated for all patient.

Results: Total 30 patients (25 females and 5 males) with 60 wrist and hand joints were evaluated. Sensitivity of ultrasonography for detecting periarticular changes in left wrist joint was calculated to be 83.33% and that of clinical examination as 58.8% while in case of right wrist joint sensitivity for ultrasonography was 81.81% and for clinical examination was 64.3%.The prevalence of carpal tunnel syndrome was found to be 25% amongst 60 wrists joint examined by USG with 95% CI: 14.8-37.8.On ultrasonography most common pathological abnormality on left wrist joint was erosion of carpal bones and on right side was synovitis of carpal bones. Most common pathological changes were synovitis in bilateral hand joint.The cases with high Das 28 score had higher significant pathological periarticular involvement in bilateral wrist and hand joints in comparison to one with low DAS 28 score.

Conclusion: Ultrasonography is more sensitive than clinical examination for detecting periarticular changes in bilateral wrist and hand joint. There was significant association between ultrasonography changes in bilateral wrist and hand joint and disease activity as well as duration of illness.
Figure 1: USG showing erosion of metacarpophalangeal joint

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  PC0038: Reinforcing clinical skills into research: A survey of the musculoskeletal abnormalities in school going boys using PGALS in urban population Top


B Nithya, P Umapathy, J Mahesh; Department of Rheumatology, Division of Pediatric Rheumatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Background: pGALS (Pediatric Gait, Arms, Legs and Spine) – a simple quick musculoskeletal system assessment. Musculoskeletal pain affects between 10% and 20% of pediatric populations. The use of pGALS is aimed as a basic clinical skill to be used to identify various musculoskeletal pathologies.

Objective: To identify the musculoskeletal abnormalities using (pGALS) screening tool and to identify hypermobility using beighton's scoring in apparently healthy school going boys

Methods: A prospective cross-sectional study was conducted in school going boys aged between 7 to 17 years of age in Chennai enrolling 1547 children from 3 schools. History, general examination, screening questions were recorded in the proforma. Pgals was demonstrated in groups and was asked to perform and the Results were recorded in the screening Proforma and Results were analyzed.

Results: The pGALS examination was completed in 1547 boys, detecting Hypermobility (11.1%) Growing pain (4.6%), mechanical pains (12%). Overweight and obese children had higher incidence of pain or stiffness of joints (P value: 0.012). Maximum prevalence of hypermobility was in 7-9-year age group. We were able to identify one case of inflammatory (Enthesitis related arthritis) and one case of Non inflammatory pathology(Slipped Capitofemoral epiphysis)

Conclusion: pGALS is an Excellent screening tool for detection of musculoskeletal abnormalities on apparently healthy children.


  PC0039: Symptom avoidance behavior of patients with very early rheumatoid arthritis Top


Akshay Saxena, Arup Mahapatra, Prasanta Padhan, Sakir Ahmed; Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India

Introduction: Patients with rheumatoid arthritis(RA) often present late especially in our country. All guidelines recommend initiation of disease modifying drugs as early as possible. Recognition of symptom avoidance behavior can help plan strategies to increase earlier presentation.

Objective: To identify the common symptom avoidance behavior in patients with very early RA

Methodology: All patients meeting the ACR/EULAR2010 criteria for RA and presenting to the hospital within 12 weeks of onset of symptoms were queried with a questionnaire about their symptom avoidance behavior. These were compared against age, gender, occupation and educational status using standard statistical tests

Results: 250 patients with mean(SD) age[median age : 49] with 88.4% females were included. The median time from onset of symptoms to first visit to a rheumatologist was 46 days. The first allopathic doctor visited was a non-rheumatologist in (147) 59%.The prevalence of common symptoms avoidance behavior is summarized in [Table 1]. Females were more likely to use self-administered splints(p=0.03), and to presented only when their daily activities were disrupted(p=0.005). Married persons were more likely to take self-prescribe drugs(p=0.04) and present only after their daily activities were disrupted (p=0.01). Employed persons were more likely to consider themselves too young to have arthritis(p=0.04) and to present only after disruption of daily activities(p=0.01). Paradoxically persons with higher education were more likely to consider themselves too young to have arthritis(p=<0.001) and to try to treat themselves by limiting their mobility or with the help of exercises(p=0.001).Also more educated persons were more likely to search for self-treatment on the internet(p=<0.001) and less likely to try to treat themselves by restricting their diet(p=0.01).
Table 1: Frequencies of common avoidance behaviours

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Conclusion: Even in this cohort of early RA, most patients did not visit rheumatologist first. Being female or married or employed was associated with presentation only after disruption of activities of daily living.


  PC0040: Mixed connective tissue disorder: Clinical features and short-term follow-up of 27 patients Top


Shreyansh Deosale, Sakir Ahmed, S S Panda, Prasanta Padhan; Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India

Background: Mixed connective tissue disorder(MCTD) is a well-defined entity with varied clinical manifestations. Though understanding of this disease has improved in the last decades, there is still limited data on MCTD.

Objective: To enumerate the clinical features at presentation and short term follow-up of a cohort of MCTD patients.

Methods: All patients with minimum 12 months follow-up at a tertiary care centre, and meeting Alarcon-Segovia classification criteria for MCTD were included. Uncontrolled diabetes and hypertension were excluded. Clinical features at diagnosis were noted, and current status was found out from patient interview.

Results: Of 27 MCTD patients, median age was 40(IQR:20.5) years with 23(88.46%) being female. Median time to presentation was two months(IQR:3.5). 6 patients were diagnosed at 6 months or more after onset of symptoms. Median follow up duration was 12months(IQR:1.25).Fever was the presenting symptom in 21 patients. All patients had arthralgia but only 8 had arthritis (most common affecting knee). Raynaud phenomenon(RP) was absent in 1 patient. Concomitant depression was diagnosed in 10 patients. 10 patients had swollen hands. 14 patients had symptomatic interstitial lung disease(ILD). Pulmonary arterial hypertension was present in 11 patients. One patient had myositis. All patients had anemia on presentation but no other cytopenia. Among autoantibodies, besides U1RNP positivity, five patients had Ro-52 positivity. All patients received steroids and hydroxychloroquine; 20 received mycophenolate.During the follow-up period, 3 patients had severe infections requiring hospitalization. 6 patients had relapse of disease that was managed with dose adjustments. Arthritis, and Raynaud are in remission in all patients. All patients with ILD had improvement in functional parameters.

Conclusion: Arthralgia, fever and RP are the major presenting features in our cohort. All patients were in clinical remission and seem to have good response to therapy on short term follow-up.
Table 1: Comparison with similar studies

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  PC0041: Subacute psychiatric presentation-treat them or leave them? Top


AdamyaGupta, Archana Verma, V P Pandey; MGM Medical College and My Hospital, Indore, Madhya Pradesh, India

Introduction: Subacute presentation of psychiatric complaints like behavior abnormalities, cognitive dysfunction along with neurological complains like seizure,headache,fever with normal csf sugar with variable combinations of proteins and cells, with/without mri brain findings, should be highly suspected for treatable like herpes simplex encephalitis and autoimmune encephalitis.

Materials and Methods: 6 months study of 30 cases visiting my hospital with encephalitis along with psychiatric manifestations. exclusion of all bacterial encephalitis & tubercular encephalitis.

Observation: In our case series of 30 cases we found:73% behavior abnormalities,73% fever,46% seizures, 36% headache. On imaging 60% normal brain imaging, 40% meningeal enhancement.

0n csf examination, normal glucose 93%, normal protein 53%, normal cells 56%,those with increased cells(43%) had 100% lymphocytic predominance. EEG s/o diffuse cerebral dysfunction 9%.6% csf anti NMDA antibody positive, 12% csf anti VGKC antibody positive, out of which 3% positive for autoimmune antibodies in serum. Only 6-9% positive for csf HSVPCR.

Conclusion: Subacute psychiatric manifestations treated by acyclovir and steroids for HSV encephalitis and autoimmune encephalitis showed good response and fair recovery, even with normal csf and mri brain with negative HSVPCR and negative autoimmune encephalitis.


  PC0042: Comparison between intraarticular and intramuscular depot methyl prednisolone injection in functional improvement of hand in patients suffering from early rheumatoid arthritis Top


Swarnali Mandal, Biswadip Ghosh1, R N Halder2 Deptartment of Physical Medicine and Rehabilitation, Murshidabad Medical College, Berhampore, Deptartments of1Rheumatology and2Physical Medicine and Rehabilitation, IPGME and R, Kolkata, West Bengal, India

Aim: Primary aim in management of Rheumatoid Arthritis is to decrease inflammation and thereby, to prevent joint damage.

Objective: The objective of this study was to compare the improvement in hand function and disease activity among patients receiving intra-articular with intramuscular depot methyl prednisolone injections.

Design: A prospective cohort of 136 patients with RA were randomly allocated in two parallel groups intra-articular (IA) and intra-muscular (IM) who were given depot methyl prednisolone injection either IA or IM after assessment of wrist Range of motion (ROM), grip strength, Clinical disease activity index (CDAI) & Keitel functional test (KFT) scores on 1st visit and reassessed at 1 month and 3 months post injection.

Results: Wrist flexion and extension, power grip and all pinch grips of both hands have shown statistically significant improvement over time between any 2 visits, except right wrist extension, right and left lateral pinch grip did not have statistically significant improvement between 2nd and 3rd visits. CDAI scores reduced & KFT scores improved in persistent manner more in IM group over time between any 2 visits which is statistically significant. It is also seen that with decrease in disease activity there is improvement of functional ability in the subjects.

Conclusion: Intra-articular injections have sustained effects throughout the study period in ROM improvement and intramuscular injections have short-lived effects but are more effective in improving outcomes of functional status and disease activity. So, we think, IA corticosteroids should be used more frequently in early rheumatoid arthritis, to prevent joint damage.


  PC0043: Anti beta-2 glycoprotein antibodies and not lupus anticoagulant associate with digital gangrene in systemic lupus erythematous Top


M Praveen Kumar, Liza Rajasekhar, D Phanikumar; Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background: Previous studies [1,2] showed inconsistent association of antiphospholipid antibodies with digital gangrene (DG) in lupus patients.

Objective: Using all three tests for anti-phospholipid antibodies we aimed to assess their association with DG in lupus patients.

Methods: Lupus patients identified from lupus registry/ outpatients or inpatients, with an episode of DG after January 2008 and seen between to August 2018 to April 2019 were enrolled as cases. Age and sex matched lupus patients without digital gangrene were included as controls. Both were followed prospectively for 6 months. Clinical, laboratory variables at episode of DG and disease activity (SLEDAI) and damage index(SLICC-DI) at enrollment and last follow-up were recorded. Serum titers of anticardiolipin antibodies(aCL ab),beta-2 glycoprotein 1 antibodies (B2GPI ab) and lupus coagulant (LA) were done for all cases and controls.Statistical analysis done using chi-square test for categorical variable, Mann-Whitney test for continuous variable.

Results: Forty cases and 40 controls were enrolled and included in analysis. Median (IQR) disease duration before onset of DG was 8 months (1-37) months with median(IQR) duration till last follow-up 32 (24-64) months. Four cases had multiple episodes of DG. Cutaneous vasculitis Raynaud's phenomenon and mononeuritis multiplex (MNM) occurred more frequently in cases. Clinical and laboratory parameters are mentioned. Baseline aptt was higher in cases( p=0.005). The titres of anticardiolipin antibodies (aCL ab) and beta 2 glycoprotein (B2 GPI) were significantly higher among cases.

Conclusion: Digital gangrene occurs very early in course of lupus. It is clinically associated with Raynaud's phenomenon, cutaneous vasculitis and MNM.B2GPI ab and aCL antibodies but not LA associated with DG in lupus.

References

  1. Jeffery RC, Narshi CB, Isenberg DA. Prevalence, serological features, response to treatment and outcome of critical peripheral ischaemia in a cohort of lupus patients. Rheumatology (Oxford) 2008;47:1379-83.
  2. Rajasekhar L. Prevalence,serologicalfeatures,and outcome of critical peripheral ischemia in chort of lupus patients. Rheumatology 48:451-7.



  PC0044: Treatment response to conventional DMARDs in psoriatic arthritis Top


Taral Parikh, Sapan Pandya, Rakesh Solanki; Vedanta Institute of Rheumatic diseases, Ahmadabad, Gujarat, India

Background: soriatic 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: and so are the current guidelines.

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

Methods: 80 patients, from 2012-2019, 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 outcome assessed was improvement in (psoriatic arthritis skin index) PASI and dactylitis.

Results: (n=80) Male 66/ female 24. Mean age 42.1(±10.90), weight 68.9(±13.9), duration of PsA 3.83(±4.14). 28% -family history of psoriasis/PsA.Pattern of PsA:Polyarthritis 22 (30%), oligoarthritis 11(15%), axial 5 (7%), polyarticular +axial 13 (18%), oligoarticular +axial 17 (23.4%), axial + DIP 4 (5.4%)Baseline:PASI 3.39 (±5.86), SJC 4.73(±4.49), TJC 4.49(±7.65), dactylitis 18, physician global assessment (Ph GA) 3.28(±.0.79), patient global assessment of PsA (Pt GA) 3.49(±0.78). Mean duration of follow up 2.8(±1.35) months.Treatment response:42(67%) patients were started on NSAIDs, 4(6%) on prednisolone, 47(81.5%) on methotrexate, 17(32.5%) on sulfasalazine, 13(26.5%) on combination (MTx + Ssz) and 3(4%) on leflunomide.At first follow up visit 49 (61.25%) were PSARC responders.PASI reduced from 3.39(±5.86) to 1.58 (±4.20). 21 (26.2%) PASI 90%, 2 (2.5%) achieved PASI 75 and 3 (3.75%) PASI 50, dactylitis improved in 14 (17.5%) patients.

Conclusion:

After 3 months 61.25% patients were PSARC responders, with conventional DMARDs. There was improvement in other domains of PsA as well.


subtilis Scientific Name Search  1972 and  Bacillus coagulans Scientific Name Search 1969) as an adjunctive therapy in the treatment of rheumatoid arthritis: A prospective study">  PC0045: To evaluate the efficacy of probiotic- BIOTHERAPI® (combination of bacillus subtilis 1972 and bacillus coagulans 1969) as an adjunctive therapy in the treatment of rheumatoid arthritis: A prospective study Top


Arindam Nandy Roy, Yarram Ashok Kumar, Syeda Sana Fatima; Yashoda Hospitals, Secunderabad, Telangana, India

Background: The concept that microorganisms play a role in etiology, pathogenesis, and treatment of RA has been advanced over 100 years. There is good evidence that Probiotics reduce gut permeability and modulate immunity. Therefore, it might be speculated that Probiotics may down regulate the abnormal inflammatory response and alleviate the symptoms of RA.

Objective: To evaluate the effects of BIOTHERAPl® (Combination of Bacillus Subtilis 1972 and Bacillus Coagulans 1969) on disease activity and functional ability of RA patients when used in combination with pharmacological anti-rheumatic medications.

Methods: This was a prospective, randomized, single centre, two arm, open label trial to evaluate the efficacy of Probiotic supplement to standard of care (Biotherapi group) Vs standard of care alone (Nonbiotherapi group) in patients with active RA. 250 male and female subjects on RA treatment for at least 3 months were randomized to receive 1 capsule orally, twice daily containing Probiotics supplements. Examinations were performed at Day 0, day 45±3, and day 90± 3.The Primary outcome was to see the number of patients who shows improvement in DAS28-ESR score. Secondary outcome was to see the changes in CDAI, HAQ and physician global assessment. Student t test was applied to see the difference between these two groups.

Results: At day 90, 26% cases among Biotherapy and 4.7% cases among Nonbiotherapi achieved DAS-28 remission(p=0.001) whereas 29.2% cases VS 7.1% cases achieved CDAI remission respectively (p=0.001).Mean HAQ and Physician general assessment showed a significant fall of 44.0% and 69.1% among Biotherapi VS 12.7% and 42.3% among Nonbiotherapi (p=0.001) during the same period. Six subjects had Giddiness, leg swelling, constipation and abdominal bloating in the Biotherapi group.

Conclusion: Overall, this study proved that BIOTHERAPI® has significant effect on disease activity and functional ability of RA patients when used in combination with pharmacological anti-rheumatic medications.


  PC0046: Myositis specific antibodies and muscle biopsy improve subclassification in ACR/EULAR IIM classification criteria in adults Top


M Irfan, L Rajasekhar, M Uppin, A Desai, D Phani Kumar, M Gavali; Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Background: The ACR/EULAR criteria capture the most frequent IIM subgroups but a subset remains unclassified.Myositis specific antibodies may improve the precision of classification.

Objectives: To describe clinical and serologic profile of idiopathic inflammatory myositis (IIM) patients and sub classifying them as per the ACR/EULAR criteria.

Methods: All consecutive patients diagnosed as IIM as per ACR/EULAR criteria between August 2018 to August 2019 were included. Myositis associated with other CTD were excluded.

Results: 27 patients were enrolled. Using criteria cut offs for with and without biopsy, 21 patients were definite and 6 probable IIM.Mean (SD) age was 42.4 (±11.9) years. Median (IQR) time to diagnosis was 7.8 (2-9) months.Mean MMT8 was 37/80. Muscle enzymes were elevated in 25, median(IQR) CPK 3513 (258-5099) IU/ml and LDH911 [76-293] IU/ml. 18 patients had skin manifestations:13 (9 adults) had DM specific rash (heliotrope, gottron sign/ gottron papule), 3 skin ulcers, 2 calcinosis, 1 digital gangrene.All 4 patients less than 18 years had classic DM rash.Of 27 patients, 9 were subclassified as DM, 4 JDM, 1 PM, 1 IMNM [Figure 1]. Twelve remained unclassified.Myositis Antibody profile (16 Antigen) was done in 20 patients (6 Mi2+,8 negative, Ro52 positive in 9). Muscle biopsy was done in 19 patients.
Figure 1

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In patients without classic DM rash, MSA helped in classifying 4 as DM (Mi2 positive) and 2 as ASS (OJ, PL7 positive). Findings of Perifascicular atrophy (2) and Perivascular inflammation (1) sub classified further 3 patients as DM and probable DM. Three remained unclassified despite MSA or biopsy being done.

Conclusion: Mi2 is the most frequent MSA observed in this cohort. MSA and biopsy improved subclassificationfrequency from 47% to 87% in adult patients with IIM. These Results: should be validated in larger cohorts and included in future revisions of IIM criteria.


  PC0047: A study of disease profile of adult and juvenile lupus patients at disease onset at tertiary care centre of northern India Top


Jeet Patel, Lalit Duggal, Neeeraj Jain, Mayank Gupta; Sir Gangaram Hospital, New Delhi, India

Objectives: to study disease profile of 100 adult and pediatric SLE patients at disease onset to find any significant difference in organ involvement in both cohorts.

Methods: 100 adult and 100 pediatric patients who classified SLE-SLICC criteria were recruited in study after taking consent at tertiary care centre in northern India. Demographic data and clinical profiles were recorded. Patients were asked direct questions related to different organ involvement (SLICC features and non-SLICC features like fatigue, Raynaud's Phenomenon etc.) at disease onset. Retrospective review of initial blood reports, including initial ANA, initial complements and anti-dsDNA were done. Fisher's test was used to find p value and p value less than 0.05 was taken as statistical significance.

Results: Out of 100 patients, females were 85% in adult cohort Vs 76% in pediatric cohort (with no statistical difference for sex distribution). Median delay in diagnosis was more with adult than pediatric cases. Lupus nephritis, mouth ulcers, NPSLE was common in pediatric SLE patients but lacked statistical significance. In adult cohort, there was significant association for fatigue, RP, and thrombosis. Average initial SLEDAI was higher in pediatric (16.59) than adult (12.9) SLE patients. Higher SLEDAI in Pediatric cases was might be due to weight based SLEDAI (CNS and lupus nephritis), even though the difference was not statistically significant. Results: suggest that pediatric and adult lupus patients differ in multiple aspects, and recognition of these differences helps in optimal treatment.


  PC0048: Comparing performance of UK FRAX versus Indian FRAX score for guiding treatment of osteoporosis in South Asian population living in United Kingdom Top


Suvrat Arya, Samantha Smitten, Srinivasan Venkatachalam; Cannock and Wolverhampton Rheumatology Centre, United Kingdom

Background: Osteoporosis is described as a 'progressive systemic skeletal disease characterized by low bone mass and microarchitectural deterioration of bone tissue, with a consequent increase in bone fragility and susceptibility to fracture. 'Osteoporosis' denotes a value for BMD (bone mineral density) that is 2.5 SDs or more below the young adult mean value for women. The FRAX tool (www.shef.ac.uk/FRAX) computes the 10-year probability of hip fracture or a major osteoporotic fracture.

Objectives: To compare performance of UK (United Kingdom) FRAX versus Indian FRAX score for guiding treatment of Osteoporosis in 100 South Asian patients living in United Kingdom.

Methods: Retrospective data of 100 consecutive South Asian patients who underwent DEXA (dual energy X-ray absorptiometry) Scan at Cannock& Wolverhampton Rheumatology Centre between April to July 2019 was analysed.

Results: Mean age of patients was 64.5 ± 10.8 years. Female to male ratio was 79:21. Mean BMI of patients was 27.7 ±5.1 kg/m2. 45% patients had previous fragility fracture, 12% had history of parental hip fracture. Only 9% patients had rheumatoid arthritis. Mean neck BMD and T- score were 0.85 ± 0.15 gm/cm2 and -1.24 ± 1.22 respectively. Correlation between UK and Indian FRAX was 0.76. Assessing fracture risk by UK FRAX and Indian FRAX recommended treatment in 34 and 23 patients respectively. There were 23 patients which warranted treatment in accordance with both UK and Indian FRAX, 11 patients by UK FRAX alone and none by Indian FRAX alone.

Conclusion: Assessing fracture risk in South Asian population by UK FRAX tool leads to more patients getting treated as compared to using Indian FRAX tool. Indian FRAX tool should be used for patients of South Asian ethnicity in UK to avoid over treating osteoporosis in this population subset.


  PC0049: Clinical features, management and follow-up of SLE patients seen at a rheumatology clinic Top


Anuj Shukla, Priyanka Gaur; Niruj Rheumatology Clinic, Ahmedabad, Gujarat, India

Background: Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease characterized by antibodies to nuclear antigens with remitting-relapsing clinical course. It is a heterogenous disease with variable clinical presentation. Here we share the experience of managing these patients.

Objective: To share the experience of patients presenting with the clinical features of SLE.

Methods: Clinical data of patients with the diagnosis of SLE from July2016 to June2019 was collated using clinical notes and personal interviews. EULAR-ACR Classification criteria, 2019 was used for inclusion in the study as SLE.

Results: 55-patients were diagnosed as SLE with mean age of 31+11years and disease duration 29+10months. 4-patients (7%) had juvenile onset of SLE. Among clinical features: 69%(n=38) had arthritis, 62%(n=34) skin rash, 49%(n=27) fever, 38%(n=21) oral ulcers, 34%(n=19) lymphopenia, 15%(n=8) nephritis, 9%(n=5) myositis, 9%(n=5) hemolytic anemia and 9%(n=5) thrombocytopenia. 11%(n=6) had secondary Anti-phospholipid-antibody syndrome.Kidney biopsies were available in 6-patients. Complement levels were low in 67%(n=37) patients and Anti dsDNA was positive in 69%(n=38) patients. Baseline SLEDAI score was 12+6.Mean follow-up duration was 15+11months. Immuno-modulators used for induction were mycophenolate mofetil(MMF) 34%(n=19), azathioprine 25%(n=14), cyclophosphamide 14.5%(n=8) and rituximab 9%(n=5). Immuno-modulators used for maintenance were azathioprine 41%(n=23), MMF 36%(n=21), methotrexate 11%(n=6), tacrolimus 1.8%(n=1), MMF+tacrolimus 1.8%(n=1) and hydroxychloroquine(HCQ) alone 1.8%(n=1). Mean prednisolone dose used during maintenance is 5+5mg/day.Out of 55-patients, 34(62%) are in remission (on drugs, n=27; off steroid, n=6; off treatment, n=1), 7(13%) have active grumbling disease, 13(24%) patients are on induction-phase and 1(1.8%) patient expired. 2-patients had pregnancy with successful outcome. Complications were infections 5%(n=3), hyperpigmentation 5%(n=3), osteonecrosis of hip 3.6%(n=2) and glucocorticoid-induced-osteoporosis 1.8%(n=1).

Conclusion: Thus, we collated data of patients with the diagnosis of SLE for better understanding of their clinical presentation, management and outcome. This will help in future to better formulate this cohort and plan a focused research topic.


  PC0050: Epidemiology and outcome of chronic nonhealing vasculitic ulcers: A retrospective study from tertiary care centre Top


C Saranya1, C Balaji1, M Vignesh1, R Shanmugesh2, R Ramu2, Mukaaram3, R Shankar3, S Rajeswari4 1Assistant Professor,2Senior resident,3Medical officer,4Professor and Head

Background: Cutaneous vasculitis due to primary or secondary vasculitis can manifest as chronic nonhealing ulcers.

Objective: To study the epidemiology and outcome of patients with chronic nonhealing vasculitic ulcer.

Methods: A retrospective medical record review study was done over a period of 1 year in our centre. We included patients with chronic nonhealing ulcer for more than 3 months with biopsy proven vasculitis. Patients with poor compliance and irregular follow up were excluded. We analyzed the clinical, laboratory parameters, treatment and outcome of these patients.

Results: 12 patients were analyzed, of which 25% were males and 75% were females. Mean age of the patients was 45+8.6 years. The mean duration of ulcers was 1.8years. 50% had unilateral leg ulcers and 50% had bilateral leg ulcers. Most common systemic involvement was musculoskeletal system(83.3%). The final diagnoses in our cohort were connective tissue diseases(CTDs) associated secondary vasculitis(50%), primary vasculitis(25%) and unclassified vasculitis(25%). Among the patients with CTDs associated vasculitis, 50% had Systemic lupus erythematosus, 33.3% had Rheumatoid arthritis and 8.3% had primary Sjogren's syndrome. Cutaneous manifestations other than ulcers include palpable purpura(25%) and digital gangrene(16.6%). Most common laboratory findings were anemia(75%) and leukocytosis(66.6%). 83.3% had bacterial superinfection of ulcer, of which 70% had polymicrobial infection. Most common organisms cultured were pseudomonas(60%), klebsiella(40%) and enterococcus(30%). Common comorbidities associated were hypothyroidism(25%), hypertension(25%), hyperthyroidism(8.3%) and gout(8.3%). Cyclophosphamide (66.6%) and mycophenolate mofetil (33.3%) were used as the immunosuppressive therapy in our cohort. 16.6% patients received surgical treatment(skin grafting) and 8.3% received platelet rich fibrin therapy. 66.6% patients showed complete resolution of ulcer following immunosuppression, while 25% had recurrence inspite of therapy. One patient died due to sepsis and high disease activity.

Conclusion: We analyzed the epidemiology of chronic nonhealing vasculitic ulcers. CTDs associated vasculitic ulcer was the most common cause and most amenable to treatment.


  PC0051: Factors influencing the damage and quality of life of patients with rheumatoid arthritis for more than five years, including adherence to treatment Top


Ram Krishna Giri, S Chandrashekara, C Renuka1, K R Anupama, Sani-E-Zehra, Veena Ramachandran, J Prakruthi; ChanRe Rheumatology and Immunology Centre and Research,1ChanRe Diagnostic Centre, Bengaluru, Karnataka, India

Background: Rheumatoid arthritis in long term causes joints damages and impairs the quality of life. The low compliance to drug is often attributed as one of the detrimental factors. The present study analyzed the factors influencing the cumulative damage and quality of life (QoL), including medication adherence.

Materials and Methods: Cross-sectional retrospective cohort study considered RA patients undergoing treatment for more than 5 years. Demographic details including. disease duration at presentation (DOP) and total disease duration (TDD) were collected from patient records. At the point of recruitment, patients' cumulative damages were assessed using Rheumatoid Arthritis Articular Damage (RAAD) score, QoL was assessed using the Indian Health Assessment Questionnaire (HAQ) Disability Index and Short Form of Arthritis Impact Measurement Scales 2 (AIMS2-SF). Medication adherence was evaluated using 8-Item Morisky Medication Adherence Scale (MMAS-8).

Results: 579 patients, (females 543, males 36) , mean age of 57 years and total duration of disease of 151 months. RAAD score moderately correlated (P <0.01) with DOP and TDD, while age and follow-up duration demonstrated week correlation (P <0.01) (Table 1). The Indian HAQ DI correlated weakly with age (P <0.01), gender (P <0.05), DOP and TDD (P <0.01); however, MMAS-8 had weak inverse correlation (P <0.01). AIMS2-SF normalized physical score correlated weakly with age, TDD (P <0.01), and DOP (P <0.05). AIMS2-SF normalized affect score was inversely weakly correlated (P <0.01) with age. AIMS2-SF normalized social interaction score was weakly correlated (P< 0.05) with follow-up duration.

Conclusion: Age, gender, duration of disease at presentation and total duration of disease influence the cumulative damage and QoL in RA patients. Medication non-adherence influenced the disability but had no significant influence on the damage. The study emphasizes the fact that early intervention is the key.


  PC0052: Factors predicting long-term outcome in patients with undifferentiated connective tissue disease Top


J Prakruthi, S Chandrashekara, C Renuka1, K R Anupama; ChanRe Rheumatology and Immunology Centre and Research,1ChanRe Diagnostic Centre, Bengaluru, Karnataka, India

Background: Undifferentiated connective tissue diseases (UCTD) include a wide variety of conditions that share clinical features and autoantibody profile with other connective tissue diseases, but do not fulfil their criteria. However, it is difficult to predict the duration of treatment and involvement of other organs and the need for regular medications.

Objective: To verify the factors associated with complications or those requiring additional DMARD treatment in patient with UCTD.

Materials and Methods: The longitudinal retrospective study considered an interim analysis of 57 patients (48 females and 9 males) diagnosed with UCTD during the period June 2004 and January 2015. The corresponding mean±sd age and median (range) total disease duration noted for the subjects were 48.4±13.36 yrs and 82 (29-300) months. Demographic, laboratory and clinical parameters were extracted at baseline and last follow-up visit. The patients' outcome was grouped into group 1, who either discontinued medications or could be maintained only with hydroxychloroquine and group 2 who developed into definable CTD or complication or required higher DMARD. Comparison and univariate and multivariate logistic regression were performed to assess the factors associated with disease complications p <0.05 was considered statistically significant.

Results: Twenty were in group 1, 37 in group 2. Group 2 had significantly increased ESR (48 (9-120) vs. 27.5 (10-70)) and follow-up duration (71 months (26-179) vs. 50 (25-179)) than less severe UCTD patients. Logistic regression showed that NLR, ESR, and presence of any autoantibody at presentation were associated with increased likelihood of complications in UCTD patients by 17%, 4% and 41% respectively.

Conclusion: Female gender, NLR, high inflammation parameters like ESR, and presence of any autoantibodies at presentation predicts the patients diagnosed to be UCTD for future organ involvement. Further studies with large sample size and individual autoantibody markers are underway.


  PC0053: Factors predicting the pattern of outcome of rheumatoid arthritis: A more than five-year follow-up study Top


Veena Ramachandran, S Chandrashekara, C Renuka, Ramya Rao, Ramkrishna Giri; ChanRe Rheumatology and Immunology Centre and Research, Bengaluru, Karnataka, India

Background: With regard to long-term outcome of rheumatoid arthritis (RA), patients follow three classical patterns. Type 1: patients who attain remission can be either maintained on minimum or no DMARDs, type 2: patients having an undulant course of remission and relapse, and need to switch over to alternate DMARD, and type 3: patients who have an aggressive course and remission is hardly achieved. The present study has investigated the factors influencing the final outcome.

Materials and Methods: Seven hundred and fifty RA patients fulfilling the 2010 ACR/EULAR criteria were recruited. The study was approved by the institutional ethics committee and informed consent was obtained from all patients. The demographic details and duration of illness at diagnosis, duration of first symptom and the treatment given were retrieved from their records. The laboratory parameters obtained at the recruitment phase and at fixed intervals were RF, ANA, ESR, CRP, and NLR. The DAS scores at the specified intervals were also obtained, and average DAS scores were calculated. Interim analysis of the data of first 84 patients was done. P<0.05 was considered for statistical significance. One-way ANOVA was performed.

Results: Eighty-four patients (mean age:54.75±12.76) with mean duration of illness(13.26±6.92) and mean duration of first symptom (5.49±6.04) were grouped as type 1(n=22, 26.2%), 2(n=22, 26.2%)and 3(n=32, 38.1%) based on the average DAS scores. 8(9.5%) were excluded from the study. Age (P<0.001), duration of first symptom (P<0.005), NLR (P<0.001) rheumatoid factor (P<0.001) at presentation, total duration of disease (P<0.021), CRP (P<0.024) and ESR (P<0.042) were found to be significant with regard to the disease outcome.

Conclusion: Age, duration of first symptom, CRP, NLR, ESR, and rheumatoid factors at presentation may assist in predicting the probable course of RA.

Keywords: Disease activity score, neutrophil to lymphocyte ratio, rheumatoid arthritis


  PC0054: The clinical profile of macrophage activation syndrome secondary to systemic lupus erythematosus Top


Soumya Dey, Basil Paul, Dipendranath Ghosh, Alakendu Ghosh, Parasar Ghosh, Pradyot Sinhamahapatra; IPGMER and SSKM Hospital, Kolkata, West Bengal, India

Objective: To investigate the clinical profile of macrophage activation syndrome (MAS) associated with systemic lupus erythematosus (SLE).

Methods: The clinical profile of 10 patients with SLE associated MAS diagnosed in the Department of Rheumatology, IPGMER and SSKM Hospital, Kolkata from September 2018 to August 2019 was retrospectively analyzed.

Results: Seven patients were female and three were male. There were 3 cases of Juvenile SLE. The average age was 22.2 years. The average disease duration of SLE before the occurrence of MAS was 2.9 years, and the average SLE disease activity index (SLEDAI) was 15.6. The mean complement levels at the time of onset of MAS was 49.5 and 8 for C3 and C4 respectively. Six out of 10 patients had a major organ involvement secondary to SLE, nephritis being the most common. All patients developed fever, cytopenias, elevated triglycerides, hyperferritinemia, transaminitis and elevated LDH. Splenomegaly was found in 9 patients. Six patients developed coagulopathy. Only one patient had a bone marrow picture suggestive of hemophagocytosis. Glucocorticoids were used in all patients, in the form of initial intravenous pulse Methylprednisolone therapy for 3-5 days followed by oral Prednisolone at 1 mg/kg/day. All ten patients responded to therapy and had improvement in clinical as well as laboratory parameters. There was no mortality.

Conclusion: In patients with SLE, MAS was most commonly seen in young females with active disease. Fever and splenomegaly were the most consistent symptom and examination finding respectively. Consistent lab findings included cytopenias, elevated triglycerides, hyperferritinemia, transaminitis and elevated LDH, with low complements. Although the typical bone marrow picture of hemophagocytosis was found in only one patient, early therapy with high dose glucocorticoids was instituted on the basis of initial clinical suspicion and fulfillment of the other lab criteria for diagnosis, leading to improved clinical outcomes in all the patients.


  PC0055: Interstitial lung disease in sjogrens syndrome: A descriptive analysis of a retrospective cohort from a tertiary centre Top


Prathyusha Manikuppam, Aswin Nair, G Arvind, Ashish Jacob Mathew, Ruchika Goel, DebashishDanda, John Mathew; CMC, Vellore, Tamil Nadu, India

Introduction: Pulmonary disease affects 10% to 20% of patients with Sjogren's syndrome.

Aim: To describe the different characteristics of patients with interstitial lung disease in Sjogren's syndrome.

Patients and Methods: A retrospective chart review of Sjogren's patients with interstitial lung disease was done using hospital records between the years 2014 to 2019.

Results: and Conclusion: We observed that there were 30 Sjogren's patients with interstitial lung disease.Age of onset was in range of 40 to 60 years of age. There were 27 females and 3 males.21 of 30 patients had Anti- SSA positivity. Anti – SSB was done for 26 patients , 17 patients had positive antibodies.The following patterns of ILD were observed:NSIP – 14 patients, LIP – 4 patients, UIP – 3 patients, Early ILD – 3 patients, Undifferentiated pattern – 4 patients. 26 patients received 0.5mg/kg prednisolone equivalent at the time of diagnosis except in 4 patients where 1mg/kg was used. This was for vasculitis in 2, immune mediated neuropathy in 1, and BOOP in 1.Mycophenolate mofetil was started in 24 out of the 30 patients as first steroid sparing agent. One patient developed immune mediated neuropathy when on azathioprine , so 4 doses of pulse cyclophosphamide were given followed by Mycophenolate mofetil.One patient was given 4 doses of pulse cyclophosphamide followed by MMF.Two patients received Rituximab , one received in view of leucocytoclastic vasculitis and ILD NSIP , one received for arthritis and ILD NSIP followed up with MMF.7 patients received azathioprine as first steroid sparing agent, 1 patient received azathioprine after he had symptomatic arthritis on MMF.We will be describing the response of ILD to different second line agents , MMF,azathioprine, and rituximab.


  PC0056: Profile of antisynthetase syndrome in a tertiary care centre Top


K S Sreejitha, D Phani Kumar, Meghna Gavali, Liza Rajasekharl; NIMS, Chennai, Tamil Nadu, India

Introduction: Antisynthetase syndrome (ASS) is characterized by antibodies against different aminoacyl-tRNA synthetases and presents with arthritis, myositis,and interstitial lung disease (ILD). Raynaud's phenomenon,mechanic's hands, and fever are other findings in ASS.

Materials and Methods: Patients with ASS were identified based on diagnostic criteria proposed by Connors et al.[1] 2014 to 2019.Patients' demographic details, clinical features, autoantibody profile, treatment & outcomes were noted.

Results: There were a total of 23 patients and 18 of them were females. The median age was 44 years(37.5-53) and disease duration 18 months(12-39). The most common presenting complaint was arthritis (18/23) followed by fever (14/23).On examination, mechanics hand was found in 12,myositis in 10 and Raynaud's in 4 patients. 18 patients had ILD, with 17 having NSIP and 1 with UIP pattern on HRCT chest.PFT values were available at baseline for 9 patients (mean FVC 65± 3.6%, mean FEV1/FVC 0.94). Anti Jo1 antibodies were the commonest(14/23) followed by PL-7 (5/23),PL-12 (3/23) and EJ (1/23).Ro 52 was positive in 10 patients and ANA in 11,the commonest being cytoplasmic pattern. The combination of arthritis, myositis and ILD was present only in 6 .In the non Jo1 group, all patients had ILD (9/9) and only 2/9 had myositis, compared to 9/14 and 8/14 respectively in Jo1 group (p=0.06) .In a median follow-up of 26 months(4-24) ,2/23 expired and the rest have good response to immunosuppressive treatment.

Conclusion: ASS is more common in females.Arthritis was the most common initial manifestation. Arthritis and myositis are more common among Jo1 positive patients.ILD is more common than myositis in nonJo1 patientsNSIP pattern in HRCT is the most common type of ILD seen.

Reference

1. Connors GR, Christopher-Stine L, Oddis CV, Danoff SK. Interstitial lung disease associated with the idiopathic inflammatory myopathies: What progress has been made in the past 35 years? Chest 2010;138:1464-74.


  PC0057: A single centre study to assess therapeutic outcomes in patients with idiopathic inflammatory myositis on CRD Pune Indian HAQ Top


Nachiket Kulkarni, Anuradha Venugopalan, Manjit Saluja, Arvind Chopra; Centre for Rheumatic Diseases, Pune, Maharashtra, India

Objectives: To assess therapeutic outcome In patients with Idiopathic Inflammatory Myositis on CRD Pune Indian HAQ.

Materials and Methods: Data from 108 patients of connective tissue disorders (CTD) with dominant Inflammatory Myopathy(IM) evaluated in CRD with patient database since 1996. Data obtained for period 2004-2016. Center for Rheumatic Diseases (CRD) Pune developed a modified version of the Health Assessment Questionnaire(HAQ) catering customs, lifestyle of the Indian community (CRDHAQ). It was recorded in all patients. It is a validated instrument and available in many Indian languages. Physician Global recorded on Likert scale of five-category response with an ascending score ranging from 1 to 5 (asymptomatic to severe). Patient Global health recorded on horizontal 100-mm line (VAS), anchored at 0 for 'extremely poor health' and 100 for 'perfect health. Indices recorded at first visit, on 6 monthly visit and at 1 year.

Results: Dermatomyositis (DM), Polymyositis (PM) and Overlap Myositis (OCTD) noted in 36, 28 & 41 patients respectively. All Patients received glucocorticoids. Methotrexate prescribed in (92%), Azathioprine in (28%), hydroxychloroquine in (88%) Mycophenolate Mofetil in (6%). IVIG in 2 patients for acute IIM with interstitial pneumonitis. Rituximab in 4 resistant cases; all responding favorably.The average CRDHAQ scores at first visit, 6th month and 1 year for DM, OM & OCTD were( 10, 18 & 16), (2, 10, 4) and (2, 8, 2) respectively. The Physician Global for these groups at first visit, 6th month and 1 year were (moderate, severe, moderate), (mild, moderate, mild), (mild, mild, mild) respectively. The Patient Global Scores were (70, 80, 70), (40, 50, 30), (20, 30, 10) respectively in these three visits.

Conclusion: The therapeutic outcome of IIM in current cohort was favorable. CRD Pune Indian HAQ could be used as an easy office based patient centric tool for monitoring response to therapy in patient with IIM.

[TAG:2]PC0058: Clinical, laboratory profile and treatment response of a South Asian sarcoid cohort from a tertiary centre: A retrospective study [/TAG:2]

Silas Vinay Vidyadhar Rao, Rahul Sahu, Aswin Nair, G Arvind, Debashish Danda, John Mathew; Department of Clinical Immunology and Rheumatology, CMC, Vellore, Tamil Nadu, India

Background: Sarcoidosis is a heterogeneous multisystem inflammatory disease of unknown etiology.

Objectives: To study the epidemiological, clinical, laboratory and histopathological profile of patients diagnosed to have sarcoidosis.To assess the outcome and treatment response of these patients on follow up.

Methods: Patients diagnosed as Sarcoidosis under the department of Clinical Immunology and Rheumatology at CMC, Vellore were retrospectively chosen from the institutional electronic medical records. Demographic profile, clinical features, laboratory parameters, management and outcome of these patients categorized definite or probable sarcoidosis were analysed.STUDY DURATION: 10 yrs –June 2009 to June 2019

Results: A total of 181 patients with sarcoidosis were identified. Mean age of our cohort of patients was 43.8 yrs (SD+-11.19); 62(34.3%) were male and 119(65.7%) were female. Angiotensin Converting Enzyme (ACE) levels were elevated in 80(44.1%) patients. 103 (56.9%) patients underwent biopsy, of which 93 (90.3 %) had non caseating granulomas. HRCT was done in 164(90.6%), with findings as mentioned in [Table: 1]. 11(6 %) patients each had hepatic and skin granulomas, cardiac involvement was noticed in 7(3.8%), and 2(1.1%) had vasculitis.In this study the different second line immunosuppressive agents used and their response will be described. We will also be looking at the total steroid dosage and the duration of use.
Table 1

Click here to view


Conclusion: This is the largest South Asian cohort till date to the best of our search. The male to female ratio was 1:2. Among those patients with a definitive diagnosis of sarcoidosis less than half patients had elevated ACE levels.


  PC0059: Diffuse alveolar haemorrhage in systemic lupus erythematosus: A single centre retrospective study Top


Saurabh Chahande, Aswin Nair, G Arvind, Ruchika Goel, Ashish Mathew, Debashish Danda, John Mathew; Christian Medical College, Vellore, Tamil Nadu, India

Introduction: Diffuse Alveolar haemorrhage (DAH) is a rare, but serious manifestation of SLE. It may occur early or late in disease evolution.

Aim: Reporting our experience with Diffuse Alveolar Haemorrhage in patients with Systemic Lupus Erythematosus.

Patients and Methods: Records of SLE patients admitted between years 2005 and 2019 were reviewed. 18 patients of SLE were admitted with episodes of DAH. For all study subjects, the demographic, clinical, laboratory, therapeutic and outcome data were Abstracted.

Results and Conclusion: All 18 patients were females. Their age ranged from 14 to 50 years and disease duration ranged from 3 to 84 months. All our patients presented with pulmonary infiltrates and hemoglobin drop. 4 out of 18 patients had Central Nervous System involvement. 12 out of 18 patients had renal involvement which was the most common extra pulmonary SLE manifestation. 8 out of 18 patients were admitted in ICU. Average stay of patient in hospital ranged from 7 to 32 days. 6 out of 18 patients had APS serology positivity (4 lupus anticoagulant, 1 anti ps/pt, 1 beta-2 glycoprotein, 1 anticardiolipin ). All patient had low C3 levels. Initial treatment included intravenous methylprednisolone in 16 out of 18 patients. Plasmapheresis in 8 patients, Cyclophosphamide was given in 4 patients, Rituximab in 3 patients and intravenous immunoglobulin in 1 patient. There were 4 deaths in the 18 patients.Out of 8 patient who received Plasmapheresis, 4 patient who received only plasmapheresis died, while one who received only plasmapheresis survived. Two patients with Cyclophosphamide along with Plasmapheresis survived, while another patient with plasmapheresis with subsequent cyclophosphamide and IVIG survived. All 3 patient receiving Rituximab and 4 patient receiving Cyclophosphamide alone survived


  PC0060: Our patients with primary sjogren's: Does our social structure help despite severe symptoms? Top


S Pandya, R Solanki, A Parmar, M Bavaliya, N Patel, R Sharma, A Sanap, S Chikani, P Srivastava, R Shah, A Shukla, V Sharma, T Parikh; Vedanta Institute of Medical Science, Ahmedabad, Gujarat, India

Objective: To analyse demographic and laboratory characteristics of patients presenting to outpatient private clinics and their HRQOL

Materials and Methods: We collected data prospectively from 12 private clinics of the city in paper proformas which also had a QOL questionnaire inclusive of both physical and psychosocial aspects. Study duration was one month (June 15th to July 15th, 2019). The baseline data was analysed using descriptive statistics.

Results: Total 59, Please see table. On the QOL-PSS Questionnaire, 1. More patients had physical discomfort than psychosocial problems and 2. except for the questions 'my family understands me' and 'I have a good feeling about my body' where the majority were very positive, the rest were equivocal about their replies.

Conclusion: While the demography matched from patients elsewhere, more patients had physical pain/discomfort than being negative on the psychosocial aspects. Does living in joint families give greater social security to our patients and hence a less negative feeling – will need to be explored by further studies.
Table 1

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  PC0061: Palindromic rheumatism, agonizing but remitting variant! Top


S Pandya, P Shenoy, R Solanki, T Parikh; Vedanta Institute of Medical Science, Ahmedabad, Gujarat, India

Objective: To analyse demography of patients of PR, their response to therapy and comparison with another group.

Materials and Methods: Study Period: Feb '16 to July '19. Retrospective analysis (descriptive) of data – demographic, laboratory and response to therapy. Remission was defined as no attack of pain or swelling over 6 months. We also compared our data to that from another group from South India

Results: Please see table. While 87% of our patients were CCP positive, 71% were Rheumatoid factor positive.85% of our patients were in remission at a mean of 11.7 months' follow up.Compared to the other group, our patients presented earlier.

Conclusion: Although the severity of pain was more in PR, most patients achieved remission on cDMARDs. More data is needed on whether HCQ alone should be enough in managing these patients.



[TAG:2]PC0062: Comparison of HRCT and mantoux test in patients with quantiferon TB gold positive for screening of latent tuberculosis infection in patients with systemic inflammatory rheumatic diseases prior to treatment with biological disease-modifying antirheumatic drugs[/TAG:2]

Vidya K Bangar, John Mathew, Debashish Danda, Ashish, Ruchika, Ashwin, Arvind, Shivraj; Christian Medical College, Vellore, Tamil Nadu, India

Introduction: Comparison of HRCT and Mantoux test in patients with Quantiferon TB gold positive for screening of latent tuberculosis infection (LTBI) in patients with systemic inflammatory rheumatic diseases (SIRDs) prior to treatment with biological disease-modifying antirheumatic drugs (bDMARDs).

Aim: To compare HRCT and Mantoux test in patients with Quantiferon TB gold positive for LTBI in patients with SIRDs for treatment with bDMARDs.

Materials and Methods: The study included patients admitted in ward for last 8 months for bDMARD treatment and who had Quantiferon TB gold positive . They were analysed for HRCT and Mantoux test. These patients were given TB prophylaxis before initiating bDMARDs . They were followed and monitored for any features of tuberculosis flare.

Results: We found 20 patients with Quantiferon TB gold positive. It was found that 5(25%) were positive for Mantoux (>10mm) and 15(75%) were negative for same. 5 (25%) out of 20 had HRCT evidence of infection and 15 (75%) patients had normal HRCT. 2 (10%) patients had all 3 test positive. 2(10%) had both Mantoux and Quatiferon TB gold positive and HRCT Normal. 3(15%) had HRCT and Quantiferon TB gold positive with Mantoux negative. They were followed and monitored for any features of tuberculosis flare, none of them develop tuberculosis.

Conclusion: In our patients screened for latent TB by Quantiferon TB gold , Mantoux test and HRCT, Quantiferon TB gold was the most sensitive test.


  PC0063: Assessment of quality of life in patients of axial spondyloarthritis using asqol questionnaire and its correlation with disease activity and functional indices Top


S Bhatt, N Pachera, V Vasdev, Ramakant, A Kumar; AHRR, Delhi, India

Background: The Ankylosing Spondylitis Quality of Life (ASQOL) questionnaire is an AS-specific measure of QOL designed for monitoring patients and evaluating treatment effects of new pharmaceutical products. There is paucity of studies assessing ASQOL in Indian population and various factors associated with poor QOL.

Objectives: In this study, we aimed to assess the QOL in AS patients using this tool and its correlation with disease activity and functional indices. Secondarily, we also studied the factors associated with poor QOL

Methods: 102 consecutive patients attending Rheumatology clinic at the institute fulfilling the latest ASAS criteria for AS were enrolled. Those having any other comorbidities that affect QOL were excluded. Recruited subjects were provided with the ASQOL questionnaire to self-assess the effect of disease on their QOL. Simultaneously, disease activity and functional status were assessed using BASDAI, BASFI, BASMI and ASDAS scores. Subjects were followed up at 1 month and 6 months and scores calculated. Association was studied between age and sex, duration of study, HLA B27 status and the ASQOL.

Results: Mean ASQOL score at baseline was 9.6 ± 4.4 and declined significantly during follow-up (6.04 ± 3.6 and 5.39 ± 3.4 respectively). ASQOL scores positively correlated with BASDAI, BASFI, ASDAS and BASMI scores. A significant positive correlation was also observed between age, duration of disease and ASQOL scores at various time intervals. HLA-B27 status did not affect QOL or any other disease activity indices significantly in our study.

Conclusion: ASQOL is an objective measure of QOL and varied significantly at different time intervals probably in relation to the effect of treatment. QOL was also adversely affected by age of the subjects and the duration of the disease as reflected by higher ASQOL scores. High disease activity and poor functional status was also associated with poor QOL.


  PC0064: Comparison of sulfasalazine versus leflunomide based combination therapies in patients with psoriatic arthritis failing methotrexate monotherapy: A randomized controlled trial Top


Manoj Kumar, K C Shanoj, Laxmisha Chandrashekar, K T Harichandra Kumar, M B Adarsh, K G Chengappa, V S Negi; Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

Background: Evidence regarding efficacy of various conventional disease-modifying anti-rheumatic drugs among psoriatic arthritis (PsA) patients are scarce particularly among those with inadequate response to methotrexate (MTX).

Objective: The study compared the efficacy of Sulfasalazine (SSZ) and Leflunomide (LEF) based combination therapies in terms of achievement of psoriatic arthritis response criteria (PsARC) response among the patients with PsA who had persistent active disease despite maximum tolerable dose of MTX.

Methodology: In this 12 weeks, assessor-blinded randomized controlled trial, patients with predominant peripheral polyarthritis who failed to achieve minimal disease activity (MDA) with maximum tolerable doses of methotrexate, were randomized to receive: sulfasalazine (2 gm per day) and methotrexate combination (24 patients); or leflunomide (20 mg per day) and methotrexate combination (25 patients). Primary outcome measure was proportion of patients achieving PsARC response and secondary outcome measures were proportions of patients achieving ACR20, ACR50, MDA, and DAPSA remission response.

Results: Forty-nine patients were enrolled after screening 188 patients. Baseline characteristics were comparable in both the treatment arms. At 12 weeks, PsARC response was achieved by 100% and 84% of patients treated with MTX and SSZ versus MTX and LEF respectively (P = 0.11). MDA was achieved by 75% and 60% of the patients treated with MTX and SSZ versus MTX and LEF respectively (P = 0.26). DAPSA remission response was achieved by 58% and 40% patients treated with MTX and SSZ versus MTX and LEF respectively (P = 0.19) [Figure 1] LEF was discontinued in one patient due to rise in creatinine. No other serious adverse effects were noted.

Conclusion: Both the combination therapies are efficacious and safe among the South Indian patients with polyarticular psoriatic arthritis and inadequate response to methotrexate monotherapy. There was a trend (non-significant) toward better response with sulfasalazine and methotrexate combination suggesting need of large scale.
Figure 1: CONSORT flow diagram and treatment reasponses among two treatment arms. ACR-American college of rheumatology; DAPSA-Disease activity in psoriatic arthritis; ITT-Intention to treat; LEF-Leflunomide; MDAMinimal disease activity; MTX-Methotrea

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  PC0065: Smartphone application based management in rheumatoid arthritis: SMART- RA study, one year experience Top


Ashit Syngle, Nidhi Garg, Kanchan Chauhan; Healing Touch City Clinic and Fortis Hospital Mohali, Chitkara University, Rajpura, Punjab, India

Background: Compliance in RA remains critical to treatment outcomes but poses challenges in day-to-day care. In technology driven era, with more people having access to smart phones, opportunities exist for use of phone-based technologies.

Objective: Investigate impact of smart-phone application (HealthCius) on inflammatory disease activity and QOL in RA over one year.

Methods: 75 consecutive RA patients recruited. Subjects randomized into 2 groups. First, having access to smart-phone were assigned to intervention group using Healthcius application (n=45) and second, the control group not using application (n=30). Patients in two groups received standard treatment for RA. To patients, app was their individual treatment plan. It helped them comply by providing an easy to refer checklist, reminders, alerts and visual dashboard of their progress through the day. The app served as the doctor's virtual assistant inside the patient's smart phone. For the doctor, it was a live dashboard of all patients and their real time compliance levels. Outcome measures included ESR, CRP, DAS28 and HAQ-DI at baseline and 1 year.

Results: The two groups did not differ significantly for baseline characteristics. There was significant difference between control and intervention group for DAS28 (p <0.05), ESR (p≤0.05), CRP (p≤0.05) and HAQ-DI (p≤0.5) after 1 year in favor of smart phone application. Analysis within the groups revealed significant improvement in DAS28 (p<0.05), ESR (p=0.01), CRP (p=0.001) and HAQ-DI (p=0.01) in the application group as compared to control group. Impact of DMARDs usage was also evaluated at the end of the study and it was found that average drug usage of DMARDs was less in intervention group.

Conclusion: Greater improvement in inflammatory disease activity and QOL in smart phone application assisted RA patients suggesting that smart phone technology can be used to leverage health benefits in RA.


  PC0066: Demographic profile of patients presenting with vasculitides (especially AAVs) from western India Top


Dhaiwat Shukla, Prashant Chotalia, Puja Srivastava, Rutviz Mistry, Sapan Pandya; Smt NHL MMC, Ahmedabad, Gujarat, India

Objective: To study the demographic and clinical profile of patents presenting with vasculitides (AAVs).

Materials and Methods: Retrospective analysis of the patients presenting with vasculitides (especially AAVs) during the period Oct 2017 to Oct 2019 at one tertiary care government centre(i.e. VSGH) and one private OPD(i.e. Vedanta) was done.Informed consent was taken.

Results: Total 107 patients have been included in the study. Mean age was 41.38+-15.35. Of them, 26(24.30%) had large vessel vasculitis (Takayasu arteritis), 11(5.34%)had medium vessel vasculitis(polyarteritis nodosa) rest had various forms of small vessel vasculitides. Mean BVAS of the patients was 17.68+-11.26.Subgroup analysis:-patients of AAV (ANCA associated vasculitides)

Conclusion: Of vasculitides presenting to the OPDs, maximal were AAVs. Distribution of these and their demographic and clinical features matched those from another study from North India. More data with follow up is needed to compare outcomes across different zones of India.
Table 1

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  PC0067: A retrospective analysis of disease outcome at 6 months in patients of RA managed at a municipal tertiary care centre: An observational study Top


Authors: Affiliation:

Objective: To study outcome in patients of RA at 6 months of starting their treatment, and to decide the factors influencing remission

Methodology: All patients fulfilling ACR 2010 criteria attending Rheumatology OPD in a tertiary care hospital in the Western part India from December 2015 to August 2019 have been included. Patients taking oral steroids at any point of time during study period have been excluded. Their baseline data, including demographic data and disease activity were analysed. At 6 months follow up their disease outcome measures were reanalyzed. Patients in remission at 6 months ( DAS28 <=2.6) were compared to those who were not.

Results: Total 256 patients with mean duration of illness 5.47+-5.96 years and a mean age was 47.25+-12.34. Their baseline DAS28 ESR was 4.18+-2.57. Mean HAQ at baseline was 1.1+-1.0.

Conclusion: At 6 months of follow up, about 2/3rds achieved EULAR remission with DMARDs and no cortiocosteroids. Males, those with higher baseline DAS 28 ESR and seropositive patients were more likely to achieve remission. More data with longer follow up would be useful to validate the judicious use of DMARDs WITHOUT corticosteroids in our settings.
Table 1: Validation of 2019 European League Against Rheumatism/American College of Rheumatology classification criteria in 303 systemic lupus erythematosus patients

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  PC0068: A study on efficacy of adalimumab biosimilar in patients of spondyloarthritis Top


H Raghavendra, T N Tamilselvam, S Balameena, R Ramesh, S Mythili, S Karthikeyan, N Balakrishnan; Department of Rheumatology, Madras Medical College and RGGGH, Chennai, tamil Nadu, India

Aim: To study the efficacy of adalimumab biosimilar in bDMARD naïve patients and compare it with infliximab response in an age matched group of SpA patients.

Materials and Methods: Patients admitted in Institute of rheumatology, Madras medical college with newly diagnosed SpA(Axial) as per ASAS definition were included in the study. 20 patients were given Inj. Adalimumab biosimilar 40 mg sc every fortnightly for a total of 12 weeks . Their baseline ASDAS, BASDAI, BASFI were calculated and followed after 12 weeks of therapy. The response was compared with age-matched control group of 20 patients who had received Infliximab for the above indication.

Inclusion Criteria:

  1. Patients above the age of 16 years.
  2. Known case of AS diagnosed as per ASAS definition.
  3. Patients who are HLA-B27 positive.
  4. BASDAI>4, ASDAS> 2.1, CRP>6mg/L.


Exclusion Criteria:

  1. Spondyloarthritis associated with psoriasis, IBD, Reactive arthritis.
  2. HLA-B27 negative patients.
  3. Active tuberculosis, HBV or HCV infection.
  4. Inactive or low disease activity according to ASDAS-CRP scoring.


Primary Outcome: ASAS 20 response at 12 weeks.

Results: A total of 20 patients received Inj Adalimumab biosimilar as bDMARD along with NSAIDs . 30%(6) patients achieved ASAS 20 response at the end of 12 weeks whereas 80%(16) of patients in the infliximab group had achieved this response after the bolus doses. Patients in infliximab group had a greater improvement in outcome scores when compared to adalimumab biosimilar.

Conclusion: Adalimumab biosimilar was less efficacious in patients with AS when compared to infliximab bio-originator however, the patients who had response had good improvement in BASDAI and BASFI scores.


  PC0069: Validation of 2019 Eular/Acr classification criteria for systemic lupus erythematosus in a south Indian cohort Top


B Harikrishnan, Suma Balan, Jyothi Srikanth, C B Mithun; Department of Clinical Immunology and Rheumatology, AIMS, Amrita ViswaVidyapeedham, Kochi, Kerala, India

Background: New 2019 EULAR/ACR classification criteria for SLE have been recently published. Validation studies for the new criteria were done in other parts of the world.

Objective: To compare the real world performance of 2019 EULAR/ACR classification criteria when applied to a known cohort of SLE cases in Kerala.

Methods: We retrospectively reviewed the electronic medical record of 30,541 patients who visited the Rheumatology department of AIMS, Kochi, a tertiary care center, from January 2014 to June 2019. 347 patients diagnosed with SLE by qualified experienced rheumatologists were included in the study. 44 patients were later excluded as they had overlap syndrome. Thus, there were 303 SLE patients (107 juvenile SLE and 196 adult SLE). From the 30,541 patients, another 303 patients(107 juvenile and 196 adult), who attended the rheumatology department on first week of each month, from 2014 January to 2019 June and diagnosed with other autoimmune diseases, were selected as controls. They were selected regardless of their specific clinical or immunologic manifestations. Patients were excluded if the diagnosis was uncertain. Each patient was evaluated to see if he or she satisfied the 1997, 2012, and 2019 criteria.

Results:

Conclusion: The new 2019 EULAR/ACR criteria attained better sensitivity, PPV, NPV and accuracy when compared to ACR 1997 and SLICC 2012 criteria; and better and almost same specificity as compared to SLICC 2012 and ACR 1997 criteria, respectively.


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  PC0070: Clinical characteristics of patients with isolated AntiDFS 70 antibody positivity Top


B S Vishnu, S Rajesh1, Department of Family Medicine, Kerala Institute of Medical Sciences,1Kerala Institute of Medical Sciences, Thiruvananthapuram, Kerala, India

Dense fine speckled (DFS) pattern is the most frequent pattern in high titre ANA-positive healthy persons is associated with anti-DFS70 antibody. There is negative association between anti-DFS70 antibodies and systemic autoimmune rheumatic disease, especially if no concomitant ANA associated rheumatic diseases (AARD)-specific autoantibodies are found. Published cohorts revealed association with various conditions like alopecia, asthma, atopic dermatitis, behcet's disease, celiac disease, chronic fatigue syndrome etc. We assessed the isolated Anti DFS positive patients to look into the clinical characteristics. 91.6 % of female patients predominated the group with equal proportion in less than 30yrs and 30 – 50 yrs group of 45.8% each. Fibromyalgia was the most common clinical feature accounting for 37.5 % among these patients. 12.5 % of patients with clinical features suggestive of undifferentiated connective disease had Anti DFS antibody positivity. Other system involvement was of predominant dermatological involvement seen as psoriasis, bullous pemphigoid and erythema dyschromicum perstans. Single organ autoimmune disease was another group with Anti NMDA encephalitis and autoimmune thyroiditis accounting for same. Other inflammatory spectrum included idiopathic uveitis and inflammatory bowel disease.

References

  1. Mahler M, Parker T, Peebles CL, Andrade LE, Swart A, Carbone Y, et al. Anti-DFS70/LEDGF antibodies are more prevalent in healthy individuals compared to patients with systemic autoimmune rheumatic diseases. J Rheumatol 2012;39:2104-10.
  2. Mahler M, Hanly JG, Fritzler MJ. Importance of the dense fine speckled pattern on HEp-2 cells and anti-DFS70 antibodies for the diagnosis of systemic autoimmune diseases. Autoimmun Rev 2012;11:642-5.
  3. Miyara M, Albesa R, Charuel JL, El Amri M, Fritzler MJ, Ghillani-Dalbin P, et al. Clinical phenotypes of patients with anti-DFS70/LEDGF antibodies in a routine ANA referral cohort. Clin Dev Immunol 2013;2013:703759.



  PC0071: A study on atherogenicity in systemic lupus erythematosus and its relationship with disease activity Top


Sujatha Narayanan, T N Tamilselvam, R Ramesh, S Mythili, S Karthikeyan , M Sabarinath, S Balakrishnan, S Nikhila, Archana Singh; Department of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: Cardiovascular disease is one of the major causes of morbidity and mortality in patients with systemic lupus erythematosus (SLE). The increased cardiovascular risk is both due to increased prevalence of classical risk factors and inflammation due to SLE per se. We wanted to study how much the risk is increased among SLE patients compared to healthy controls and among patients with active SLE(SLEDAI ≥4) and those in remission, by estimating the Atherogenic index of plasma(logarithmic value of triglycerides by HDL cholesterol), a value shown to correlate with cardiovascular risk in various studies.

Objectives: 1. Estimate the Atherogenic Index of Plasma(AIP) in SLE patients and comparison with healthy controls.2. Comparison of AIP among patients with active SLE and those in remission.

Methods: This cross-sectional observational study included newly diagnosed SLE patients. Data was collected from a total of 72 SLE patients, 72 non SLE controls. Disease activity was measured by SELENA-SLEDAI. SLE patients with Metabolic Syndrome(MetS) were excluded in the data analysis comparing patients with active SLE and those in remission. Data analysis was done using Wilcoxon signed rank test and Mann-Whitney U test.

Results: AIP was significantly increased among SLE patients when compared to general population (p value<0.0001) and significantly increased among SLE patients with MetS when compared with those without MetS (p value <0.01).However, there was no significant difference in AIP values between patients with active SLE and those in remission (p value>0.05)

Conclusion: The study Results: reiterate the fact that both metabolic syndrome and SLE contribute independently to the increased cardiovascular risk seen in SLE as measured by AIP. However, there was no alteration in atherogenicity in SLE patients with relation to disease activity. This emphasizes the importance of careful monitoring for cardiovascular risk in SLE even in remission.


  PC0072: Neutrophil to lymphocyte ratio and platelet to lymphocyte ratio as activity markers in rheumatoid arthritis: A cross sectional study from Rajasthan, India Top


Parag Vijayvergia, Gopal Krishana Bohra, Abhishek Purohit, Kamala Kant Shukla, Kuldeep Singh; All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

Introduction: Rheumatoid arthritis (RA) is an inflammatory disease with a prevalence of 1%.Disease severity is assessed by the Disease Activity Score of 28 joints (DAS-28) system. Recent studies suggest that NLR and PLR correlate with disease severity and other inflammatory markers suggesting a potential role for novel but inexpensive activity markers.

Objective: To assess correlation of NLR and PLR with CRP, ESR and explore the relationship with DAS 28 score.

Methodology: Patients diagnosed with RA using ACR/EULAR 2010 criteria were included. Patients with any other co-morbidities including diabetes, hypertension, cardiovascular diseases and other connective tissue diseases were excluded. DAS 28 ESR score was calculated and inflammatory markers and complete blood count were performed.

Results: Sixty-eight patients of RA were screened and 54 included. Fifty were females, 4 patients were early RA while others had established disease. All patients were on methotrexate with 25% on additional DMARDs. Five patients were on prednisolone at the time of assessment. 78% patients had active disease with DAS 28 ESR >3.2. A weak positive correlation was observed between PLR and Hs CRP levels(r = O.31, p= 0.02). No correlation was observed between NLR and PLR with disease activity or with ESR.

Discussion: In contrast to previous reports, NLR or PLR were not associated with disease activity. We have excluded any comorbidity which might affect NLR and PLR. Steroid use was <5% in our patients. These make our observations fairly robust.

Conclusion: Though NLR and PLR levels are reported to be higher in RA patients, they do not appear to reflect disease activity.


  PC0073: Clinical and laboratory features in the first inception cohort of SLE in India: The data of first 466 patients Top


Avinash Jain, Murugavangini Egambaram1, Asma Chougule2, Janany Parathasarthy3, Parmeshar Sindhu4, Sindhura Gajula5, V S Negi1, K G Chengappa1, Ramnath Misra, Vineeta Shobha2, Benzeeta Pinto2, Ashish Jacob Mathew3, Parasar Ghosh6, Manish Rathi4, Liza Rajasekhar5, Ranjan Gupta7, Bidyut Das8, Saumya Ranjan Tripathi8, Amita Aggarwal

SGPGI, Lucknow, Uttar Pradesh,1JIPMER, Puducherry,2St. John's Medical College, Bengaluru, Karnataka,3CMC, Vellore, Tamil Nadu,6IPGMER, Kolkata, West Bengal,4PGI, Chandigarh,5NIMS, Hyderabad, Telangana,7AIIMS, Delhi,8SCB, Cuttack, Odisha, India

Introduction: SLE is a multisystem disease with varying clinical manifestations and multiple autoantibodies. To date, all series of SLE from India have been retrospective. Thus, we prospectively studied clinical features at presentation in an inception cohort. Further, we studied if there were difference(s) in clinical or laboratory(lab) features of patients presenting early (within 6 months of symptoms) or late.

Methods: Indian SLE inception cohort for research (INSPIRE) is a multi-institutional cohort in which patients, satisfying SLICC criteria and disease duration ≤ 3 years, presenting to nine institutions after Aug 31, 2018 were enrolled. All demographic, clinical and lab data were collected in a standardized case report form and entered on web-based database.

Results: Among 466 patients, 426 were females. Most common clinical manifestations were mucocutaneous (87.1%) and fever (70.6%). Other manifestations included arthritis 309 (68.4%), serositis 110 (23.6%), renal 214 (45.9%), CNS 124 (26.6%), hematological 234 (50.2%), DVT (12.4 %) and ILD (8.5%).In mucocutaneous domain, most common was non-scarring alopecia (78.5%) followed by oral ulcers (57.7%), ACLE (55.1%), DLE (22.7%) and SCLE rash (15%). Among 214 patients with renal involvement, 87 out of 134 biopsied patients revealed proliferative nephritis. Mortality rate was 1.1 % with active disease in all.Lab abnormalities are highlighted in [Table 1]. ANA was negative in ten patients. High dsDNA and low complements were seen in 66.8% and 72.1% respectively. Most common antibodies after dsDNA were anti-smith and anti-Ro (37.1% and 35.4%) and 21.3% had anti-phospholipid antibodies.Patients presenting late more commonly had arthritis, hemolytic anemia and renal involvement but were less commonly febrile. Mean baseline SLEDAI did not differ but they had more patients with severe disease activity. Mortality rate was similar.

Conclusion: Mucocutaneous, constitutional features were most common in our cohort with higher renal involvement and severe disease activity in patients presenting late.
Table 1: Clinical, biochemical and scrological profile

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  PC0074: Gender differences in psoriatic arthritis and relevance of age of onset of psoriasis Top


Taral Parikh, Sapan Pandya, Rakesh Solanki; Vedanta Institute of Rheumatic diseases, Ahmadabad, Gujarat, India

Background: Males with SpA have more axial disease, in females peripheral involvement is dominant.

Objective: As gender differences have not been studied well in PsA, we did this study.

Methods/Results: 180 patients, from 2012-2019, fulfilling the CASPAR criteria, analysed. Population was stratified by age of onset of psoriasis (cutoff 40 years).Male 117/ female 63. Mean age 43.07(±11.3), weight 70.8(±13.8), duration of psoriasis onset 7.9(±7.2), duration of PsA 3.5(±4.34), PASI; 3.4(±6.09), SJC 4.3(±4.5), TJC 6.2(±5.3), patient global assessment for psoriasis 3.4(±2.6), patient global assessment of PsA 6.6(±1.7). 16.5% -family history of psoriasis.Pattern: Axial+oligoarthritis 25(21%), axial+polyarthritis 21(17%), axial+distal 4(3.5%).Isolated: polyarthritis 32(27%), oligoarthritis 29(24%), axial 5(4%), 1 (0.8%)DIP.Enthesitis 1(7.5%), dactylitis 49(33.7%).

Conclusion: Males had more axial and oligoarticular involvement and females polyarticular involvement, even when stratified by age of onset. Females had higher joint counts and disease activity and males were more obese.


  PC0075: Appearances are deceptive- behavioral patterns are different. Purpura of sjogren's syndrome versus henoch schonlein purpura- experience from a tertiary care centre Top


Rajeswari Sankaralingam, Ramu Ramasamy, Saranya Chinnadurai, Balaji Chilukuri, Vignesh Manthram, Shanmugesh Selvaraj, Aishwarya Ramachandran Department of Rheumatology, SRIHER, Chennai, Tamil Nadu, India

Background: Enigmatic among all vasculitides is the small vessel vasculitis – Purpura especially in Sjogren's Syndrome(SS) along with its closest mimic Henoch Schonlein Purpura(HSP).

Objective: To compare the Purpura of Sjogren's syndrome and HSP.

Methods: A prospective observational study was conducted by Department of Rheumatology, in a tertiary care centre between Jan 2018 to June 2019. The clinical, laboratory profiles and histopathology(HPE) of adult HSP and Sjogren's were studied.

Results: Total number of adult onset HSP was n=4, M:F was 2:2, mean age 39.25 years, mean duration of illness:13.5 days. All had palpable purpura, most common site was lower limbs(n=3), abdomen(n=1)and generalized(n=1). Other clinical features were polyarthralgia(n=2), polyarthritis(n=2), abdominal pain(n=2), vomiting(n=2)and hematuria(n=1). UGI scopy showed chronic gastritis(n=2) and erythematous ulcer in terminal ileum with proctitis(n=1). Investigations showed leukocytosis(n=4), mean ESR 37mm/hr, raised CRP(n=2), positive cryoglobulins(n=1)and reduced complements(n=1). HPE showed leucocytoclastic vasculitis(LCV)(n=4). Renal biopsy showed IgA deposits(n=1).Total number of Sjogren's patients was n=15, of whom 9 had recurrent palpable purpura. M:F was 0:9, Mean age was 43.11 years and mean disease duration 3.6 years. Most common sites were generalized(n=8) and localized(n=4) in lower limbs. Common striking clinical features were polyarthralgia(n=6), polyarthritis(n=1), sicca symptoms(n=9) and interstitial lung disease(n=2). Antibodies positive were RF(n=4), ANA(n=5), SSA & SSB(n=6), Low complements C3 & C4 (n=3) and elevated serum gammaglobulins(n=3)were seen. HPE showed varying Results, LCV(n=1), lymphocytic vasculitis(n=3) and anetoderma(n=1).

Conclusion: Both males and females were equally affected in HSP showing LCV in the HPE. Only females were affected in SS and were older. In SS, polyarthralgia was common and disease duration longer. All who had RF positivity had elevated acute phase reactants, severe purpura, hypergammaglobulinemia and mononuclear vasculitis. Cryoglobulin positivity –Is it a separate entity?


  PC0076: Prevalence of articular and extra articular features of joint hypermobility in fibromyalgia Top


Pooja Belani, C B Mithun1, Joe Thomas2, Arun Tawari1, Vishad Viswanath; Institute of Rheumatology and Immunology Sciences, Thiruvananthapuram,1Department of Rheumatology, Amrita Institute of Medical Sciences and Research Centre,2Aster Medcity Hospital, Kochi, Kerala, India

Background: Fibromyalgia(FMS) and Joint hypermobility syndrome(JHS) are common causes of chronic pain syndrome with many overlapping features.

Objective: To study the prevalence of joint hypermobility and extra-articular features of JHS in patients with FMS.

Methods: A prospective cohort of FMS and JHS each were recruited from three tertiary rheumatology care facilities in Kerala from June2019 to August2019.Prevalence of Joint hypermobility and extraarticular manifestations of Joint Hypermobility were captured in the FMS cohort using a predesigned proforma and compared to those in the cohort with JHS.Patients satisfying ACR2010 criteria for FMS and Beighton score of ≥ 4/9 for BJHS were included in the respective group.Statistical analysis was done using SPSS 25.0.

Results: A total of 40 FMS patients and 74 JHS patients were recruited. Mean age was 39.9±11yrs in FMS vs 49.9 ±12.6yrs in JHS. Noteworthy,Beighton score ≥ 4/9 was present in 67%FMS patients while 36.5% of JHS patients fulfilled FMS criteria. Among the clinical features,dragging pain (77% vs 34%) and fatigue (95% and 77%), both p<0.005, were more common in FMS group,whereas myopia (54% vs 28%) and scoliosis((35% and 2.6%) were more frequent amongst JHS group,both p<0.001. 2 groups were comparable in terms of other features of JHS as varicose veins,joint crepitus,calf pain,foot abnormality and enthesopathy.Significantly,Beighton score negatively correlated with age in both the groups (-0.334 in FMS group and -0.49 in JHS, p<0.05).

Conclusion: Clinical features of JHS are common in FMS patients and could contribute substantially to the morbidity in FMS.Overlooking the features of Joint Hypermobility Syndrome,can lead to mislabeling as FMS. Importantly , there is a negative correlation between age and Beighton score. Therefore, at older age,diagnosis of JHS may require special attention to extraarticualar features of JHS rather than relying on Beighton score alone.


  PC0077: Questionnaire based assessment of patients' beliefs, knowledge and perception about rheumatoid arthritis Top


Ranjan Gupta, Anju Mohan Renjith; Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, India

Background: The long-term outcomes in rheumatic diseases not only depend upon the drugs but also on the patients' education, beliefs and their perception about their disease and other non-pharmacological interventions like exercise.

Objective: To assess the patients' beliefs, knowledge and perception about rheumatoid arthritis (RA) through a questionnaire

Methods: A questionnaire (Hindi Language) containing 28 multiple choice questions assessing the patients' knowledge about RA in 4 domains (a. etiology, disease process, signs & symptoms b. drug therapy & monitoring c. joint protection, exercise, coping and goal setting d. individual specific issues) related to the disease was prepared and applied to 300 consenting patients. All questions had an option of 'I don't know'. Epidemiological information was collected for all including their socio-economic and education status.

Results: There was a wide variation in the range of correct responses in different domains. Option 'I don't know' was exercised for total of 36.7% times in whole questionnaire. Only 33% patients knew that etiology for RA is unknown and 66% were aware that RA is not congenital. Only 19% patients are aware that it's not curable and 70% patients follow dietary restrictions for better relief. 25% patients are aware of the reasons for repeating liver and renal function tests and 55% believe that rheumatoid factor and anti-CCP antibodies are useful for treatment monitoring. Only 27% patients knew about DMARDs and 33% and 11% could correctly identify NSAIDs and steroids respectively. Almost 42% patients knew that regular exercises can prevent deformities but only 7% knew that they need to be done during active disease also. Only 7% patients are aware of using larger joints than smaller to preserve energy and prevent joint damage.

Conclusion: This study highlights the poor knowledge base of RA patients in a north Indian population and the need for effective educational programs.


  PC0078: A prospective study on the relationship of USG findings, dental hygiene and antiCCP titres on joint erosions in rheumatoid arthritis Top


Debarup Das, Shaoli Ghosh, Koushik Basu; Medical College and Hospital, Kolkata, West Bengal, India

Background: We have tried to assess the correlation between the USG findings with the extent of joint damage in Rheumatoid arthritis patients to see whether it can be reliably used for monitoring in follow up and to seek any relationship of dental health status and anti-CCP titers with the erosive changes.

Objectives: 1. To assess the correlation of USG joint erosions with corresponding X Ray findings. 2. To assess the correlation of anti-CCP and dental hygiene with correspondingX-ray erosivefindings.

Methods: 50 Rheumatoid patients as per ACR/EULAR criteria, of disease duration < 2 years, attending our center selected randomly. Patients were clinically assessed for condition of their dental hygiene, and RF and anti-CCP titres, ESR & CRP values were assessed. High resolution Power Doppler (4-12 Hz) USG of joints was done to assess the joint erosions and digital X-rays obtained were analyzed to assess the joint erosion as per modified Van der Heijde(VDH) scoring system. Parameters were checked at presentation, and after 8 months. Changes in USG joint erosion scores at the end of the study were compared with that of X-ray erosions by correlation and regression analyses. Anti-CCP titres were compared with VDH scores at presentation, and at 8 months, and an Anova test was done to assess the effect of the condition of dental hygiene on VDH scores.

Results: We found a positive correlation between change in USG and X Ray erosions (correlation coefficient 0.66). Poor dental hygiene and higher anti-CCP titres were found to be associated with higher erosive changes, both at presentation and at the end of study period (p value 0.008).

Conclusion: USG was found to be a sensitive tool in assessing progression of erosive changes in rheumatoid. Dental health and antiCCP titres also showed a significant impact on joint erosions.


  PC0079: Differences between adult and pediatric onset henoch-schonlein purpura from south India Top


Pranav R Chickermane, Vishnu S Chandran, Clint Sunny, Suma Balan, C B Mithun, Jyothi Srikanth; Department of Rheumatology and Clinical Immunology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Background: Henoch-Schonlein purpura (HSP) also known as IgA vasculitis, is an immune complex-mediated small vessel vasculitis. Predominantly a disease of childhood, it is considered to be rare and to have a more severe course in adults. There is paucity of data comparing the disease spectrum in children and adults.

Objectives: To investigate the differences in clinical manifestations and outcomes in pediatric and adult onset HSP.

Methods: The case records of a total of 149 patients fulfilling the new EULAR/ PRINTO/PRES criteria for HSP, who attended the Rheumatology OPD at a tertiary care hospital in south India over the last 6 years (2013-2018) were retrospectively analyzed. Pediatric onset HSP was defined by age at onset of HSP below 18 years. The clinical features, laboratory investigations and outcomes in the paediatric and adult onset HSP groups were compared. Outcome (complete recovery, relapse, persistent proteinuria and/or hematuria, or progression to chronic renal failure) at last visit was assessed in patients with a minimum follow up duration of 6 months.

Results: 78 patients with pediatric onset and 71 with adult onset HSP were seen during this period. During the disease course, children had a higher frequency of gastro-intestinal involvement (84.8% vs 45.1%, P< 0.01) compared to adults. Renal involvement was more common in adult onset than pediatric onset HSP (57.7% vs 39.2%, P <0.05) with 3 adult onset HSP patients progressing to chronic renal failure compared to one in the pediatric onset HSP group. Complete recovery was more common in children than adults (75.7% vs 41.2%, P<0.05).

Conclusions: We found statistically significant differences in clinical manifestations and outcomes between patients with pediatric and adult onset HSP. Gastro-intestinal involvement was more common in children than adults. Adults with HSP had more frequent renal involvement and worse renal outcomes than children.


  PC0080: A study of serum procalcitonin levels in systemic onset juvenile idiopathic arthritis Top


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

Background: Patients with systemic onset Juvenile idiopathic arthritis (SOJIA) often present with clinical features similar to an infection. Differentiating the infection from a disease flare is a challenge to the clinician and there are very few studies in the literature about the role of procalcitonin in differentiating these two.

Objectives: 1. To determine the diagnostic role of serum procalcitonin in differentiating between infectious and non-infectious inflammation in febrile patients of SOJIA. 2. To determine the role of procalcitonin and CRP in differentiating the disease flare from macrophage activation syndrome(MAS).

Materials and Methods: Patients who are diagnosed with SOJIA (diagnosed by ILAR criteria) and presented with fever(≥38oC) between March 2018 to July 2019, were recruited and were stratified into three groups( Infection group, Disease flare, & Disease flare with MAS) based on their clinical profile. Respective Mean & Mode values are calculated and compared. ROC curve analysis was used to determine the respective cutoff values.

Results: Out of 43 hospital visits of 20 patients with SOJIA, 7 had infection, 15 had disease flare with MAS and 21 had disease flare without MAS. The median values of procalcitonin in infection & non-infectious groups were 1.72(IQR:15.01) 0.38(IQR:2.12). There was no statistical significance(Mann whitney U test was used, p value: 0.35). On the ROC curve, the cutoff value of procalcitonin to differentiate MAS group from disease flare alone group is 0.46ng/ml with a sensitivity and specificity of 80% and 81% respectively (p value:0.001). Likewise, the cutoff value of CRP is 93.6 mg/L with a sensitivity and specificity of 73.3 & 61.9% respectively (p Value: 0.049).

Conclusion: From our study we conclude that procalcitonin may not be helpful in differentiating the infectious from non-infectious inflammation in febrile patients of SOJIA. Those patients with MAS, even without any infection, procalcitonin levels can be elevated.


  PC0081: Rituximab in patients with refractory myositis: A single center experience Top


Sharma Lucky, Natasha Negalur, J N Durga Rao Yadavalli, Tanna Dhaval, Dhiren Raval, Shounak Ghosh, Vinay Singal, Rajiva Gupta; Medanta-The Medicity Hospital, Gurgaon, Haryana, India

Background: Rituximab, a B cell depleting CD20 monoclonal antibody is being widely used in the management of refractory IIMs. B cells play an important role in the initiation and propagation of the immune response and are implicated in the pathogenesis of myositis.

Objective: Our objective was to assess the efficacy of Rituximab in patients with refractory myositis.

Methodology: We conducted a retrospective cohort study of 11 IIMs patients (5DM, 5PM, 1 Overlap) who attended Rheumatology and Clinical Immunology Clinic at Medanta, The Medicity between March 2017- 2019. All patients had refractory disease (inadequate response to at least two immunosuppressive agents along with concomitant glucocorticoid therapy unable to taper in 6 months) criteria. The patients received two infusions of rituximab (1 g each) at baseline, followed by repeated dose after 6 months. Baseline immunosuppressive therapy was maintained, and glucocorticoid dose was tapered according to clinical/laboratory parameters.

Results: 11 patients were included in the study.10 were female and 1 male patient. The mean disease duration was 2.2 +/- 1.21 years. The demographic, clinical and laboratory profile is shown in table I and II respectively. Almost 75% of the patients attained clinical and laboratory response after 1 yr. A significant reduction in median glucocorticoid dose was achieved at the end of one year (20mg vs 8.86mg per day). However, none of the patients were off steroid completely. No severe infection was noted during study period.

Conclusion: This study shows that rituximab is an effective therapy in refractory IIM patient. Though the MMT 8 score did not improve significantly, Rituximab therapy did help in reducing the dose of corticosteroid. However, a larger cohort with definite trial design is warranted.


  PC0082: Jo1 versus non Jo1 myositis: Flip side of the same coin? Top


J N Durga Rao Yadavalli, Natasha Negalur, Wasim Kazi, Sharma Lucky, Rohit Bajaj, Kaustubh Telang, Shruti Bajad, Rajiva Gupta; Medanta-The Medicity Hospital, Gurgaon, Haryana, India

Background: IIM is a heterogeneous group of disorders with varied phenotypes and prognosis. Myositis specific antibodies have helped in sub classification and in both diagnosis, predicting the response to treatment and prognosis.

Objective: This study was aimed to study the differences in clinical features between Jo1 and Non-Jo1 subgroups.

Methods: This was a prospective observational study carried out at the department of Rheumatology and Clinical Immunology at Medanta Hospital, Gurgaon from August 2017 to July 2019. A Total of 89 consecutive patients who were diagnosed with IIM and fulfilling the Bohan and Peter criteria were included. These patients were divided into Jo1 and Non-Jo1 subgroups. Disease characteristics, lab features, treatment details were noted and analyzed.

Results: Total 25 pts(28%) out of 89 had MSA positivity. Among those Jo1 positivity was seen in 10(11.23%), PL-7 in 2(2.24%), PL-12 in 1(1.12%), SRP in 4(4.49%). Mean age was 35 +/- 8.37 and 42.64 +/- 15.85 years respectively in both groups. PM was seen in majority, about 70% in Jo1 and 50% in Non-Jo1 respectively, with Overlap Myositis in 2(25%) of Non-Jo1 group. Mechanics hands, Arthritis, Fever were seen more commonly in Jo1 as compared to Digital ulcers which were seen in Non-Jo1 group. ILD was seen in 7(70%) , 5(62.5%) respectively in both the groups. Ro was the predominant MAA observed in the study population. Median CPK (mg/dl) was 2994.5 in Jo1 and 604.5 in Non-Jo1 respectively. Non-Jo1 patients had less severe muscle disease.

Conclusion: Jo1 and Non-Jo1 subgroups have distinct phenotypes. Fever, Mechanics hands, Arthritis in Jo1 and Digital ulcers in Non-Jo1 were seen predominantly. Also, Non-Jo1 group had less severe muscle weakness and lower CPK values and association with Overlap Myositis.


  PC0083: Wait for it to work: Building up right dose of duloxietine in fibromyalgia Top


Arun Tiwari, C B Mithun, Sandeep Surendran, V Shekhar; Amrita Institute of Medical Sciences, Kochi, Kerala, India

Background: Fibromyalgia is a prevalent chronic pain syndrome characterized by widespread pain and other unspecified somatic symptom. Duloxetine doses are to be increased gradually to maximum of 60 mg/day to attain maximum efficacy in fibromyalgia patients. Assessing efficacy at the doses lower then target doses can be misinterpreted as non-responders.

Objectives: To evaluate the efficacy and side effect profile of Duloxetine in fibromyalgia patients after 12 weeks of therapy.

Methods: The study was conducted under Department of Rheumatology, AIMS, Kochi. Patients enrolled in study, had visited rheumatology department from January 2018 to September 2019. It was a single centre, retrospective, crosses sectional study. Fibromyalgia patients who had good compliance to therapy and completed at least 3 follow ups at 12 weeks , were included in the study. Total 169 patients were finally included in the analysis after exclusions. The response to treatment was noted with difference in fibromyalgia revised questionnaire from baseline. Improvement in FIQR of >14 was consider as responder to Duloxetine.

Results: The response of treatment was noted on follow up visits after at least 2 months of treatment. Out of enrolled cases 102 were responders and 67 were non responders. Most common side effect noted in our patients was constipation (19) followed by dyslipidemia (18), gastritis /gastroesophageal reflux disease (GERD) symptoms (10), dry mouth (5), deranged liver function (4), giddiness (3), headache (1) and one patient had to discontinue duloxetine because of severe tiredness.

Conclusion: This study provides evidence that 12 weeks treatment with Duloxietine with gradual escalation dose upto 60 mg /day, in fibromyalgia patients improves fibromyalgia symptoms significantly. The relief is beyond pain improvement. The drug is well tolerated in fibromyalgia patients and the side effect profile is mild and it can be managed mostly with symptomatic treatment in appropriate doses.


  PC0084: Juvenile lupus nephritis: Clinico-serologic presentation and outcomes compared to adults in a tertiary referral center Top


Anu Daber, Manoj Kumar, M B Adarsh, K G Chengappa, V S Negi; Jawaharlal Nehru Institute of Post Graduate Medical Education and Research, Puducherry, India

Background: Juvenile lupus nephritis presents with severe disease at onset and follows an aggressive disease course compared to adult-onset disease, with higher morbidity and irreversible renal damage.

Objective: 1.To study demographic, clinic-immunologic characteristics, renal activity, outcomes and cumulative renal and extra-renal damage in juvenile lupus nephritis.2.To compare renal response and cumulative renal and extra-renal damage with an adult population of similar disease severity at onset.

Methodology: The hospital records of children diagnosed with lupus nephritis below 18 years of age from 2009 to 2018 were reviewed. 211 juvenile l patients were screened and 31 patients with lupus nephritis having adequate records and regular follow up were included.59 adult patients with comparable renal disease at baseline were selected for comparison. The data collected included demography, clinical, serologic and renal biopsy parameters, therapy given, response at 12 months, renal and extra-renal damage.

Results: Both groups were comparable at onset of nephritis with regard to hypocomplementemia, SLEDAI scores, severity of proteinuria, histopathologic class of lupus nephritis and renal biopsy activity and chronicity indices. However, hematuria and high titre anti-dsDNA positivity was significantly higher in juvenile group. Anti-Sm, anti-Ku and anti-cardiolipin antibodies (IgG and IgM) were more commonly present in juvenile group. Both groups received similar treatment with IV cyclophosphamide for induction followed by azathioprine or MMF for maintenance. Renal response at 12 months was comparable between the groups. However, the juvenile group had a significantly higher percentage of patients with eGFR<50 ml/min at 12 months. The renal and extra-renal SDI scores at end of follow up were comparable between the groups.

Conclusion: Despite similar response to treatment among both groups, juvenile patients are predisposed to develop CKD stage 4 or 5 at end of 12 months with anti-phospholipid antibody positivity being an important confounding factor.

https://drive.google.com/file/d/1plQdr2ktlzSb-ScMJv7MFkpMZkK7Rafx/view?usp=sharing.
Figure 1: (a and b) Renal outcome at last follow up, (b) Extra-renal damage

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  PC0085: Experience of biologics and small molecules in rheumatic disease in Gujarat Top


Sapan Pandya, Reena Sharma, Nishil Shah, Puja Srivastava, S S Chikani, Namisha Patel, Ripal Shah, Amruta Sanap, Himanshu Pathak, Manish Bavaliya, Alpana Parmar, Vishnu Sharma; Rheumatology Association of Gujarat, Gujarat, India

Background: Rheumatology Association of Gujarat (RAG) maintains a biologic registry of rheumatic diseases since 2017. This study aims to provide insight on the use of various biologics and small molecules used in various indications.

Methods: A registry was initiated in January 2017 and a common proforma was used to record data from 13 centers of patients on biologic in various rheumatologic conditions. The demographics, indication, duration of disease, co-morbidities were documented.

Results: The registry has 862 patients (454, 53.66% males) with a mean age of 41.51 ± 15.42 years and total duration of illness of 7.28 ± 6.18 years. The details of the baseline disease and frequency are shown in the chart. The table summarizes the relative use of biologics and small molecules, mortalities and cause of death associated with the use of the drugs. Out of 540 patients, 485(9.19%) patients had partial improvement/remission after starting biologic/small molecule. However, inflation of this percentage due to loss of followup of those who did not improve could not be ruled out.

Conclusion: Most common indication of biologic use is Rheumatoid Arthritis while the most commonly used biologic is Rituximab.Majority of the deaths were due to infections after starting biologics.
Figure 1: Frequency of use of biologic in various rheumatic diseases

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  PC0086: Assessment of response of conventional synthetic disease-modifying anti-rheumatic drugs after withdrawal of abatacept on patients of rheumatoid arthritis with remission or low disease activity Top


A T Atal, S Kartik; Command Hospital, Pune, Maharashtra, India

Background: One biological Disease-Modifying Anti-Rheumatic Drug (bDMARD) Abatacept was withdrawn from Indian markets recently which necessitated a change in therapeutic strategy. Patients who had achieved remission or low disease activity were switched over to conventional synthetic DMARDs and followed up.

Objective: Assess the response to csDMARDs after withdrawal of Abatacept on patients of Rheumatoid Arthritis in remission/low disease activity.

Methods: This prospective study included patients of RA on Abatacept followed up at our Center from Jan 2018 to Jan 2019. Patients in remission or low disease activity(LDA) were switched to the csDMARD regime in optimised combinations and adequate doses as per existing guidelines.Clinical Disease Activity Index (CDAI )was documented at baseline, 3 and 6 months. Primary end-point was the maintenance of at least LDA as defined by a CDAI of 10.

Results: 19 patients( Males- 01, Females-18) were assessed for change of regime from Abatacept.1 patient with moderate disease activity was commenced on monthly subcutaneous Golimumab. She achieved LDA at 3 months and remission at 6 months. Of the remaining 18, 4(22%) were in remission and 14(78%) had LDA. In the remission group, at 3 months, all remained in remission, however, at 6 months, 3 were in remission and 1 in LDA. In the LDA group, 10 remained in LDA at 3 months while 4 progressed to moderate disease activity (MDA): 9 remained in LDA at 6 months and 5 patients had MDA. No patient defaulted and no one was lost to follow up. Of the 18 patients with LDA or remission at study onset, maintenance of at least LDA was achieved by 13 (72%)at 6 months follow up.

Conclusion: Majority of patients who have achieved remission or low disease activity with Abatacept can be continued on csDMARDs .


  PC0087: Rituximab in refractory lupus nephritis Top


Subodh Gururani, D Phani Kumar, Liza Rajasekhar; NIMS, Hyderabad, Telangana, India

Background: Many patients with lupus nephritis(LN) are resistant to initial immunosuppressive therapy.Outcomes with change of treatment in patients with therapy refractory LN(RLN) [no improvement at 3 months, no partial remission (PR) at 6 months or no complete remission (CR) by 2 years] are not well documented.

Objectives: To document outcomes of change in immunosuppressive therapy in RLN patients.

Methods: It is observational, longitudinal, bidirectional study.Patients fulfilling criteria of RLN were enrolled consecutively from August 2018 to January 2019.Patients were observed after enrolment up to August 2019 and followed up three monthly.Treatment details,outcomes [CR, PR,no-response (NR)], doubling serum creatinine and death were recorded.Group comparisons were done using t-test and chi-square test. Factors affecting renal response were studied using linear regression.

Results: Fifty-one patients were enrolled. Duration of follow-up after first change in treatment was1 year (0.5-1.6).Thirteen received rituximab and thirty-eight change in conventional immunosuppressants (CIS):cyclophosphamide,mycophenolic acid,tacrolimus alone or in combination.Baseline characteristics of two groups are shown in [Table 1]. Median duration of nephritis was significantly longer in rituximab group.There were more patients refractory to >1 immunosuppressant in rituximab group (p=0.002). There was no difference in proportion of biopsy classes (proliferative, membranous and mixed) between two groups.CIS group had significantly greater renal response than rituximab group.Raised dsDNA, low complements,number of systems involved, biopsy class and duration of SLE or nephritis did not affect outcomes.Three deaths were recorded in CIS group and none in rituximab group.No patient developed doubling of serum creatinine or ESRD.

Conclusion: Rituximab was given to patients who had longer duration of nephritis and were refractory to multiple immunosuppressants.Renal responses were significantly better in those receiving CIS.A crossover study of early versus late rituximab in RLN is needed.
Table 1: Baseline characteristics and outcomes achieved

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  PC0088: Retrospective analysis of MAS/2°HLH patients presenting to a rheumatology department in north India over one year Top


Manesh Manoj, Prashant Bafna, Kasturi Hazarika, Rasmi Ranjan Sahoo, Anupam Wakhlu; King George's Medical University, Lucknow, Uttar Pradesh, India

Discussion: We retrospectively analysed 8 patients of MAS/2°HLH who presented to the Rheumatology department of a tertiary care centre over a period of one year. The 8 cases consisted of 3 cases of SLE, 1 case of T cell lymphoma masquerading as SLE, 1 case of probable disseminated TB with granulomatous myositis, 2 cases of AOSD and 1 case of SoJIA. The mean age was 30 years (range 10 - 46 years) with equal number of males and females. The median duration of illness was 12 months (range 1 to 60 months). One patient's diagnosis had to be revised after clinical evolution and another patient's diagnosis could not be conclusively proved by histopathology. The clinical features and lab values are described in [Table 1]. In three (38%), infection was considered the possible triggering factor. One was associated with malignancy. In patients with infections, one had lower respiratory tract infection and two were positive for CMV by PCR. ALT and/or AST were elevated in six patients (75%). Three patients had pancytopenia (38%), four had bicytopenia (50%) and one had only thrombocytopenia. Six patients were treated with 3 to 5 days pulse MPS (30 mg/kg) followed by Inj dexamethasone at 10mg/m2 in five. Two were not pulsed. All except one patient was started on cyclosporine. One patient of SoJIA was put on 2mg/kg prednisolone after initial pulse steroids. Etoposide according to HLH 2004 protocol was used in one patient due to cyclosporine induced PRES (posterior reversible encephalopathy syndrome). Six patients survived (75%). One succumbed to malignancy induced HLH and the second, a case of probable disseminated TB also succumbed to the disease.
Table 1: Clinical features and laboratory characteristic of patients

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Conclusion: This analysis highlights the varied presentations and the need for a high index of suspicion in order to detect and treat this deadly condition early.


  PC0089: Hypophosphatemic osteomalacia – close mimic of rheumatological disorders Top


Ishita Shah, Vaibhavi Velangi, Yogesh Preet Singh, Abhishek Patil, K Sharath, Vikram Jain, Aditya Hegde, Karthik Prabhakar, Vishad Vishwanath, Pooja Belani; Rheumatology Fellow Manipal Hospital, Bengaluru, Karnataka, India

Background: Hypophosphatemia due to increased urinary phosphate excretion is the predominant cause of the osteomalacia seen with the disorders of vitamin D metabolism. Hypophosphatemic osteomalacia can occur due to hereditary hypophosphatemic rickets syndrome or tumor-induced osteomalacia. Drug-induced Fanconi syndrome can result in renal phosphate wasting and osteomalacia. Here we present 12 cases of hypophosphatemic osteomalacia with different etiologies most of whom were referred to a rheumatologist with a diagnosis of spondyloarthritis.

Methods: Data was collected retrospectively from medical records from January 2015 to September 2019. Osteomalacia was diagnosed by Bingham and Fitzpatrick criteria (2 of the following: low calcium, low phosphate, elevated alkaline phosphatase or suggestive radiographs). The demographic is as per [Table 1].
Table 1

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Discussion: Adult onset osteomalacia can be overlooked due to lack of specific clinical features. Axial involvement in osteomalacia is commonly confused with spondyloarthropathy due to overlapping clinical features like back pain and radiological features like fuzzy sacroiliac joint outlines, calcified enthesopathies and subchondral bone resorption. Elevated serum FGF23 level and increased phosphate excretion in urine with tubular maximum phosphate reabsorption per glomerular filtration rate (TMP/GFR) reduction help to confirm the diagnosis of hypophosphatemic osteomalacia. Tumours responsible for oncogenic osteomalacia are slow growing benign tumour and removal of tumour leads to immediate and permanent improvement in the serum phosphate level.

Conclusion: Adult onset hypophosphatemic osteomalacia can be a close mimic of rheumatological condition specially spondyloarthritis (Both clinically and radiologically). Serum calcium and phosphorus level measurement should be part of evaluation of vague aches and pains not responding to analgesics. In patients not improving to phosphate correction FGF23 level should be checked. DOTANOC PET- CT should be performed to look for oncogenic osteomalacia. DOTANOC PET - CT is better modality for tumour detection as compared to FDG PET-CT. Resection of tumour helps considerably to alleviate the patient's condition.


  PC0090: Clinical and autoimmune profile of systemic sclerosis patients from central India Top


Rajat Deb, S S Nelson; Netaji Subhash Chandra Bose Medical College and Hospital, Jabalpur, Madhya Pradesh, India

Background: Systemic sclerosis (SSc) is a disorder characterized by fibrosis of skin and visceral organs. Pathogenesis of Systemic sclerosis is complex and is incompletely understood as yet. Autoantibodies in SSc represent serologic hallmarks which have clinical relevance, with diagnostic and prognostic potential.

Objective: To study Clinical features and Frequency of Autoantibodies among Subtypes of Systemic Sclerosis patients from Central India.

Methodology: 33 SSc patients were clinically classified according to the European League Against Rheumatism (EULAR) criteria. Clinical manifestations were recorded at the time of presentation. Autoantibodies were tested in them. These parameters were further correlated with clinical presentation of the disease.

Results: SSc patients had M: F ratio of 1:15 where mean age was 41.9±15.5.Clinical subtypes showed that 20 patients (60%) had diffused cutaneous (dcSSc) lesions, 13 patients (40%) had limited cutaneous (lcSSc) lesion. The overall frequency of ANA in SSc patients studied was 93.9%. The frequency of Anti-Topoisomerase (anti-Scl70) and anti-centromere were 57.6% and 21.2%, respectively. Anti-Scl70 antibodies were significantly high (90% versus 4%) among dcSSc patients (P < 0.0001) whereas anti-centromere antibodies were significantly high (0% versus 53.8%) among lcSSc patients when these two subtypes were compared (P < 0.0001).
Figure 1: Skin sclerosis with finger contracture in a patiny of systemic sglerosis

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Table 1: Autoantibodies in systemic sclerosis patients

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Conclusion: This study supports that there are geoepidemiological variations among scleroderma patients for their clinical presentation, autoantibody profile, and immune parameters across the country.


  PC0091: Efficacy and safety of adalimumab biosimilars in spondyloarthritis Top


P Akshay, G Meghna, R Pratyusha, D Phani Kumar, R Liza; Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Objective: The aim of this study was to investigate the safety and efficacy of adalimumab biosimilars (bADA) in SpA pateints and assess their efficacy.

Methods: Consecutive SpA patients who visited the SpA clinic at our centre between July – August 2019 and received bADA for atleast 3 months were enrolled. Demographic, clinical, radiological and disease activity (BASDAI) variables were collected. Descriptive statistics are reported.

Results: 25 patients were enrolled. Median age of the cohort was 25 years (IQR 19 - 33).

Table lists the disease and laboratory variables

Of the 25 patients, 4 stopped treatment abruptly, 2 due to credit issues and 2 were lost to follow up.

The mean (SD) BASDAI at initiation, 3 & 6 months of biologic was 4.8 (1.73), 1.75 (1.41) and 1.37 (1.78) respectively. 18 patients had BASDAI >4 at baseline while only 2 of them persisted to have BASDAI >4 at 3 & 6 months. 3 patients achieved BASDAI of 0 by 3 months and 10 patients by 6 months.

BASDAI 50 response was seen in 17 (68%) patients. Of the rest (early-non responders), 1 had improvement in BASDAI>2 in the next 3 months while others had a mean improvement in BASDAI of 0.25 over the next 3 months.

Seven patients with latent TB received prophylactic ATT a 1 month before biologic initiation. In 2 patients ATT and biologic were simultaneously started in view of very high BASDAI. 1 patient without LTB developed pulmonary tuberculosis 2 months after stopping biologics. He improved with ATT.

Conclusion: Biosimilars are efficacious in achieving short term disease control in 70% patients. Early non responders did not have significant improvement in disease activity even at 6 months. Prevalence of latent tuberculosis was high in this cohort but the risk of tuberculosis was not increased in short term.
Table 1

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  PC0092: Comparison of ultrasonology findings with clinical parameters and plain radiograph in patients with knee osteoarthritis Top


Shweta Nakarmi, Binit Vaidya, Rakchya Joshi; National Center for Rheumatic Diseases, Kathmandu, Nepal

Background: Knee osteoarthritis (OA) is one of the commonly encountered joint diseases in people with a prevalence ranging upto 7.9 % in adult population. There are various clinical and radiologic instruments to measure the disease severity of knee OA. Kellgren-Lawrence classification (KL) is commonly used radiographic tool to assess the severity of osteoarthritis. Nowadays, high frequency musculoskeletal ultrasonology (MSUS) is being used as a reliable tool for assessment of osteoarthritis.

Objectives: To compare and correlate musculoskeletal ultrasonology findings with clinical and xray findings in patients with knee OA

Methodology: A prospective, cross sectional study was conducted in patients with knee OA attending a tertiary level rheumatology center in Kathmandu, Nepal. Baseline demographic profile of the patients along with clinical parameters like WOMAC and VAS for pain and stiffness were recorded in predesigned data sheet. An AP view standing xray was obtained for involved knee and KL grading done. High frequency MSUS was done for same knee. Synovial hypertrophy, suprapatellar effusion, cartilage thickness (medial, lateral and intercondylar), Doppler activity and osteophytes were noted. Pearson's coefficient of correlation was calculated to assess correlation between ultrasonology and radiologic parameters.

Results: A total of 138 patients with knee OA were enrolled in the study with female predominance of 83% and mean age of 56.87 ± 10.61 years. The mean VAS for pain and WOMAC scores were 4.95 ± 1.78 and 28.36 ± 13.31, respectively. A negative correlation was observed between cartilage thickness (medial) with joint space narrowing (KL grade) and between WOMAC and VAS scores and cartilage thickness.

Conclusions: MSUS may be used in clinical practice to diagnose and monitor cases of knee osteoarthritis. It is a potential imaging technique which might help in therapeutic interventions and disease monitoring too.


  PC0093: Psychometric assessment of Telugu version of lupus-QOL and its correlation with SLE disease activity measures Top


Rajasekhar Liza1, Baisya Ritasman1, Dhundra Bhavani1, Vineeta Shobha2, Asma Chougle2 Avinash Jain3, Amita Aggarwal3. Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana,1St John's Medical College and Hospital, Bengaluru, Karnataka,2Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Introduction: Lupus-QOL, a newly developed questionnaire for quality of life assessment in SLE patients, has 34 items grouped into 8 domains and is validated in many non-Indian languages. Psychometric assessment is not available in Indian languages. The aim of the study was to determine whether in Telugu version, the items were internally consistent, and they correlated with SLE disease activity measures.

Methodology: Patients from the INSPIRE cohort, which included patients with disease duration less than 3 years and seen in the hospital after August 2018. Demographic status, SLEDAI (Mexican-SLEDAI) and BILAG score were recorded simultaneously. Psychometric testing for internal reliability and correlation of different domains with SLEDAI and BILAG were done using Cronbach alpha statistic and Pearson's correlation coefficient respectively using SPSS.

Results: There were 134 patients with mean (SD) age 26.8 (7.7) years. The median SLEDAI (IQR) was 7 (0-27). The mean scores in each domain indicated significant impairment of QoL with intimacy most affected. There was excellent internal consistency among questions in each domain. The musculoskeletal domain of BILAG correlated with most of the LQOL domains.

Conclusion: Lupus QOL questionnaire is a reliable measure of quality of life in our population. Body image and intimate relationship are the most affected QOL measures in SLE patients. BILAG musculoskeletal domain showed modest correlation with Lupus QOL domains.

Keywords: BILAG, Lupus-QOL, mSLEDAI


  PC0094: ASDAS is better than BASDAI for estimating disease activity in patients with axial spondyloarthritis: An experience from tertiary care centre in western India Top


Prashant Chotalia, Dhaiwat Shukla, Rutwiz Mistry, Sapan Pandya, Puja Srivastava; Smt. NHL Municipal Medical College and its Affiliated Hospitals, Ahmedabad, Gujarat, India

Background: Measuring Disease activity in Axial Spondyloarthritis (AxSpA) is difficult. Hence, we planned to compare the two disease activity parameters in patients with Axial Spondyloarthritis.

Objectives:

  1. To describe clinical features of patients of Axial Spondyloarthritis
  2. To correlate disease activity scores BASDAI and ASDAS in these patients


Materials and Methods: Patients fulfilling ASAS criteria for Axial Spondyloarthritis(SpA) were included in the evaluation. Demographic, clinical, radiological and laboratory data of included patients were evaluated. BASDAI score, ASDAS were calculated. Correlation between BASDAI and ASDAS was evaluated.

Results: One hundred and thirty eight patients with median age 28years(IQR: 22-38) and sex ratioM:F of 2.9:1.106(76.81%) were AS and 32(23.18%) were axial SpA not fulfilling modified New York criteria. The demographics are as shown in the table.

Most common extra articular manifestations were uveitis (11.59%) and oral ulcer (9.42%).Sacroiliitis was present on imaging in 68.84 % patients.Mean BASDAI (91/138 patients) was 3.42± 1.75 and Mean ASDAS (82/138 patients) was2.75±1.25.There was no gender difference in disease activity in patients with Axial SpA.

Although, moderate correlation was observed between the two disease activity parameters (0.634), In our study population, 8.53 % had inactive disease, 32.92% had moderate active disease, 29.26 % had high disease and 29.26 % had very high disease activity as per ASDAS. 64.83 % had inactive disease while 35.16 % had active disease as per BASDAI. 87.5 % percentage of patients inactive according to BASDAI was active in ASDAS.

Conclusion: Although BASDAI and ASDAS correlate well for patients with high disease activity, a significant proportion of patients inactive according to BASDAI are still active according to ASDAS. Thus, ASDAS is probably better tool to detect mild and moderate disease activity in Axial SpA.


  PC0095: Assessment of hand dysfunction in systemic sclerosis by Indian version of cochin hand function scale Top


Devender Bairwa, M B Adarsh, K G Chengappa, V S Negi; Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

https://drive.google.com/file/d/1203n8j2VJxRg-KJKr_c0ZWNAFZdvacAA/view?usp=drive_open

Background: Hand dysfunction in Systemic Sclerosis (SSc) is common yet neglected. Cochin Hand Function scale (CHFS) is validated questionnaire consisting of 18 questions regarding day to day activities with a total possible score of 0-90. Considering differences in cultural and ethnical Background, all questions of CHFS may not be applicable to Indian population.

Objective: To assess the hand dysfunction in SSc patients using a modified CHFS (mCHFS) and an Indian version of CHFS (iCHFS) and to correlate it with disease burden.

Methodology: Females fulfilling ACR/EULAR 2013 classification criteria for SSc were enrolled. mCHFS was formulated by removing three questions (questions 7,9 and 10 in CHFS) not suitable in Indian setting (score=0-75) and iCHFS was formulated after replacing the three omitted questions with questions more related to the day to day activities in Indian setting. Clinical parameters like modified Rodnan skin score (MRSS) , finger to palm (FTP) distance, Finger to table (FTT) distance, oral aperture including vertical inter-incisional (VIID) and vertical inter-labial distance (VILD) were recorded. Quality of life was assessed using European quality of life 5 D (Eqol-5D) instrument. Damage was assessed using adjusted Scleroderma Clinical Trial Consortium Damage Index (ad-SCTC-DI).

Results: Thirty females with mean age of 41.33± 7.7 years were enrolled. The mean mCHFS score was 31.67±22.30. Ten patients had mild hand dysfunction (Group1; mCHFS score=0-17) and 20 patients had moderate to severe hand dysfunction (Group 2; mCHFS score= 18-75). Group 2 had statistically more severe skin thickening and reduced oral aperture. iCHFS (n=16) and mCHFS (n=30) showed significant positive correlation with FTP, MRSS, ad-SCTC-DI and EQOL 5D difficulty in usual activity score [Figure 1].
Figure 1: Correlation of clinical parameters with modified CHFS and indial version of CHFS. CHFS – Cochin han function scale; VIID-vertical interincisor distance; VILD-vertical inter-labial distance; FTP (F) –finger to palm distance in flexion; FTT- finger to table distance; MRSS – modified rondnan skin score; MHISS-mouth handicap in systemic sclerosis; E qol 5D vas –European qualiry of life 5 dimension visual analogue health scale; adjusted-SCTC-DI-scleroderma clinical trial consortium damage index

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Conclusion: Hand dysfunction in systemic sclerosis is substantial. Indian version of CHFS correlated well with the disease burden. But this needs to be validated in a larger population with further modifications.


  PC0096: Non-adherence to treatment is an added risk for serious infection in lupus: result from a retrospective cohort analysis Top


Sanket Shah, K G Chengappa, M B Adarsh, Vir Singh Negi; Department of Clinical immunology, JIPMER, Puducherry, India

Background: Infections impact the disease course, therapeutic outcome, prognosis and socio-economic burden in Systemic Lupus Erythematosus (SLE). Data from high infection settings like India is sparse. Identifying the demography and determinants for infection can help us in better care of patients with SLE.

Objectives:

  1. To study the profile of infectious burden and its outcome in SLE.
  2. To identify determinants of serious infections.


Methods: Case records of 530 patients satisfying inclusion criteria were screened for serious infection, requiring hospitalization, surgical intervention or intravenous antibiotics. Demographic information, clinical and serological profile, disease activity, comorbidities, details of treatment including cumulative steroid dose, treatment non-adherence and flares were recorded to assess the determinants of serious infections using analytic statistics.
Figure 1

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Results: Out of 530 patients screened, 167 (31.50%) had 195 episodes of serious infections. Patients with serious infections had higher disease activity, major organ involvement, leucopenia, and higher steroid and immunosuppressant use (Table-1). Non-adherence led to a higher incidence of serious infections (53.8% vs 20.1%, p=0.01), of which 60% occurred within 6 months of drug default. The respiratory tract was the commonest site of infection. Higher numbers of infections were observed in the initial year of the treatment with predominant occurrence in months of May, July and September (Figure 1). Majority of the patients were in the induction phase of treatment and 24 (14.67%) patients died of infection.

Conclusions:

  • Infection burden in SLE is significantly higher in India compared to the cohorts from the western world.
  • Counselling of patients towards compliance and judicious use of immunosuppressant may reduce infection burden.



  PC0097: Prescribing pattern and efficacy of methotrexate in psoriatic arthritis – experience from Karnataka psoriatic arthritis cohort Top


Uma Karjigi, K Chanakya, S Chandrashekhara, K Sharath, H Vikram, V Rao, R Jois, M Daware, B Dharmanand, S Singhai, J Ramya, C Pramod, R Subramanian, A Kamath, D Chetana, Shivaprasad, Srinivas, Vikram R Jain, Harshini, B Pinto, K M Mahendranath, V Shobha Apollo Hospitals, St Johns Medical College Hospital, Chanre Rheumatology and Immunlogy Center, Columbia Asia Hospital, Manipal Hospitals, Vikarm Hospital, Bengaluru, Narayana Health city, Sakra Hospital, Bengaluru, Arthritis Specialty clinic, Hubli, SDM Medical College, Dharwad, JSS medical college, Apollo BGS Hospital, Mysore, Yenepoya Specialty Hospital, Mangalore, Karnataka, India

Introduction: Ideal therapy for psoriatic arthritis (PsA) should ameliorate all aspects of disease process viz both cutaneous and joint disease, peripheral & axial manifestations including dactylitis and enthesitis. Methotrexate is an invaluable anchor in the paradigm for PsA treatment.

Objectives: 1) To describe prescribing pattern of methotrexate and other csDMARDs used for management of PsA among rheumatologists practising in Karnataka, India. 2) To identify the efficacy and safety of methotrexate monotherapy.
Figure 1

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Methods: Treatment information was extracted from KPsAC (n=378) which is an ongoing cross sectional, non-interventional study across 17 rheumatology practicing centres in Karnataka. Standard disease activity outcome measures were used for assessing response to therapy (DAPSA, PASI, HAQ, MDA5).

Results: The prescribing pattern of csDMARDs used either alone or in combination is depicted in Figure 1a. Overall, 270 patients were on Methotrexate either alone or in combination with other csDMARDs, median disease duration being 62 months. Three csDMARD combination was used in only 10 patients, all on Background of methotrexate. TNFi were used in 5% of patients and all were anchored on methotrexate. Outcome measures for Mtx monotherapy(n=159) is shown in [Figure 1]b. No major safety concerns were noted.

Conclusion: Methotrexate remained the most frequently used csDMARD in KPsAC associated with low skin scores and low disability in three fourth of the patients when used as monotherapy.


  PC0098: Clinical and immunological profile of patients with idiopathic inflammatory myopathies in a tertiary care hospital Top


D Sai Kumar,M B Adarsh, K G Chengappa, V S Negi; Jawaharlal Nehru Institute of Post Graduate Medical Education and Research, Puducherry, India

Background: Idiopathic inflammatory myopathies (IIMs) are a group of rare and less understood autoimmune disease. The present study was designed to analyse demographic, clinical and serological characteristics and compare anti-synthetase syndrome with anti MDA-5 positive dermatomyositis (aMDA-5 DM).

Methodology: Out of 151 patients screened 116 with adequate records were included. Anti-cellular antibody status was available for 99 and line immune assay for 65 patients.

Results: Demography and clinical characteristics are shown in table 1 and autoantibody profile. Two patients had breast carcinoma, one each had carcinoma cervix and gastric adenocarcinoma. Anti MDA 5 antibody was more prevalent than anti-JO1 antibody (19.5% vs 15.2%) [Table 1].
Table 1: Demography of the cohort and comparison between antisynthetase syndrome and anti malondialdehyde-5 dermatomyositis positive dermatomyositis

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Conclusions: Anti MDA5 antibody associated IIM is an under-recognized entity in our population. Given its poor prognosis, further characterization in a larger cohort is necessary.


  PC0099: Could apremilast be the first choice DMARD in combination with methotrexate in psoriasis/psoriatic arthritis? - Results: from KPsAC Top


Uma Karjigi, K Chanakya, S Chandrashekhara, K Sharath, H Vikram, V Rao, R Jois, M Daware, B Dharmanand, S Singhai, J Ramya, C Pramod, R Subramanian, A Kamath, D Chetana, Shivaprasad, Srinivas, Vikram R Jain, Harshini, B Pinto, K M Mahendranath, V Shobha Apollo Hospitals, St Johns Medical College Hospital, Chanre Rheumatology and Immunology Center, Columbia Asia Hospital, Manipal Hospitals, Vikram Hospital, Vikram Hospital, Narayana Health city, Sakra Hospital, Sakra Hospital, Aster CMI Hospital, Fortis Hospital, Mahaveer Jain Hospital, Sparsh Hospital, Bengaluru, Arthritis Specialty Clinic, Hubli, SDM Medical College, Dharwad, JSS medical college, Apollo BGS Hospital, Mysore, Yenepoya Specialty Hospital, Mangalore, Karnataka, India

Introduction: Over the last decade, treat-to-target (T2T) strategy has been in the forefront for psoriatic arthritis (PsA). As of now, there is no consensus on use of combination DMARDs in management of PsA.

Objectives: To compare efficacy of combination DMARDS in Karnataka Psoriatic Arthritis Cohort (KPsAC).

Methods: Data collected from 378 PsA patients included in multicentre, cross-sectional, observational study over a period of 6 months using a structured CRF. Efficacy and safety information was extracted for various combinations of csDMARDs. Standard outcome measures were used to compare the efficacy of the treatment groups.

Results: Patients were divided in to three groups based on combination csDMARDs used: Methotrexate(Mtx)+Leflunomide(Lef), Mtx+Sulfasalazine(SSz) and Mtx+Apremilast(Apr). Their characteristics along with outcome measures are depicted in [Table 1]. In Mtx+Apr group: remission or low disease activity was present in 46%, HAQ score of <0.5 was seen in 80%, and no patients had a PASI of > 10. PASI was significantly lower in the Mtx+Apr group compared to Mtx+Lef group (p=0.04). Combination of Mtx with Lef or SSz achieved remission or low disease activity in 37-44% however the HAQ score in both these groups remained high in three fourth patients.
Table 1: Characteristics and comparison of combination conventional, synthetic disease modifying anti rheumatoid drugs

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Conclusions: Despite the differences between the groups and observational design of the study, skin disease seemed to be lesser in patients on Apremilast and MTX combination.


  PC0100: Comparative analysis of antinuclear antibody tests and their clinical significance in an Indian population Top


Samrat Bordoloi, Sharath Kumar1, S Nagaraj2, G C Yatish3, Sangita Sathyamurthy4, M P Kavitha, Jayaram Iyenger; Anand Diagnostic Laboratory,1Optima Arthritis and Rheumatology Clinic,2TRACC Clinic and Optima Arthritis and Rheumatology Clinic,3optima Arthritis and Rheumatology Clinic and Manipal Whitefield,4Columbia Asia Hospital Whitefield, Bengaluru, Karnataka, India

Background: Detection of Antinuclear antibody (ANA) is the hallmark of laboratory investigations in Connective Tissue Disorders (CTD). However, various methodologies used in both screening tests and specific antibody detection has led to a loss of consensus and poor reproducibility of Results.

Objectives:

  1. Compare Enzyme Immunoassay with Indirect Immunofluorescence as a screening test.
  2. Compare 3 different variants of Enzyme Immunoassays used as specific tests.
  3. Propose disease specific laboratory diagnostic algorithm based on clinical diagnosis.
s

Methods: 60 cases were recruited by Rheumatologists between April 2019 to July 2019. Two screening tests by Indirect Immunofluorescence (IFA) and Fluorescence Enzyme Immunoassay (CTD Screen) along with three specific antibodies tests a) Line Immunoassay (LIA) b) Enzyme Linked Immunosorbent Assay (ELISA) and c) Fluorescence enzyme Immunoassay (FEIA) were performed. For statistical analysis, clinically confirmed cases of Systemic Lupus Erythematosus (SLE), Sjogren's Syndrome (SjS), Undifferentiated Connective Tissue Disorder (UCTD), Mixed Connective Tissue Disorder (MCTD) were considered disease positive and Rheumatoid arthritis (and other autoimmune inflammatory disorders), as well as Fibromyalgia (and other musculoskeletal conditions), were considered disease negative.

Results: Sensitivity(78%) of CTD Screen was comparable to IFA(78%) and Specificity(73%), PPV(82%) and NPV(69%) was better than IFA(67%,78%,67%). U1RNP and Ro52 were positive irrespective of the diagnosis. CTD Screen was positive in 2 cases of SjS and 2 cases of UCTD which were negative on IFA. All of these cases were positive for SSA/Ro. CTD Screen was negative in 2 clinically proven cases of SLE with high antibody titer on IFA.

Discussion: In this pilot study CTD Screen appeared comparable to IFA as a screening test with better specificity, PPV and NPV. However, in cases of high antibody titer CTD Screen appeared to can give a false negative result. In the current study, U1RNP has shown non specific positivity.
Figure 1

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  PC0101: Indian patients perspective towards treatment in ankylosing spondylitis Top


Kamini Reddy, Asawari Raut, Pravin Patil1; Department of Cinical Pharmacy, Bharati Vidyapeeth deemed to be University, Pune, Maharashtra1Apex centre of rheumatology, Chennai, Tamil Nadu, India

Objective: Ankylosing Spondylitis (AS) is a chronic disease & requires long-term treatment. The objective was to understand the patient's perspective towards allopathic medications (modern medicine) and beliefs about complementary medications.

Methods: We conducted a cross-sectional study of 100 consecutive AS patients attending a rheumatology clinic over 3 months. A structured questionnaire was used to explore complementary medicines use, reasons for switching and patient's concern with allopathy medications.

Results: and Discussion: Fifty-six percent patients had tried complementary treatments such as ayurvedic/homoeopathy. In 44 of these patients, medications were prescribed by qualified BAMS/BHMS. Ayurvedic treatment was the preferred option (46%), followed by homoeopathy (25%) and 29% had tried both. Out of 56, 34(61%) patients reported switching to allopathy due to lack of efficacy and two patients stopped the medication due to side effects. A common misconception that 'allopathic medications cause side-effects in all AS patients' was observed in 54%. Thirty-two percent patients believed that doctors hide side-effects of medications. Around 60% believed that damage to internal organs like kidneys and liver occur in all AS patients taking allopathy. A quarter of patients thought gastric problems (acidity) would be the most common potential side effect. Only 39% of patients were willing to continue allopathy treatment for AS until remission is observed by the rheumatologist. About 28% believed that a certain type of diet aggravates their pain and 7% believe that adhering to the specific diet helps relieve pain.

Conclusion: We were able to identify various misconceptions about allopathic medications. Many patients are taking treatment from unregulated practitioners of complementary therapy. Patients are reluctant to take long term allopathic treatment due to fear of side effects. We need to find ways to demystify information regarding allopathy medications and address patient's concerns so that their health-seeking behaviour is appropriately corrected.


  PC0102: Ankylosing spondylitis in India- understanding the patient journey and impact on work productivity Top


Kamini Reddy1, Asawari Raut1, Pravin Patil2;

Department of Clinical Pharmacy, Bharati Vidyapeeth deemed to be University, 1Apex Centre of Rheumatology, Pune, Maharashtra, India

Background: Ankylosing spondylitis (AS) is a progressive inflammatory rheumatic disorder which mainly affects the spine. Worldwide there is an unacceptably long delay in diagnosis.

Objective: Objective was to study the patientís journey from symptom onset and assess the impact of this illness on work productivity.

Methodology: We conducted a cross-sectional study of 100 consecutive AS patients attending rheumatology clinic. The study duration was 3 months. A structured questionnaire was used to collect patient reported information during face-to-face interview. Data on patientís journey from symptom onset to diagnosis and impact on work productivity was collected.

Results and Discussion: Average delay in diagnosis was 4.52Ī 4.42 years and 58 patients had visited 3 different specialists before reaching to rheumatologist. First point of contact for most patients (60%) was an Orthopaedic. About 61 patients had received an incorrect diagnosis before reaching rheumatologist. Fifty-two patients were referred to the rheumatologist by doctors, 32 patients relied on google search, and 16 were referred by other patient/relatives. Out of 100, 81 patients reported work productivity being affected due to AS. Nine patients had to quit work. The work productivity is reduced by 50% in 72 patients. On an average, 4.5 days/month were skipped from work in 16% patients. Remaining patients had no option but to carry on with daily work in pain. Extra-Spinal involvement was seen in 43% patients, peripheral arthritis being the commonest manifestation (31%). Prevalence of extra-spinal manifestations was more common in males.

Conclusion: This is the first study from India attempting to measure the real impact of AS on work productivity. It also reveals the long diagnostic delay for AS and lack of patient referral pathway in India. Being the first point of contact in majority cases, the awareness at the level of orthopaedics should be intensified.


  PC0103: Prevalence and factors associated with anxiety and depression in ankylosing spondylitis Top


Kamini Reddy, Asawari Raut, Pravin Patil1; Department of Cinical Pharmacy, Bharati Vidyapeeth deemed to be University, Pune, Maharashtra,2Apex Centre of Rheumatology, Chennai, Tamil Nadu, India

Introduction: Aim was to determine the prevalence of anxiety and depression in Ankylosing Spondylitis (AS) patients and examine factors associated with it.

Methods: We conducted a cross-sectional study of 85 consecutive AS patients (mean age 35.63 years, 81% males) attending the rheumatology clinic. Two questionnaires (validated in English, Hindi and Marathi) PHQ-9 for depression and GAD-7for anxiety were used as a screening tool. Associations between GAD-7 scores and sociodemographic parameters, extra-spinal involvement (peripheral arthritis, eye inflammation, psoriasis and IBD), fear of medication's side effects and work productivity were assessed. We classified patients in 2 groups; 18-35young adult, >35 older adults.

Results: Out of 85, 62%(n=53) patients had anxiety and 73%(n= 62) had depression. Severe anxiety is more common in young adults [31.65±5.72 years] (p<0.05). χ2 (chi-square) test revealed extra spinal manifestations are associated with severe anxiety (75% with severe anxiety vs. 12% no anxiety). Work productivity due to AS is affected by 63 % in severe anxiety and by 22% in patients with no anxiety. Severe anxiety patients reported a high level of difficulty in performing daily activities whereas patients with no anxiety had no difficulty. Severe anxiety was associated with smoking, lack of exercise, shorter disease duration and having misconceptions that AS medications cause side effects in all patients but these findings weren't statistically significant.

Conclusion: To the best of our knowledge, this is the first report from India that describes the impact of AS on mental health. There is a high prevalence of anxiety and depression in AS patients. The risk factors for severe anxiety are young age and extra-spinal manifestations.


  PC0104: A randomised clinical trial of curcuma longa extract for treating symptoms and effusion-synovitis of knee osteoarthritis Top


Benny Antony, Zhiqiang Wang, Tania Winzenberg, Guoqi Cai, Laura Laslett, Dawn Aitken, Ingrid Hopper1, Rob Jones2, Changhai Ding, Jurgen Fripp3, Graeme Jones; Menzies Institute for Medical Research, University of Tasmania,3Department of Radiology, Royal Hobart Hospital, Hobart,1Drugs and Device Registry, Department of Epidemiology, Monash University, Melbourne,3Department of Biomedical Informatics and Image processing, CSIRO Health and Biosecurity, The University of Queensland, Brisbane, Australia

Background: Pharmacological therapies are limited, associated with off-target effects, and only modestly effective for pain in osteoarthritis (OA).

Objectives: The CurKOA trial aimed to compare the efficacy of Curcuma longa extract versus identical placebo for treating knee pain and effusion-synovitis on MRI.

Methods: In this double-blind trial, we randomised 70 participants with significant knee pain (>40 mm on VAS), knee OA (ACR criteria) and presence of a moderate amount of ultrasound-defined effusion/synovitis (>4 mm thickness in the suprapatellar region) on ultrasound to receive Curcuma longa extract (80% aqueous-based extract standardized to turmerosaccharides + 20% curcuminoids, 2x500 mg capsules/day) (n=36) or identical placebo (n=34) for 12 weeks.

Results: Of the 112 participants screened, 70 participants (age 61.8±8.6 years, 56% female) were randomised, and 68 (97%) completed the study.

There was a reduction in VAS knee pain in the treatment (-23.75[-29.78,-17.73]) and placebo (-14.64[-20.80,-8.47]) group, with a significant between-group difference of -9.11mm[-17.79,-0.44] [Figure 1]a equivalent to a standard effect size of 0.49. There was a no significant between-group difference (1.25 mL[-1.21,3.72]) in the MRI assessed effusion-synovitis volume.
Figure 1

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Significant changes favouring the treatment group were seen in WOMAC knee pain (between-group difference: -47.22[-81.22,-13.22]) [Figure 1b], WOMAC function (-112.26[-222.79,-1.74]) and OARSI-OMERACT treatment responders (63% treatment and 38% placebo [Risk Ratio=1.64(1.00,2.70)]. No significant change in T2 relaxation time of the femoral cartilage (-0.38 ms[-1.10,0.34]) was seen. The number of adverse events was similar in the treatment (n=18) and placebo (n=29) groups.

Conclusions: Curcuma longa extract significantly improved knee pain in an inflammatory phenotype of knee OA patients over 12 weeks compared to placebo. There was a moderate standard effect size of the treatment which appears greater than other conventional pharmacological therapies. In this short term study, Curcuma longa extract had no effect on knee structural measures assessed using MRI.


  PC0105: Regeneration of degenerative Joints with Regenerative injection therapies Top


Shashipal Sadana; Ortho Surgeon, Agra, Uttar Pradesh, India

Introduction: Management of degenerative joint disorders has always been a tricky and tedious job for Orthopaedicians. Most of the time degeneration of ligaments, tendons capsules, and other supporting soft tissues around joint leads to the altered biomechanics of joint and then eventual cartilage degeneration. It has become a clinical necessity to develop the novel therapeutic approaches to accelerate the regeneration of these tissues.

Objectives: The study aims to investigate the outcomes of 178 patients with unresolved chronic pain in different regions namely: Knee joint, Lower back, Hip joint, Shoulder joint, and Ankle joint.
Figure 1

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Methods: All of these patients underwent one or a combination of the following regenerative therapies: Chemical Regenerative Therapy, Platelet Rich Plasma Therapy (PRP), and Bone Marrow Aspirate Concentrate (BMAC). On average, 26 months following their last RIT (Regenerative Injection Therapy) session, patients were contacted and asked numerous questions. The questions included, but were not limited to, levels of pain, physical and psychological symptoms, and activities of daily living- before and after their last RIT.

Results: The Results: of this study showed that patients had a statistically significant decline in their levels of pain, stiffness, crunching sensation. It also showed improvements in their range of motion, with 82% showing improvements in walking ability, medication usage, anxiety, depression, and overall disability. Apart from that, 74% patients showed radiological improvements.

Conclusion: In this study, patients with unresolved degenerative joints, treated with RIT, showed reasonably good improvements in many clinically relevant parameters and overall quality of life. Degenerative disorders of joints are not merely referred to cartilage but surrounding soft tissues are also important. Regenerating stabilizing tissue of joints should always be considered in management.


  PC0106: Role of on-demand adalimumab in spondyloarthritis – can less be safe and effective? Top


Avik Medda, Rudra Prosad Goswami1, Mehebubar Rahman, Parasar Ghosh1, Rama Prosad Goswami; Department of Tropical Medicine, STM,1Department of Rheumatology, IPGME and R, Kolkata, West Bengal, India

Background: Spondyloarthritis (SpA) is a chronic inflammatory arthritis involving axial skeleton and peripheral joints. Non-steroidal anti-inflammatory drugs and disease modifying agents are moderately useful and pose significant toxicity on long-term use. TNFα inhibitors used in patients with active disease provide fast and often dramatic relief. However continuous use of TNFα inhibitors may inculcate into serious health hazards like infections, especially tuberculosis.

Objectives: To assess the effectiveness, adverse effects of on-demand adalimumab therapy in spondyloarthritis and consequent change in quality of life of the patients.

Methods: In this prospective observational study, we included patients with SpA, fulfilling New York criteria, 1984 with active disease (Bath AS disease activity score ≥ 4) and treated them with on-demand subcutaneous adalimumab (40mg) for 6 months. Outcome measures were attainment of AS disease activity index (ASDAS) low disease activity (<1.3) and ASDAS improvement; change in physical and mental component summaries (PCS and MCS) in SF-12.

Results: We included 30 patients with SpA (median age 36 years, interquartile range (IQR): 29-42; 60% males (18/30)). Baseline extra-articular disease activities were present in 22/30(73.33%). Baseline ASDAS was low in 3.3% (1/30), high 76.7% (23/30) and very high in 20% (6/30). Patients received median 5 injections (IQR 5-6). At the end of follow up after 6 months of treatment, inactive disease state was achieved in 13.3% (4/30), clinically significant improvement occurred in 50% (15/30) and major improvement occurred in 20% (6/30). PCS improved from 25.1±3.34 to 41.56±6.48 (p<0.0001) and MCS improved from 35.12±7.59 to 51.92±4.59 (p<0.0001). There is significant reduction in ESR (pre-treatment mean 60.07±20.7 and post-treatment mean 25.33±12.23, p<0.00001) and CRP (pre-treatment mean 23.78±14.65 and post-treatment mean 8.2±6.41, p<0.00001). There is only one (3.33%) incidence of tuberculosis. No incidence of non-infective adverse reactions.

Conclusions: On-demand adalimumab therapy is safe and effective in the treatment of spondyloarthritis.


  PC0107: Study of intestinal microbiota in juvenile idiopathic arthritis: A cross sectional observational study Top


Malika Yadav, Deonath Mahto, Anu Maheshwari, Ravinder Kaur, Rama Chaudhry, Kamal Kumar Singhal; Lady Hardinge Medical College, New Delhi, India

Background: Juvenile Idiopathic Arthritis (JIA) is the most common chronic rheumatic disease of childhood and there is emerging data that supports the presence of dysbiosis in the pathogenesis of this disease. However, there are limited studies and data on this concept, especially in India.

Objectives: To analyse the composition of fecal microbiome of children with JIA compared to healthy children and to see the correlation of fecal microbiota with disease activity.

Methods: Stool specimens from 30 patients with JIA aged 1-18 years and 60 healthy controls in the same age group were studied. None had any gastrointestinal symptom or history of antibiotic use in preceding 3 months. Relevant clinical data including lab investigations and disease activity status of patients was recorded. Stool specimens were analysed using different culture media and biochemical Methods: for aerobic and anaerobic organisms.

Results: In culture, among anaerobic organisms, growth of Bacteroides (p<0.01) and Lactobacillus (p 0.02) was found to be statistically significant and among aerobic organisms, growth of Klebsiella (p 0.01) was found to be statistically significant as compared to healthy children.

Clostridium perfringens was found in 2 (66.6%) patients of JIA with moderate disease activity and 2 (7.4%) patients with high disease activity. The difference between the two groups was statistically significant (p 0.03). No significant differences were found between growth of other bacteria with disease activity.

Conclusions: Our Results lend support to the hypothesis of gut dysbiosis in JIA patients, in terms of richness and compositional deviation from healthy subjects. However, no significant differences were instead found between active and inactive disease. This indicates that the gut microbiota profile is specific to the individuals with JIA, rather than disease activity status.


  PC0108: Study of cardiovascular risk profile in rheumatoid arthritis Top


Sumanth Madan, Rama Bhat, Rajagopal1, Akhya Sharma, Aditi; Departments of Internal Medicine and1Radiology, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India

Background: The problem of increased cardiovascular disease (CVD) in patients with rheumatoid arthritis (RA) is well established.

Scoring systems in RA are an objective interpretation of said risk. The 2 scoring systems included were Extended Risk Score for Rheumatoid Arthritis (ERS- RA) and Atherosclerotic Cardiovascular disease (ASCVD) scores.

CIMT is a non-invasive method used to evaluate for subclinical atherosclerosis, opening opportunity for early intervention and diagnosis of CVD.

Objectives:

  • To compare cardiovascular risk in rheumatoid arthritis using CV risk scores and correlating with disease activity
  • To study CIMT and relation with CV risk scores
  • To assess coexisting cardiovascular disease in rheumatoid arthritis.


Methods: All patients >40 years, satisfying EULAR guidelines were included. They underwent a Carotid ultrasound for CIMT. ASCVD and ERS RA, disease activity was calculated. The scoring systems were compared with CIMT to assess their performance.

Statistically, sample size was determined using kappa. Mean, median, standard deviation and Chi-squared tests were applied, p-value< 0.05 was considered significant.

Results: In this study, 106 patients were included with female predominance (82%), with maximum distribution in the age group 40-50 years. 62.3% were CIMT positive. There was no association between RF, DAS28, CDAI and CIMT, but RF had 80.3% sensitivity for a positive CIMT.

ASCVD had a strong positive predictive value for positive CIMT (p <0.001). Arbitrary cut off of 6.5 had superior sensitivity and specificity. Similarly, ERS-RA had a high sensitivity with a high negative predictive value (p<0.01). A cut off of 7.1 performed better than a cut off of 7.5. Longer duration of RA, translated to a positive CIMT. Comparison of ROC curve showed equal discriminatory ability for ERSRA and ASCVD.

Conclusions: RA was associated with a higher risk of cardiovascular events. ERS- RA performed as good as ASCVD as a CV risk score.


  PC0109: An observational study of ILD in various rheumatic diseases in tertiary care center Top


M Sri Anusha, T N Tamilselvam, R Ramesh, S Mythili; Department of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: ILD is one of the pulmonary complication of connective tissue disorders. Common rheumatic disease to cause ILD are SSc, MCTD, Sjogren's syndrome, RA and rarely SLE.

Objectives: In our study we focused on prevalence and clinical profile of ILD among various autoimmune rheumatic diseases.

Methods: This observational study was conducted at Rajiv Gandhi Government Hospital over 4 months between April – July 2019. Patients who are diagnosed as ILD with various autoimmune rheumatic diseases were included in this study.

Results: Total number of patients included in our study was 57 patients, of whom females were 53 (93%). In our study the most common rheumatic disease associated with ILD was Rheumatoid Arthritis - 25 (43.8 %) followed by Systemic Sclerosis - 13 (23%). Others conditions to cause ILD in descending order were SSc/Sjogren syndrome, Sjogren's syndrome, MCTD,UCTD, SSc/SLE overlap, SLE, Rhupus and unclassified vasculitis. Based on imaging (HRCT lungs) study NSIP (58%) was found to be the most common type of ILD, followed by UIP. Other rare types were LIP and BOOP. 30% were found to be asymptomatic(detected on imaging). Among the symptomatic patients, most common presentation was dyspnea followed by dry cough. Among 11 patients who were found to have PHT on cardiac evaluation, majority had systemic sclerosis. On spirometric evaluation, 90% showed restrictive pattern and 10% - combined pattern.

Conclusion: Some patients with autoimmune rheumatic diseases with ILD on evaluation remain asymptomatic in early stages of disease. Among few patients who had irreversible features, pulmonary hypertension played an important contribution.


  PC0110: Is the prevalence of SLE increasing? Top


Ram Raj Singh, Kimngan P Nguyen, Eric Y Yen, Ning Li; UCLA, Los Angeles, CA, USA

SLE prevalence in Indians has varied from 3 (95% CI, 0-7) and 20 (10-30) to 193 (141-258) per 100,000 in population surveys of 91,888 in northern-India in the late-1980s, of 56,541 across India during 2004-2010, and in a cohort of 23,305 Indians living in UK during 1999-2012, respectively (Malaviya, Singh… Kumar. Lupus 1993; Chopra, Ind J Rheumatol 2015; Rees, etal, Ann Rheum Dis 2016). Such wide variations in the prevalence of SLE have been reported from around the world, which is likely due to variabilities in study populations, case definitions, and case ascertainment sources. Most studies have been based on small numbers of prevalent cases in relatively smaller populations. Few studies have attempted to estimate SLE prevalence in large populations, which is an important unmet need.

We used national survey databases based on a multistage probability design to estimate the number of SLE patients across the entire US population. We calculated the cumulative percent change in prevalence (Chi-square test), modeled trends, and calculated the annual percent change using Joinpoint regression analysis.

The overall prevalence rate of SLE during 2001-2011 is 86.4/100,000 persons (95% CI, 69.6-103.3). Annual SLE prevalence increased 6% (95% CI, 2.5-9.5) each year from 2001 through 2011, with a cumulative increase of 59.4% during this period (72.2 in 2001 to 115.1 in 2011, p<0.0001). The period prevalence of SLE during 2001-2011 is 9-fold higher in females (137.5 [95% CI, 109.2-165.9]) than males (15.2 [95% CI, 8.0-22.4]).

The increasing trend in SLE prevalence in US during 2001-2011 may reflect an increased recognition of SLE, decreasing mortality, or actual increases in the prevalence. Such population-based studies are needed to estimate the demand-supply gap of physicians needed to care for lupus that requires intensive management and frequent follow-up to prevent its high premature mortality (Yen…Singh, Ann Intern Med 2017).


  PC0111: Monitoring the weekly progress of rheumatoid arthritis through 3 simple questions Top


S Chandrashekara, Bharath Raj, K R Anupama; Chanre Rheumatology and Immunology Centre and Research, Bengaluru, Karnataka, India

Introduction: DAS score and patients-derived measures are performed once in 3 months and they usually capture only cross-sectional measures. Moreover, the findings are not indicative of the entire time interval. The present study was to derive a tool to measure the progress of RA on weekly basis.

Materials and Methods: The prospective longitudinal study obtained answers for a self-reported questionnaire comprising of six questions every week for 14 weeks from a cohort of RA patients. The questions assessed pain on a scale of 0-10, qualitative response to exercise, feeling (improvement in clinical condition) and stiffness, pain and swelling in 16 joint zones/regions and self-care activities compared to preceding week. The demographic and clinical data were collected at baseline and assessed at 3rd and 6th months. DAS28CRP(3) was calculated and correlation of the demographic, baseline clinical characteristics and follow-up DAS28CRP(3) with questionnaire was verified. Agreement of response to questionnaire with relative change in DAS28CRP(3) was assessed by linear weighted kappa method.

Results: 25 RA patients with mean age 50.8±11.24 years answered the questionnaire. Male to female ratio was 1:11.5. The respondents' median (range) of RA duration was 45 (10-169) months, baseline DAS28CRP(3) 2.3 (1.19-5.08) and the follow-up DAS28CRP(3) 2.31 (1.5-4.71). Response to pain scale, feeling of improvement in clinical condition, pain and swelling in 16 joint zones/regions correlated with clinical measures used in routine RA practice. Feeling of clinical improvement, stiffness, pain in 16 zones/regions and self-care activities had fair agreement with changes as measured by DAS28CRP(3).

Conclusion: The use of the 3 parameters namely stiffness, pain in 16 zones/regions and self-care activities may assist in measuring the clinical, structural and functional aspects of RA disease process on weekly basis. Pain scale could be an additional measure. Validation of the questionnaire in large sample size with a simple application is ongoing.


  PC0112: A prospective study for determining the aetiology of hypokalemic paralysis in patients admitted in Government General Hospital, Guntur Top


Krishna Sagar Gajula, Narmada Nangadda, Mani Kishore Maddirala, Namratha Gogineni; Department of General Medicine, Guntur Medical College, Guntur, Andhra Pradesh, India

Aims and Objectives: To determine the underlying cause for hypokalemia in patients presenting with hypokalemic paralysis to Government General Hospital, Guntur. This helps determine the common underlying disorders which lead to hypokalemic paralysis in patients with such presentation.

Materials and Methods: A total of 20 patients who presented to GGH, Guntur with hypokalemic paralysis were evaluated. Blood and urine samples were collected immediately at the time of presentation. Treatment was initiated immediately with potassium supplements. We simultaneously evaluated for the underlying condition with the help of clinical history, examination and appropriate laboratory investigations which may have predisposed to hypokalemia.

Observations and Results: Of the total of 20 cases Distal Renal Tubular Acidosis (dRTA) was found to be the cause for hypokalemia in 13 (65%) cases, Hypokalemic Periodic Paralysis in 2 (10%) cases, Bartter's syndrome in 2 (10%) cases, Thyrotoxicosis leading to hypokalemia in 2 (10%) cases and Insulin excess in 1 (5%) case.

We further found out that Sjogren's Syndrome was the most common underlying cause for distal RTA. A total of 8 (40% of total cases; 61.5% of dRTA cases) cases were diagnosed as probable Sjogren's Syndrome.

Conclusions: In Conclusion, we found that the most common cause for hypokalemic paralysis in our patients is probable Sjogren's Syndrome (40% of cases). Therefore, extensive workup must be done in every case of hypokalemic paralysis to recognize the underlying condition to direct more specific treatment and prevent further complications.


  PC0113: Predicting response to methotrexate in children with juvenile idiopathic arthritis: Role of biomarkers Top


Narendra Kumar Bagri, Subhradip Karmakar1, Partha Haldar2, Rakesh Lodha, S K Kabra; Departments of Pediatrics and1Biochemistry, All India Institute of Medical Sciences,2Centre for Community Medicine, All India Institute of Medical Sciences (AIIMS), New Delhi, India

Background: Methotrexate is the initial choice among the disease-modifying anti-rheumatic drugs (DMARD) for juvenile idiopathic arthritis(JIA). However, nearly 40% of the subjects do not respond to conventional DMARDs like methotrexate and warrants additional therapy. Owing to inability to predict the response to methotrexate upfront; the decision to augment therapy is executed only after a failed trial of methotrexate. This approach exposes some children to ineffective drugs with accompanying side-effects, ongoing inflammation and joint damage. Thus, there is a need of predictive biomarkers and inflammatory cytokines and serum MRP 8/14 may be may potential candidates.

Objective: The aim of our study was to correlate the baseline values of inflammatory cytokines (ILs-2, 6, 12 and TNFα) and serum MRP 8/14 with response to methotrexate at 3 months follow-up in children with JIA.

Methods: We enrolled consecutive children with JIA and withdrew blood samples prior to initiation of methotrexate for biomarker estimation. The response to methotrexate was assessed using the definition of improvement (DOI) as per ACR. Children achieving ACR 50 were classified as responders, while non-responders were defined as those who failed to achieve ACR 30 at follow up. The data were analyzed using STATA 13.

Results: Out of the 69 children (36 boys) with JIA, 48 (69.5%) children met the responder criteria while 18 (26%) were classified as Non-responders. The baseline values [median (IQR)] of serum MRP 8/14, were significantly higher in responders as compared to non-responders [144.34 (88.54, 188.34) ng/ml vs 95.34 (76.54, 130.28) ng/ml; p = 0.047]. The subjects with higher baseline levels of serum MRP 8/14 had a higher odds of achieving ACR 50 response criteria [OR=1.01;95% CI (1.00-1.02)].

Conclusion: The baseline serum levels of MRP8/14 are significantly raised in the children achieving ACR50 and thus it may have prognostic value in predicting response to methotrexate.


  PC0114: Study of prevalence and pattern of haematological profile in patients of rheumatoid arthritis Top


Ganesh Koppad, Silas Nelson, Yashwant Bandole; Netaji Subhash Chandra Bose Govermenet Medical College, Jabalpur, Madhya Pradesh, India

Background: Among all the extra-articular manifestations of rheumatoid arthritis, haematological abnormalities are most characteristic particularly anemia.

Objectives: To study prevalence and pattern of haematological profile in patients of rheumatoid arthritis.

Methods: It is a case-control study involving 50 patients fulfilling inclusion criteria and satisfying the ACR criteria 2010 of Rheumatoid arthritis and 50 age & sex matched controls. DAS 28 score was used for establishing severity. Complete blood count and anaemia profile was done for both cases and controls.

Results: Sex ratio of females:males in this study is 4.55:1. Maximum cases were in the age group of 41-50 years (32%). The mean DAS 28 score for all 50 cases were 4.69±1.03 and p value <0.0001 which was highly significant compared to the control group. Mean Hb in cases was 10.6g/dl and in controls was 12.95 p<0.0001. Mean MCV in cases 78.62 fl in control group was 88.94 fL p<0.0001. Mean MCH in cases was 25.92 pg and in controls was 29.49 pg p<0.0001. Mean value of MCHC in case group was 30.96% and in controls was 33.73% p<0.0001. The prevalence of anemia in RA was 74% most common cause being anaemia of chronic disease (48.60%) followed by iron deficiency anemia (40.50%) and dimorphic anemia (10.80%). The mean DAS 28 score was higher in cases of anemia of chronic disease (5.64) compared to IDA (4.8). Mean TLC of case group was 10308.44±5363.41 while mean TLC of control group was 7650.44±1640.57 with a p value of <0.0001. Mean platelet count in cases was 2.94±0.13 and in controls was 2.61±0.56 p=0.05.

Conclusion: In patients with RA,serious haematological abnormalities were revealed.Therefore,patients with RA require regular haematological monitoring and if necessary,timely and adequate correction of any violations.


  PC0115: Anti-Jo-1 antisynthetase Syndrome often presents with only arthritis: A single center experience Top


Rajiv Ranjan Kumar, Saket Jha, Aadhaar Dhooria, GSRSNK Naidu, Susheel Kumar, Shefali Khanna Sharma, Aman Sharma, Sanjay Jain, Varun Dhir; PGIMER, Chandigarh, India

Background: Antisynthetase syndrome is characterized by a triad of myositis, arthritis and interstitial lung disease. Anti-Jo-1 is the most common associated autoantibody. This study planned to look at the presentation of the anti-Jo-1 antisynthetase syndrome, in a single Indian center.

Materials and Methods: This was a retrospective single-center study that included patients with anti-Jo-1 antisynthetase syndrome over 10 years.

Results: This study included 27 patients with the anti-Jo-1 antisynthetase syndrome; with a mean age of 40±9.2 years and female preponderance (F: M=4:1). At presentation, the characteristic triad was present in only 4 patients. A majority presented with the incomplete form; with two clinical features (of the triad) in 11 and a single feature (of the triad) being present in 12 patients at initial presentation. Seven presented only with polyarthritis –out of which six had been earlier diagnosed as rheumatoid arthritis. The time gap from the diagnosis of 'rheumatoid arthritis' to antisynthetase syndrome ranged from 3 to 20 years. In patients who had only arthritis in the beginning, there was a significantly longer delay to diagnosis of the antisynthetase syndrome, higher frequency of rheumatoid factor and lower frequency of anti-Ro-52. Overall, the outcome was good, with ECOG class 1 or 2 in most except two patients.

Conclusions: Anti-Jo-1 antisynthetase syndrome commonly presented as incomplete (not a triad); and often only with arthritis. These patients were diagnosed and treated as rheumatoid arthritis for many years, before a diagnosis of the antisynthetase syndrome. Being aware of this presentation may help in earlier diagnosis by actively searching for subtle clues.


  PC0116: Rheumatology clinic patients denies to adhere; what we know and do not know Top


Vivek Kattel, Sourabh Malviya; Medanta Superspeciality Hospital, Indore, Madhya Pradesh, India

Background: Autoimmune disease is alarming in developing world reason being high prevalence and inadequate point of care compared to the burden. Early diagnosis and adherence to therapy is key to success against rheumatologic disorders which still remains a current challenges in Indian subcontinent.

Objective: To explore the reason of poor adherence among common rheumatology patients.

Methods: It was a cohort study carried out for 52 weeks among 250 patients of common rheumatological disorders (Rheumatoid Arthritis (RA), axial spondyloarthropathy (axSpA), Primary Sjogrens' Syndrome (PSS) and Sytemic Lupus Erythematosis (SLE)) who presented first time in our clinic. A base line data about treatment history was obtained and the patient was followed up further.

Results: Only 5% (n=12) were adherent to treatment before they visited our clinic. The most common reason of non-adherence as coded by the 95% patients (n=238) were doctor's associated factor (not counselling about disease(84%), inadequate time give for consultation(73%), inadequate physical examination(67%), frequent visit(61%)) followed by drug associated factors (prolong treatment course(96%), side effects(41%), expensive then alternative medicine drugs(37%), inferior to drugs given by alternative medicine in symptom relief(34%)) and patient related factors (belief that allopathic is symptomatic treatment only(68%), financial constrain(21%), inadequate family support(18%), inaccessibility(15%)). Counsel by doctors focused on etiology(74%), disease severity if not treated(96%) and prolong treatment courses(31%). Only 36% doctors mentioned they perform musculoskeletal examination. Adherence was 62% for first two visit followed by 54% over 12 weeks and 37% over 24 weeks. Yearly adherence was maximum with SLE(24%) followed by PSS(17%), RA(14%) and axSpA(12%).

Conclusion: Structured, graded and customized counselling regarding the persistent nature of disease that demands lifelong disease modifying drugs with ample of time possibly can increase the adherence among autoimmune patients.


  PC0117: Widening demand-supply gap in rheumatic diseases: We must act now! Top


Ram Raj Singh1,2, Shivani Garg1,2;1The University of California, Los Angeles, California,2The University of Wisconsin, Madison, Wisconsin, USA

Rheumatic or musculoskeletal conditions comprise over 150 diseases, which are leading causes of morbidity and disability, giving rise to loss of work and income, and enormous healthcare expenditures. Based on a few reports on prevalence of rheumatic diseases and the projected 2020 population of India, we calculated the total number of patients with various rheumatic conditions, total annual visits, and the number of specialists needed to provide rheumatic diseases care (Table). According to these estimates, 26,770 physicians are needed to provide rheumatic disease care, but currently there are <1,000 rheumatologists in India. As a comparison, there are over 6,000 rheumatologists in the United States where >21% of adults (46.4 million) were found to have self-reported doctor-diagnosed arthritis in 2003-2005. This workforce supply of 1.7 rheumatologists per 100,000 rheumatic diseases patients was deemed short by 13% to meet the demand. It is projected that the number of persons with doctor-diagnosed arthritis will increase by ~40% to ~67 million by 2030 in the United States, leading to the demand-supply gap of 102%. A multi-pronged approach must be urgently adopted to address the rheumatology workforce shortfall. These approaches may involve broad-based awareness campaigns to showcase the need as well as the remarkable advances in the management of these diseases over the last two decades. There is also an urgent need to undertake curriculum redesign in undergraduate and postgraduate medical education, in partnership with medical college principals and deans. The use of innovative classroom models in place of traditional hierarchical classroom model will facilitate education and help in attracting and recruiting trainees to rheumatology. Lobbying for resources at the state and national level, efforts by rheumatology societies to increase funding and trainee positions, telemedicine, increasing nurse practitioner and physician assistant training, and outreach clinics will all help in narrowing the rheumatic disease demand-supply gap.


  PC0118: Quality of life in patients with juvenile idiopathic arthritis Top


Debabrata Sarkar, Tapas Kumar Sabui; Department of Pediatrics, R. G. Kar Medical College and Hospital, Kolkata, West Bengal, India

Introduction: Quality of life (QoL) assessment is particularly important in any chronic disease not only because of its chronicity but also for its effects on mental and social health. Most often these domains of health are ignored. Our Objectives were to assess quality of life in patients with different subtypes of JIA and to compare the quality of life between active phase and remission phase of the disease.

Methods: Within the age group of 5-16 years 26 patients with different subtypes of JIA were included. Juvenile arthritis Disease activity score (JADAS 27 score) used for disease activity assessment and Juvenile Arthritis Quality of Life Questionnaire (JAQQ) used for QoL assessment. Both scorings were done at first visit and after 6 months of treatment. Scores of first visit and follow up visit were compared. More JAQQ score indicates poor QoL.

Results: By this observational longitudinal study we found all 26 patients had high disease activity at first visit. There were no cases of psoriatic and unclassified arthritis. Mean JADAS-27 score and JAQQ score at first visit were 26.5538 ± 10.20714 and 4.51 ± 0.69 respectively. Physical domains were more affected than psychosocial domain. Polyarticular JIA had worst QoL than other subtypes. 65% patients achieved remission. With treatment significant improvement in QoL seen in remission phase in every subtypes of JIA (mean follow up JAQQ score: 2.00±0.748).

Conclusion: Poor QoL seen in patients with JIA irrespective of subtypes. Polyarticular JIA had worst QoL than others. Although only 65% patients achieved remission, with treatment significant improvement in QoL found in all major subtypes of JIA irrespective of their final disease activity. Major improvements occurred in physical domains compare to psychosocial domain. Interventions focusing physical and psychosocial rehabilitation may change the outcome further.


  PC0120: A study on clinical profiles of patients with spondyloarthritis at a tertiary care hospital in North India Top


Vijay Karthik Bhogaraju, Venkatesh Srinivasa Pai; Department of Internal Medicine, AIIMS, Rishikesh, Uttarakhand, India

Background: Since 1984 the diagnosis of Ankylosing spondylitis (AS) has been based upon the modified New York (mNY) criteria with mandatory presence of radiographic sacroiliitis, without which the diagnosis is not tenable. However, it may take years or decades for radiographic sacroiliitis to develop delaying the diagnosis for long periods.Assessment of SpondyloArthritis International Society (ASAS) classification criteria published in 2009 was a significant step towards this goal. ASAS criteria described an early stage of the disease where sacroiliitis was demonstrable only on MRI but not on standard radiograph.In this present scenario of newer approach to disease with ASAS criteria and limited studies done in the Indian setting on clinical profiling of patients with SpA, clinical profiling would help us understand the various entities of SpA

Aim: To study and assess the clinical profiles of patients with Spondyloarthritis.

Objectives: (1) To study clinical profiles as per clinical and laboratory entities in patients of spondyloarthritis with subgroup analysis of radiographic and non-radiographic spondyloarthritis.

(2) To study disease severity in patients of spondyloarthritis with subgroup analysis of radiographic and non-radiographic spondyloarthritis.

Methods: This is a prospective, non-interventional, observational, cross-sectional study at A.I.I.M.S, Rishikesh from The first of Jan 2018 – 30th of June 2019.

Recruitment of Patients: Patients diagnosed as SpA (ASAS 2009) by treating physician were recruited from the OPDs/ IPDs of General Medicine,Orthopedics.

Results: Out of 110 cases in our study,56 cases were analysed.The mean age of presentation in both the groups of Nr-ax spA and AS were 24.67 and 26.37 respectively. The mean BASDAI, ASDAS scores weresimilar in both the groups. (4.35 vs 4.56 (p-0.047) respectively. The mean BASMI scores were higher in AS group.

Conclusion: These both groups are likely to have similar clinical activity, however larges studies in Indian setting are required.


  PC0121: Biological usage in spondyloarthritis: A single centre observation Top


Nagaprabu V N, Velammal P1, Gayathir Anand; Sakthi Rheumatology Centre Pvt., Ltd.,1PSGIMS & R, Coimbatore, Tamil Nadu, India

Introduction: Spondyloarthritis(SPA) is a group of disease with limited therapeutic options unlike rheumatoid arthritis. The usage of Biologicals have increased considerably to achieve disease control.

Materials and Methods: We analyzed the outcome of patients who received Biologicals in our centre during June 2014 - July 2019. Patients with less than 6 months post biological follow-up were excluded. The patients had received Biologicals as per the disease activity assessment and not as per the schedule.

Observation: There were a total of 71 patients who were included for the analysis of which there were 52 males and 19 females. The mean age of the study population was 37.43 ± 13.01 years. In the study population there were 11 psoriatic arthritis and 60 had Spondyloarthritis. In psoriatic arthritis patients secukinimab and Adalimumab was given in 3 each and infliximab 200mg was given in 4 and etanercept in one patient. 3 out of 11 patients achieved a drug free remission during this period. And NSAIDS were withdrawn in 9 out of 11 patients. In the remaining Spondyloarthritis group 32 patients received infliximab and etanercept in 6 patients and Adalimumab in 22 patient. A repeat dosing of biological after the first dose was used in only 19 patients. NSAIDS were withdrawn from 44 patients out of the 60 patients.

Conclusion: A single dose of Biologicals achieves good disease control in 73% of patients with SPA.


  PC0122: Biological as a steroid sparing agent in juvenile idiopathic arthritis Top


Nagaprabu V N, Velammal P1, Gayathir Anand; Sakthi Rheumatology Centre Pvt., Ltd.,1PSGIMS & R, Coimbatore, Tamil Nadu, India

Introduction: The management of juvenile idiopathic arthritis(JIA) is quite challenging and the usage of steroids and Nonsteroidal anti-inflammatory drugs (NSAIDS) in pediatric age group has its own limitations. Biologicals have changed the outcome of patients with JIA.

Methods: WE have analyzed the outcome of patients with JIA who had received Biologicals in the period of June 2014 to July 2019 and assessed the need for steroids and the frequency of dosing of Biologicals.

Observations: There were a total of 16 patients who had received Biologicals during this period out of which there were 5 boys and 11 girls. The mean age of the study population was 13 ± 6.23 years. There were 8 patients with enthesitis related arthritis and 4 had systemic onset JIA (SOJIA) and 3 had polyarticular JIA (RF+ve) and 1 had oligoarticular JIA (ANA+ve). Adalimumab was used in 11, Tocilizumab was use in 4 and Etanercept was used in 1. A second and repeated dosing of biological was decided based on the disease activity and was required in only 3. Eight out of these 16 patients achieved total drug free remission in these group and 11 patients were totally weaned off from steroids .NSAIDS.

Conclusion: Biologicals when used appropriately in JIA achieved good drug free remission in 50% of patients and also helped wean of steroids/NSAIDS in more than 60% of JIAs.


  PC0123: The need of regular biological usage in rheumatoid arthritis: A clinical obervation Top


V N Nagaprabu, P Velammal1, Gayathir Anand; Sakthi Rheumatology Centre Pvt., Ltd.,1PSGIMS & R, Coimbatore, Tamil Nadu, India

Introduction: The management of Rheumatoid arthritis(RA) have changed considerably in this biological era but regular and repeated dosing of Biologicals is often not practically feasible. Methods: We analyzed the case records of patients with RA who have received Biologicals in our centre during the period June 2014 to July 2019 and analyzed the need for regular dosing. Observation: There were a total of 171 patients who were included in this analysis of which there were 139 females and 32 males. The mean age was 49.88 ± 13.61 years. 114 patients had received rituximab 500mg 0& 15 days and only 4 patients during this period needed a repeat dosing. Of which 76 patients were managed without steroids. A low dose of rituximab 100mg weekly for 4 weeks was used in 19 patients but in this only 2 patients we were able to start steroids and one received repeat biological dosing. 8 patients received infliximab and 2 patients were continued on steroids with no one requiring repeat dosing. 3 patients were treated with single dose of etanercept and in 2 we were able to stop steroids.

Conclusion: A repeat dosing of biological were required in only 12.86% of patients ( 22/171) and in 60% (104/171) we were able to stop steroids.


  PC0124: Performance of American College of Rheumatology, Systemic Lupus International Collaborating Clinics and European League against Rheumatism-American College of Rheumatology weighted classification criteria in childhood lupus Top


Sabarinath M, Tamilselvam T N, Balakrishnan N, Karthikeyan, Sujatha N, Ramesh R, Mythili S; Department of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Background: The three SLE classification criteria, namely American College of Rheumatology (ACR) 1997, Systemic Lupus International Collaborating Clinics (SLICC) 2012 and the new European League Against Rheumatism (EULAR)-ACR 2019 weighted criteria are primarily validated in adults.

Objective: The primary objective of our study is to compare the sensitivity of these criteria in childhood SLE (cSLE). The secondary objective is to compare them with the original validation studies done in adults.

Methods: We conducted a retrospective medical record review study of children with clinical diagnosis of SLE admitted at our tertiary care centre between January 2016 and July 2019. We excluded children with SLE overlap. All three classification criteria were applied to these patients. All three criteria sets were compared against a gold standard of physician diagnosis.

Results: There were 66 patients (84% female) who were diagnosed as cSLE during the study period. The median age at the onset of illness was 15 years. The median duration from the onset of initial symptom to the time taken for diagnosis was 6 months. Sensitivity of ACR criteria and SLICC criteria in childhood lupus was 84% (95% CI 73% to 92%) (n=56) and 95% (95% CI 86% to 98%) (n=63) respectively. The sensitivity of new ACR EULAR weighted criteria was 94% (95% CI 84% to 98%) (n=62). 3 cases didn't satisfy EULAR-ACR weighted criteria as they didn't meet entry criteria (ANA positivity), even though all had the required positive score of >=10.

Conclusion: This study shows that the EULAR-ACR weighted criteria and SLICC has similar sensitivity in cSLE. Both of them have higher sensitivity compared to ACR criteria. The sensitivity of weighted criteria may increase further if entry criteria is not considered. The sensitivities of these criteria in children are comparable with those observed in original validation study done in adults.


  PC0125: Changes in urinary micro-albumin levels after correction of hyperuricemia in patients with gout: An observational study Top


Shweta Nakarmi, Binit Vaidya, Rakchya Joshi; National Center for Rheumatic Diseases, Kathmandu, Nepal

Background: Gout is commonly associated with metabolic syndrome. Strong association between serum uric acid level and microalbuminuria has also been observed in previous studies.

Aim: To observe change in urinary micro albumin after lowering of serum uric acid level in patients with gout.

Methodology: A prospective, observational study was conducted at a tertiary level rheumatic center in Kathmandu, Nepal. Adults diagnosed with gout were enrolled in the study after obtaining informed consent. Sociodemographic profile along with clinical history were recorded at baseline. Serum uric acid levels, spot urinary micro-albumin (MAU) excretion, blood sugar, lipid profile, blood pressure were measured at baseline, 3 months and 6 months follow up. Paired t test was used to compare MAU of the participants.

Results: A total of 778 patients diagnosed with gout were enrolled in this study among whom 97.7% were male with the mean age of 48.8 ± 12.4 years. 36 patients (4.6%) had multiple tophi and 9 patients (0.9%) had polyarticular presentation. Comorbidities like diabetes mellitus, hypertension and dyslipidemia were present in 7.2%, 24.4%, and 47.2% respectively. Most of the patients were overweight and obese with the mean BMI of 27.2 ± 7.6. Around 15.5% patients had microalbuminuria (MAU > 30.0 μg/mg) during presentation. Mean MAU level among those with microalbuminuria was 132.4 ± 124.6 μg/mg. Among 114 patients with high MAU, 35 with concomitant HTN were put on ARBs (telmisartan or losartan). In patients with ARBs, MAU reduced significantly after 3 months of treatment with ARBs. Reduction in MAU in those without ARBs was seen after 6 months follow up and the change was statistically significant.

Conclusions: There is significant reduction in MAU after lowering of uric acid levels in patients with gout.


  PC0126: Relation between cytokines, juvenile idiopathic arthritis and mature of biologics Top


Pragati Datta, Tapas Kumar Sabui; Department of Paediatrics, R.G. Kar Medical College and Hospital, Kolkata, West Bengal, India

Background: Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease affecting children worldwide. Inflammatory cytokines like TNFα, IL-6 playa significant role in the pathogenesis of different subtypes of JIA. Few studies are available on this subject.

Objective: To find out if any relation exists between these cytokines and disease activity in JIA and to find if there is any correlation between nature of cytokines and biologics being used.

Methods: This was a prospective observational cohort study carried out in a tertiary care hospital in Kolkata with 17 JIA cases and 10 healthy controls. Disease activity was measured using JADAS 27 score. Cytokines level was measured at baseline and at 6th month follow-up. Choice of treatment was not based on cytokine profile, rather it was based on availability of drugs and physician's choice.

Results: Levels of TNFα was significantly high in patients with high disease activity at baseline compared to follow up (p<0.001) and controls (p<0.0001) except in Oligoarthritis where TNFα level did not change with disease activity (p=0.4). IL-6 did not show any correlation with disease activity (p=0.07) though mean IL-6 decreased at follow-up. Polyarthritis patients were found to have high levels of TNFα, IL-6 compared to other subtypes. More than 50% patients received biologics(Etanercept, Tocilizumab etc) but levels of TNFα, IL-6 did not reduce significantly in them (p=0.16 and p=0.17 respectively) although disease activity improved in most of these patients.

Conclusion: TNFα correlated with disease activity in polyarthritis and systemic onset JIA, IL-6 did not show any correlation with disease activity. Although most of the patients receiving biologics improved, no correlation was found between nature of cytokine and biologics.


  PC0127: Design of a simple SMS/call based temperature monitoring system for freezers Top


Sonali Waghmare1, Jayakanthan Kabeerdoss2, Anne Tryphosa K3, John Mathew2, Syrpailyne Wankhar3; 1IIT Madras, CMC Vellore, and SCTIMST Trivandrum, 2Departments of Rheumatology and Bioengineering, Christian Medical College, Vellore, Tamil Nadu, India

Background: Large number of bio-specimens like blood, sera, plasma, tissue DNA and RNA, as well as proteins, are stored in freezers under controlled conditions preventing loss of biological nature. However, these specimens can still be damaged because of temperature fluctuations resulting from power failure, compressor failure or any other internal defect. The available freezers can display temperatures and provide alarm signals when alteration in temperature within the lab vicinity. However, such monitors are not useful at night or when no one is in close proximity.

Aim: To design a simple and cost-effective SMS/Call based temperature monitoring system that can remotely send alert signals when faulty conditions occur.

Methods: The device consists of a controlling unit, temperature sensor (LM35/PT100) and GSM module. When the system detects an increase in temperature of +10°C from the reference (-20°C or -80°C) freezer temperature, an alert signals is displayed on the LCD screen and simultaneously SMS and call alerts are sent to the lab in-charge. Data was collected manually every half an hour by monitoring the temperature on the LCD display and that of the respective freezers. Such a system provides a window if a fault occurs, such that necessary actions can be taken before samples incur any damage. The cost of components used in the device is less than Rs.2000.

Conclusion: We developed a cost-effective device which is capable of alert user when the drop in temperature of the freezer by calling and by sending SMS. This device has potential application in preserving valuable biological samples stored in freezers.


  PC0128: Use of biological DMARDs in rheumatology patients in Dehradun, Uttarakhand Top


Kamal Bhatt; None

Background: Use of biological disease modifying agents in rheumatological diseases is rapidly expanding. But their prohibitive cost and fear of adverse effects still preclude their wider use in needy patients. Here is a brief audit of biological use from a rheumatology clinic in Dehradun, Uttarakhand.

Methods: Patient records from January 2015 to December 2018 were analyzed and all patients in whom biological DMARDs were used were included. All patients were assessed clinically, with measures of pain, joint counts, metrology, functional limitation, inflammatory markers, and requirement for NSAID and/ or steroid, but no formal disease activity scores were calculated. Because of small number of patients, no statistical analysis was done.

Results: Biological therapy was used in a total of 60 patients during this four year period, with Spondyloarthritis being the most common indication, and Infliximab the most common drug. The average duration of follow up post bDMARD was 13.7 months with range from 3 to 42 months. Forty-four patients (73 %) had good clinical response. Because of theirs high cost, bDMARDs could only be given either for shorter duration or at long intervals. No major adverse effects including tuberculosis were reported.

Conclusion: Majority of patients had good clinical response without any major adverse effects. bDMARD use is limited by their high cost.


  PC0129: Study of clinical profile, etiology and short- term outcome of interstitial lung disease Top


Shrikant A Mandge, Iravati Waghamre, Deepak Malgutte, Aasna Khan, N T Awad, Yojana Gokhle; Department of Medicine, Rheumatology Services, Lokmanya Tilak Municipal Medical College and Government Hospital, Mumbai, Maharashtra, India

Background: ILD may present as component of CTD or independently. Clinical profile and serological test helps diagnosis of CTD-ILD. ILD is treated with steroids and immunosuppressant. Outcome differs on HRCT pattern (NSIP/UIP), primary condition (Non-CTD) or CTD-ILD, baseline FVC of patient. The literature reports CTD-ILD has better prognosis than Non-CTD ILDs.

Objectives: To study, identify proportion of CTD-ILD and their outcome against Non-CTD-ILD in Indian population.

Methods: It's prospective study. Patients of ILD diagnosed on HRCT/PFT included. Clinical features, baseline 6 minute walk test (6MWT) and PFT (FVC); serology like ANA, RA/ Anti-CCP, Anti-Joe/SCL70 (Suspected Scleroderma), U1RMP (MCTD)done.Patients treated with oral steroids/cyclophosphamide/MMF/perfinidone depending on affordability/fertility issues. Patients classified according to HRCT patterns (NSIP/UIP/MIXED/FIBROTIC-NSIP) and serology/clinical profile (CTDs/Non-CTDs). After 6 months outcome measured with 6MWT and PFTs. Increase in FVC more than 10% and 6MWT distance more than 54m considered significant.

Results: Out of 41 patients enrolled, 3 died, 6 lost follow-up. Average age was 47 years (24-72), females (78%). Average duration of illness was 40 months (6-216). Out of 41cases,26(63%) were CTD- ILDs(Scleroderma 9,MCTD 9,Sjogren 2,Dermatomyositis-Polymyositis 4,RA 1,SLE 1) and 15(37%) non-CTDs. Serology positive in 80%.

HRCT patterns observed NSIP (41.5%), UIP (41.5%), MIXED (9.8%) and FIBROTIC-NSIP (7%).At follow-up, data for FVC and 6MWT was available for 32 patients. Mean difference (baseline minus after 6 months treatment) in FVC significant NSIP>UIP (p=0.0064) and CTDs >NON-CTDs (p=0.004). Data in table-5 showed significant increase in distance covered on 6MWT (p=0.0439).The mean difference in 6MWT significantly greater in NSIP>UIP (P=0.OO59) (unpaired T test values).

Conclusion: Two-thirds of ILD patients were CTD related and had better prognosis.


  PC0130: Comparative effectiveness in pain and HAQ-DI improvement for baricitinib versus adalimumab, tocilizumab, and tofacitinib monotherapies in csDMARD-naive rheumatoid arthritis patients: A matching-adjusted indirect comparison Top


Anurag Agarwal, Bruno Fautrel1, Baojin Zhu2, Francesco de Leonardis2, Carol Gaich2, Claudia Nicolay2, Zbigniew Kadziola2, Inmaculada De La Torre2, Peter C Taylor3, Mart van de Laar4, Paul Emery5, Roy Fleischmann6; Eli Lilly and Company, Gurgaon, Haryana, India,1University Pierre et Marie Curie, Paris, France,2Eli Lilly and Company, Indianapolis, Indiana, United States,3Botnar Research Centre, University of Oxford, Headington, United Kingdom,4Arthritis Center Twente, Enschede, Netherlands,5Leeds MSK Biomed/Chapel Allerton Hospital, Leeds, United Kingdom,6University of Texas Southwester Medical Center, Dallas, United States

Background: In RA-BEGIN (NCT01711359), baricitinib (BARI) monotherapy demonstrated superior pain reduction and HAQ-DI improvement than MTX.

Objective: To assess pain and HAQ-DI for BARI monotherapy vs adalimumab (ADA), tocilizumab (TCZ), and tofacitinib (TOFA) monotherapy in csDMARD/bDMARDnaive RA patients using MAIC.

Methods: RA-BEGIN BARI 4mgarm patient data were weighted to match baseline characteristics of ADA (PREMIER), TOFA 5mg (ORAL-START), and TCZ 8mg/kg (AMBITION and FUNCTION) arms; MTX arms were also matched. Method of moments determined weights for age, gender, baseline disease scores and baseline outcome variable values. Mean Week24 changes on pain VAS and HAQ-DI (BARI) were adjusted for the characteristics with the weighted linear model and indirectly compared with respective Week-24 TCZ and TOFA and Week26 ADA data. Statistical significance of weighted treatment effect was assessed by bootstrap method. Sensitivity analyses included MAIC with studylevel matching, Bucher's method without matching adjustment, and inclusion of disease duration as additional matching variable.

Results: The mean baseline pain VAS (58.7 to 65.2; 6-month mean change in pain −28.3 to −33.5 [MTX arm]) indicated comparability between trials, with similar HAQ-DI and changes in HAQ-DI for the MTX arm. BARI showed greater improvement (Week 24) over MTX in pain than TCZ, ADA, and TOFA; statistically significant pain improvement was observed for BARI vs ADA and TCZ with all 3 matching Methods:, but only with the Bucher method for TOFA. BARI-treated patients showed significantly greater HAQ-DI Week24 improvement than TCZ and ADA, but not TOFA [Figure 1].
Figure 1: N† is the sum of both active arm and MTX arm ESS†-Effecmtive sample size (pain/HAQ-DI) after re-weighting of total N=369 (BARI, n = 159, MTX, n = 210) *P ≤ 0.05; **P ≤ 0.01; *** P ≤ 0.001

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Conclusions: Indirect comparison of studies in cs/bDMARD-naive RA patients, after adjusting for differences in baseline characteristics, suggests greater pain reduction and improved physical function for BARI monotherapy vs TCZ and ADA. There is greater pain reduction for BARI monotherapy vs TOFA, but no differences in improved physical function.

Reference

  1. Presented at EULAR 2019. Ann Rheum Dis 2019;78 Suppl 2. [doi: 10.1136/annrheumdis-2019-eular.691].



  PC0131: Effects of baricitinib on haematological laboratory parameters in patients with rheumatoid arthritis Top


Anurag Agarwal, Thomas W Huizinga1, Jonathan Kay2, Masayoshi Harigai3, Edward Keystone4, Josef Smolen5, José Rosas6, Paul Emery7, Stephen Hall8, Filip van den Bosch9, Morton Scheinberg10, Jean Dudler11, Ran Liao12, Gabriella Meszaros13, Jane Barry14, Joel Kremer15; Eli Lilly and Company, Gurgaon, Haryana, India,1Leiden University Medical Center, Leiden, Netherlands,2Division of Rheumatology, UMass Memorial Medical Center and University of Massachusetts Medical School, Worcester, Massachusetts,3Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan,4The Rebecca MacDonald Centre For Arthritis, Mount Sinai Hospital, Toronto, Canada, USA,5Medical University of Vienna, Vienna, Austria;6Marina Baixa Hospital, Alicante, Spain,7Leeds MSK Biomed/Chapel Allerton Hospital, Leeds, England, UK,8Cabrini Medical Centre, Malvern, Australia,9University Hospital, Ghent, Belgium,10Albert Einstein Hospital, São Paulo, Brazil,11Hôpital Cantonal, Fribourg, Villars-sur-Glâne, Switzerland;12Eli Lilly and Company, Indianapolis, Indiana, USA;13Eli Lilly and Company, Vienna, Austria,14Eli Lilly and Company, Basingstoke, UK,15Albany Medical College, Albany, New York, USA

Baricitinib (BARI), an oral Janus kinase (JAK)1/2 inhibitor, is used for treating adults with moderate-to-severe rheumatoid arthritis (RA).

Objective: To summarise changes in absolute neutrophil counts (ANC), absolute lymphocyte counts (ALC), platelet counts, and haemoglobin (Hb), and associated adverse events, with BARI treatment.

Methods: Data were pooled from completed Phase 1/2/3 studies and an extension study.

Results: BARI treatment was associated with a decrease in ANC and an increase in ALC and platelets, which returned to baseline with prolonged treatment or treatment discontinuation. Incidence of neutropaenia (<1000 cells/mm3) was rare (<1%) and was not associated with higher risk of overall or serious infections. Lymphopaenia was associated with slightly higher rate of overall infections (Table).More BARI 4-mg (2.3%) compared to placebo-treated (1.3%) patients had platelet count ≥600×109/L. In a 6-study placebo-controlled set (0-24 weeks), 5 BARI 4-mg-treated patients (vs 0 placebo-treated) had “deep vein thrombosis” (DVT) and/or “pulmonary embolism” (PE). Incidence rate of overall and serious DVT/PE in ALL BARI-RA set remained low at 0.5 and 0.3 per 100 patient-years, respectively.

With long-term BARI treatment, Hb levels decreased transiently before returning to levels slightly higher than baseline at Week 52. Incidence of severe treatment-emergent (TE) shifts in Hb (grade <3 to grade ≥3: <8 and ≥6.5 g/dL) was low across all treatment groups (<0.5%).

Conclusions: No associations were observed between ANC decrease and infections or thrombocytosis and DVT/PE. BARI treatment was not associated with an increased incidence of erythropaenia-related events or anaemia compared to placebo. Few patients interrupted/discontinued BARI due to TE laboratory abnormalities.

Previously presented at BSR (2018).
Table 1: Infection by worst neutropaenia and lymphopaenia CTCAE Grade in 6-Study placebocontrolled* period up to week 24

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  PC0132: A network meta-analysis to evaluate the efficacy of baricitinib and other treatments of rheumatoid arthritis in patients who are inadequate responders to methotrexate Top


Anurag Agarwal, J S Smolen1, P Emery2, J Dudler3, C Zerbini4, W Fakhouri5, C Nicolay6, I de la Torre7, G Burmester8; Eli Lilly and Company, Gurgaon, Haryana, India,1Medical University of Vienna, Vienna, Austria,2Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, England, UK,3HFR Fribourg Hopital Cantonal, Fribourg, Villars-sur-Glâne, Switzerland,4Centro Paulista de Investiga Sao Paulo Clinica, Sao Paulo, Brazil,5Eli Lilly and Company, Windlesham, Surrey, UK,6Eli Lilly and Company, Lilly Deutschland GmbH, Bad Homburg, Germany,7Eli Lilly and Company, Indianapolis, Indiana, USA,8Department of Rheumatology and Clinical Immunology, Charité - University Medicine Berlin, Berlin, Germany

Background: Baricitinib (BARI) is used for treating adults with moderate-to-severe rheumatoid arthritis (RA).

Objective: To assess the comparative effectiveness of BARI 4-mg and other targeted synthetic/biologic diseasemodifying anti-rheumatic drugs in RA patients with inadequate response to methotrexate (MTX-IR).

Methods: A systematic literature review (SLR) of randomized controlled trials (RCTs; Phase 3) of interventions was conducted (1999 to 2017) in Medline, Medline In-Process, Embase, Biosciences Information Service, the Cochrane Library, and trial registers. Network meta-analyses (NMAs) of RCTs reporting American College of Rheumatology (ACR) response data were conducted using Bayesian mixed-treatment comparisons. We present main Results: for the 24(±4)week timepoint (fixedeffects simultaneous models).

Results: 24 trials met the SLR inclusion criteria. Analyses, using BARI RA-BEAM trial data, showed BARI 4-mg (Background MTX) is more effective than adalimumab (ADA) 40-mg (EOW; odds ratio [OR] 1.33; 95%Credible Interval [CrI] 1.01-1.75), abatacept (ABA) 10-mg (IV/4 weeks; OR 1.47; 95%CrI 1.02-2.13), and infliximab 3-mg (IV/8 weeks; OR 1.61; 95%CrI 1.12-2.27), for ACR20. No differences were found on ACR50; BARI 4-mg (Background MTX) was found to be more effective than ADA 40-mg (OR 1.39; 95%CrI 1.02-1.89), ABA 10-mg (OR 1.85; 95%CrI 1.09-3.23), rituximab (RTX) 1000-mg (OR 2.38; 95%CrI 1.10-5.00) and 2000-mg (OR 2.44; 95%CrI 1.04-5.56) for ACR70. Bari 4-mg (Background MTX) showed better Results: than etanercept monotherapy (50 mg/week or 25 mg/biweekly; OR 2.27; 95%CrI 1.04-5.26) for ACR20 and RTX 1000-mg monotherapy for ACR20/ACR70 (OR 1.82; 95%CrI 1.023.13)/(OR 2.70; 95%CrI 1.04-7.14), respectively. Sensitivity analysis including 10 additional trials with up to 20% patients with prior biologic use allowed comparison versus tofacitinib (TOFA), showing BARI 4mg (Background MTX) is more effective than TOFA 5-mg (BID) monotherapy for ACR20 (OR 1.92; 95%CrI 1.32-2.86).

Conclusion: The analyses support BARI as an efficacious treatment option for moderate-to-severe RA patients with MTX-IR.

Previously presented at ISPOR, Barcelona (2018).


  PC0133: A study of clinico-serological parameters, management and outcome of scleroderma interstitial lung disease Top


Irawati Waghmare, Shrikant Mandge, Lalana Kalekar, Dnyaneshwar Halnor, Ruchita Dhurat, Yojana Gokhale; Department of Medicine, Rheumatology Services, Lokmanya Tilak Municipal Medical College and General Hospital, Mumbai, Maharashtra, India

Background: Scleroderma-ILD is leading cause of mortality and morbidity.It is reported in 90% patients(HRCT) and 40-75%(PFT).Reported literature regarding outcome of scleroderma-ILD with treatment with mycophenolate and cyclophosphamide is significant (p<0.01),without statistical difference in outcome between the two.

Objectives: To compare outcome with cyclophosphamide and mycophenolate in scleroderma-ILD.

Methods: It is a prospective study.Patients with scleroderma(ACR criteria 2013)with ILD(HRCT/PFT proven)were included.Baseline HRCT,PFT,DLCO,six-minute walk test and 2D-echocardiography were performed.Selection of treatment was as per patient's affordability for mycophenolate.

Comparison was made after six months of monthly pulses of cyclophosphamide at dose of 600mg/m2 versus MMF daily 1.5-2g(as tolerated).More than 10% improvement in FVC,15% in DLCO,and an increase in 54m distance on 6MWT was considered significant outcome.

Results: Total 46 patients with scleroderma-ILD were studied.80.4% were females.Study population had an average age of 39 years (range:15-61 years) and average duration of illness of 49 months (range:3-144 months).As per HRCT,NSIP=54.3%,UIP=41.3% and 4.3% had normal finding(only low FVC).

35 patients were in cyclophosphamide group and 11 in MMF group.4 patients were lost to follow up in each group.

Baseline mean FVC was 56.2%,DLCO 42.53% and 6MW distance was 311.42 m.The average increase in FVC was 9.45% with treatment. Summarises the outcome in FVC, DLCO and 6MWT in both treatment groups.

Conclusion: Significant improvement was seen in FVC in 12 patients (p=0.035),DLCO in 4 patients (p=0.004) and 6MW distance in 2 patients(p=<0.01).

There was no significant statistical difference between MMF and cyclophosphamide groups with regard to outcome(p=0.593).


  PC0134: Demographic and clinical presentation of rheumatoid arthritis Top


Agarwal N, Dubey S, Malaviya A N, Sharma S, Nagpal PS*; aIndian Spinal Injuries Centre, New Delhi,bAmity Institute of Virology and Immunology, Amity University, Noida, Uttar Pradesh, India

Introduction: Rheumatoid Arthritis (RA) is the most common inflammatory arthritis seen in clinical practice.Prevalence of RA is estimated as 0.5% to 1% worldwide and in Indian if found to be 1%. The purpose of this study was to investigate the gender ratio, the age of onset, family history, smoking status and the main clinical symptoms.

Materials and Methods: 100 Patient with RA classifiable according to ACR/ELUAR criteria, were recruited for the study. Data were collected at each visit of the patient to the rheumatology out-patients clinic of this tertiary health care facility in New Delhi, India.

Results: Out of the 100 patients, 84% were female and 16% were male with a female to male ratio of 5.25:1. The average age of the patients was was 48.02±10.63 years. The peak age of onset of the disease was between 30-50 years. Depending upon the disease activity score-28 (DAS-28) at the first presentation to the rheumatology clinic, the patients could be classified into 3 groups: namely: High Disease activity (32%), Moderate disease activity (49%) or, Low-disease activity/Remission DSA (19%). DAS28 was found to be in positive correlation with Body-Mass Index (r=0.347, p=0.000). 83% patients were found to be sero-positive and 17% were sero-negative for the auto-antibodies.

38% patients had a positive family history, of whom 84% had first degree relative(s) with the disease. Out of the 84 female patients, 45% experienced miscarriage during the first trimester of their pregnancy. Additionally, 4.7% females had death of their newborns within three days of their birth.

Conclusion: The study found a significant correlation between BMI and DAS28. Females with RA may require a close monitoring during pregnancy and peripartum period.


  PC0135: Clinical and serological characteristics of anti-nucleosome antibody in systemic lupus erythematosus Top


S Rajesh; Kerala Institute of medical Sciences, Thiruvananthapuram, Kerala, India

Background: Anti nucleosome antibody though not routinely checked is a well established antibody with correlation with clinical activity and with other serological markers.

Aim: Retrospective study from a cohort of 400 SLE patients, to assess the clinical and serological correlation of antinucleosome antibody in those patients in whom it was detected.

Methods: Patients who had antinucleosome antibody positive were collected from electronic medical record and the clinical and serological characteristics was assessed. Anti ds DNA was done by ELISA, Antinuclesome antibody was detected by Euroimmune ANA profile immunoblot assay (definite and strong positive patterns), complement assay was done by nephelometry. SLE disease activity was evaluated by using SLE-Disease Activity Index (SLEDAI) score.

Results: 52 patients who were positive for antinucleosome antibody were assessed. Predominant system that was involved was mucocutaneous followed by musculoskeletal system. All patients studied were in an active stage of disease and were untreated, of which 5 patients had renal biopsy-proven kidney involvement, which was categorized as lupus nephritis (LN) and rest did not show any renal manifestations (SLE without LN). Hematological involvement was in the form of pancytopenia and secondary antiphospholipid syndrome was present in one third of cases. Neurological involvement and vasculitis was a minority presentation in this cohort. All but 4 off these patient were anti ds DNA positive showing a significant correlation and there was a significant inverse correlation with complement c3 levels.

Conclusion: Anti-nucleosomal antibody detection in our cohort showed predominant extrarenal manifestations and was useful as an additional disease activity marker to other laboratory tests. There was significant correlation with Anti ds DNA positivity and low c3 levels.


  PC0136: Use of photo- protection in patients with systemic lupus erythematosus: Awareness, attitude and behaviour: A study by rheumatology nurse counsellors Top


Baghel SS1, Thakran R2, Messi C2, Yadav V2, Kapoor S 3, Garg SR3, Vivekanand4, Malaviya A N5; 1 Senior Rheumatology Nurse Counselor, 2 Rheumatology Nurse, 3Consultant Rheumatologist, 4Allied Health Professionals, 5Head of the Department of Rheumatology, Indian Spinal Injuries Centre Superspeciality Hospital, New Delhi, India

Background: Photosensitivity is a common manifestation of SLE, affecting an estimated 57-73% of patients. Photoprovocation tests have shown that ultraviolet irradiation (UVA and UVB) induces cutaneous lesions in these patients. Modern sunscreens that block UV rays are effective in reducing disease flares. Some reports have also suggested that Sun exposure can lead to organ damage e.g. lupus kidney disease.

Objectives: To assess the awareness and compliance of sunscreen use amongst patients with SLE and to determine whether photoprotection advice was provided at diagnosis and follow visits.

Methods: All SLE patients attending the rheumatology clinic, willing to participate in the survey, were enrolled in this study. All the patients were counselled about to avoidance of excessive Sun exposure with continuous photoprotection through physical measures such as protective clothings and daily application of broad-spectrum sunscreens. At every follow-up visit, the patients were repeatedly counselled about avoiding Sun exposure and the use of sunscreen. All the relevant information was collected in a pre-designed form.

Results: 78% of patients had photosensitivity and 96 %were aware that exposure to sunlight was related to skin exacerbation.72% of the patients had at least annual exposure to tropical sunlight. However, only 54% of patients reported using sunscreen consistently and 26% used sunscreen occasionally.45% used sunscreen with 50 SPF, 18% used 30 SPF and 17% were not aware of what SPF they were using. 20% did not use sunscreen even in when the Sun exposure was expected.

Conclusion: Although there was excellent awareness about photoprotection amongst patients with SLE.Only 54 % using them regularly and only 45% using SPF >50 the study showed that all the patients would benefit from education and counseling about appropriate photoprotective behavior, in particular, the need for consistent use of high factor sunscreen preparations together with the use of other protective measures.


  PC0137: Safety of baricitinib in patients with moderately to severely active rheumatoid arthritis: A subanalysis of the Indian population from phase 2/3 clinical studies Top


Jyotsna Oak, Syamasis Bandyopadhyay1, Sundeep Upadhyaya2, Anurag Agarwal3, Rohit Arora3, Subhashini Arthanari4; Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra,1Apollo Gleneagles Hospital, Kolkata, West Bengal,2Indraprastha Apollo Hospital, Delhi,3Eli Lilly and Company, Gurgaon, Haryana, India,4Eli Lilly and Company, Singapore

Background: Baricitinib, an oral selective inhibitor of Janus kinase 1/2, is approved in more than 50 countries for the treatment of moderately to severely active rheumatoid arthritis (RA) in adults.

Objective: To provide a comprehensive integrated summary of safety of baricitinib in Indian patients with RA who participated in global Phase 2 and 3 studies.

Methods: Integrated safety analyses for the Indian population were reported using pooled data from three completed studies (Phase 2, JADA; Phase 3, JADZ and JADX) and one ongoing long-term extension study (Phase 3, JADY). Patients were randomised to receive baricitinib 2-mg, baricitinib 4-mg, or placebo. The percentage of patients experiencing any treatment-emergent adverse events (TEAEs), serious adverse events (SAEs), and temporary or permanent baricitinib discontinuation were reported. Exposure-adjusted incidence rates (EAIRs) per 100 patient-years were calculated.

Results: Of 1469 patients, 131 (9%) patients were enrolled from India. No deaths were reported among patients from India (India: 0%; overall: 0.1%). Four patients in the Indian population reported AEs that led to permanent discontinuation of the drug. The EAIRs for TEAEs were anemia (India: 3.1; overall: 2.2) and herpes zoster (India: 2.1; overall: 2.8). The most common SAEs in both populations were infection and infestation (India: 2.3%; overall: 3.8%). One followup case of bone tuberculosis was reported. No cases of pulmonary embolism or deep vein thrombosis were reported in the Indian population [Table 1].
Table 1: Safety parameters in patients with moderately to severely active rheumatoid arthritis (Indian and overall population)

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Conclusion: Baricitinib was well tolerated by Indian patients with moderately to severely active RA, and the Results: were consistent with the overall population included in the randomised controlled studies.




  PC138: Clinical and serologic profile of patients diagnosed as idiopathic inflammatory myositis Top


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

Introduction: Idiopathic Inflammatory myositis are a heterogeneous group of systemic autoimmune rheumatic disorders, with 2 peaks of occurrence. Auto-antibodies are commonly detected in upto 80% of the cases.

Methods: A retrospective chart analysis was undertaken for 19 patients admitted to the Rheumatology Department between 01 August 2016 – 31 July 2019, who were diagnosed as Idiopathic Inflammatory Myositis.

Results: There were 12 females and 7 males. Juvenile dermatomyositis was diagnosed in 1 male only. The mean age at diagnosis was 34 years. Mechanic's hand was seen in 3. Typical rash of dermatomyositis was seen in 12. Calcinosis cutis was seen in 1. ANA positivity was reported in only 4, but their ANA profile was negative. 4 patients had Ro52 positivity on ANA profile, while 1 patient had RNP/Sm positivity. 8 patients were Mi2 positive, while 6 were Jo1 positive, PL 7 positive = 2, PL 12 positive, OJ positive and Pm/Scl 75 positive was noted in 1 each. The lowest mean MMT-8 score was seen in Mi2 Positive individuals (score: 67) Patient with Pm/SCl 75 positivity did not have any muscle weakness. ILD was seen in 8/19 patients. Cardiac involvement was noted in 3 patients. MRI of the muscle was done in 5 patients, which had shown evidence of Patchy hyperintensities in the proximal group of muscles. 2 patients had normal EMG, while the remaining 17 patients had pattern suggestive of myopathy.

Discussion: Patients with IIM are diagnosed on a constellation of clinical and laboratory parameters. Patients are afflicted at a mean age of 35. MRI shows patchy hyperintensities in the proximal muscle groups of the patients. EMG shows myopathic pattern in these patients. Many patients are unwilling for a muscle biopsy.

Conclusion: Indian patients of IIM are afflicted a decade early. Mi2 positivity is more common than Jo 1 positivity.


  PC0139: Short term and durable remission in rheumatoid arthritis: Differences and determinants Top


Sanchaita Misra, Sumantro Mondal, Satarupa Dutta1, Dipanjan Bhattacharjee, Sulagna Chatterjee, Ayindrila Saha, Sudipta Chatterjee, Pradyot Sinhamahapatra, Debasish Lahiri, Alakendu Ghosh; Department of Rheumatology, SSKM Hospital, Kolkata, West Bengal,1National Institute of Animal Biotechnology, Hyderabad, Telangana, India

Introduction: Remission is the ultimate treatment goal in the management of patients with rheumatoid arthritis (RA), more precisely a sustained remission. Subclinical synovitis can be detected by Ultrasonography (USG) in patients with RA who are in remission and it may predict disease flare. Perturbation of the levels of cytokines and angiogenic markers can be seen even in the remission state.

Aim: This study was intended to find the levels of pro inflammatory and angiogenic mediators among the short term and sustained remission RA patients and also to depict the distribution of US Synovitis score between these two groups. The clinical and laboratory determinants of sustained remission were also evaluated.

Methods: Thirty four RA patients who are in remission, fulfilling both CDAI and DAS28 criteria were recruited . Based on the duration of remission two groups were divided as follows: 1. Sustained remission (remission duration of ≥ 6months) and 2. Short term remission (remission duration < 6 months). Demographic and clinical data were collected. Every patient underwent USG evaluation of 14 joints and grey scale Synovitis (GSS) scoring was done. Following cytokines and angiogenic markers were measured by ELISA (TNF-α, IL-17, IL-1b, ILand VEGF).

Results: Increased pro inflammatory cytokines and angiogenic markers were found in short term duration remission patients [Table 1].High GSS score was observed in short term remission patients with positive correlation with IL-17 and VEGF (r=0.5, r=0.7). Negative correlation has been found between DMARDs initiation gap and duration of remission(r=-0.4).
Table 1

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Conclusion: Upregulated pro-inflammatory and angiogenic mediators and high USG GSS score can be seen in short term remission of RA pointing towards an ongoing inflammatory process. VEGF and IL 17 are associated with sub clinical synovial proliferation in remission state. Early DMARD initiation should be the therapeutic strategy to achieve sustained remission.


  PC0140: Premature atherosclerosis in rheumatoid arthritis is uncommon in patients residing in parasite endemic area Top


Meghanad Meher, Manoj Kumar Parida, Bidyut Das AIIMS BBSR, SCBMCH CTC, SCBMCH CTC

Background: Parasites are known to downregulate immune response through modulation of both innate and adaptive immune system in rheumatoid arthritis. Atherosclerosis has been found to be independent cardiovascular risk factor in rheumatoid arthritis and has been observed in 10-20% of cases.

Aim of the study: Assessment of CIMT (a standard screening test to detect atherosclerosis) in patients of rheumatoid arthritis residing in parasite endemic area.

Methodology and Results: In our cross sectional study we enrolled 58 patients of rheumatoid arthritis diagnosed by ACR-EULAR 2010 criteria. We excluded patients having diabetes mellitus, hypertension, dyslipidaemia, hypothyroidism, CKD and smoking. CIMT is measured by B mode USG scan by using 3-12 mega Hz probe on grey scale over bilateral common carotid artery. Out of 58 patients 48 were female and 10 were male. Average age was 44.1±11 years. Average duration of disease was 4.1±1.2 years. Rheumatoid factor was positive in 48 patients and negative in 10 patients. Average ESR was 73±32. Average CRP was57±42. Average DAS 28 score was 6.5±0.92. Average CIMT in Rheumatoid Arthritis patients was 0.65±0.10mm as compared to control value of 0.57±0.30mm. 3 out of 58(5.17%) patients had high CIMT value (i.e.>0.80mm).

Conclusion: Prevalence of atherosclerosis in rheumatoid arthritis is uncommon in patients residing in parasite endemic area despite a chronic inflammatory state. However a study on a larger number of patients will validate this observation.


  PC0141: To evaluate prevalence of low bone mineral density in otherwise normal premenopausal Indian women: A cross-sectional study Top


Ankur Dalal; The Sarvajanik Medical Trust Hospital, Rampura, Surat, Gujarat, India

Background: Osteoporosis and osteopenia are major public health problem and growing concern in both developed and developing countries worldwide including India. This entity is of utmost important; however, routine screening is not a common practice in India due to lack of awareness, facilities and cost-effectiveness in measuring bone mineral density (BMD).

Objectives: Evaluation of prevalence of low BMD in otherwise normal premenopausal Indian women by screening with peripheral-dexa (p-DEXA) heel BMD measurement.

Methods: Total 66 premenopausal adult women voluntarily attended free screening camp at tertiary care hospital were included in the study after applying inclusion and exclusion criteria. Peripheral heel BMD was measured using p-DEXA bone densitometer. T-score was calculated as per WHO equivalent for heel BMD [1]. Data were collected and analyzed using descriptive statistics.

Results: Mean age of study population was calculated as 34.32 ± 16.09 years. Overall prevalence of osteoporosis observed was 19.69% while of osteopenia was 39.39%. Prevalence of low BMD (osteopenia plus osteoporosis) in otherwise normal premenopausal Indian women observed in study was 59.09% [Table 1].{Table 31}

Conclusion: Prevalence of osteoporosis and osteopenia is significantly high even in otherwise normal premenopausal Indian women. Its screening by measuring peripheral heel BMD using p-DEXA in routine clinic practice is convenient, cost-effective and quite useful in planning preventive strategies to improve bone health and thus reducing the future fracture risk.

Reference

  1. World Health Organization. Assessment of Fracture Risk and its Application to Screening for Postmenopausal Osteoporosis: Report of WHO Study Group. Technical Report Series 843. Geneva, Switzerland: World Health Organization; 1994.



  PC0142: Clinical profile of primary antiphospholipid syndrome Top


Arul Rajamurugan; Madurai Medical College, Madurai, Tamil Nadu, India

Background: Antiphospholipid syndrome ( APS ) is a systemic autoimmune disorder characterised by arterial and venous thrombosis and / pregnancy morbidity in the presence of antiphospholipid antibodies. APS occurs as primary condition or it can occur with SLE or other autoimmune diseases.

Aim: To study the clinical profile of primary APS in patients attending Rheumatology OP. Methods: A retrospective analysis of the records of the patients fulfilling the diagnostic criteria of primary antiphospholipid syndrome who attended rheumatology op over a period of 2 years from 2017 – 2019 was done. After thorough Clinical examination, haematological, immunological studies were done. Results: Out of 20 patients studied,1 presented with catastrophic APS, 8 had deep venous thrombosis; 5 had cortical venous thrombosis; 1 had chronic IVC thrombosis; 1 had isolated thrombocytopenia; 4 presented with pregnancy morbidity. Out of all 12 were female and 8 were males. Manifestation incidence(%). DVT lowerlimbs 40, Cortical venous thrombosis 25, IVC thrombosis 5, Foetal loss 20, Thrombocytopenia 5, Catastrophic APS 5.

Conclusion: From this study, DVT has higher proportion in primary APS followed by pregnancy morbidity. There is a female preponderance.


  PC0143: Spectrum of connective tissue disease in a community based center Top


Bharat Veer Manchanda, Anuradha Venugopalan, Rahul Patil, Arvind Chopra; Rheumatology Fellow,

Introduction: Undifferentiated CTD and overlap CTD has been described since long but not sufficiently enough to allow classification based on the generally accepted criteria. Whether the CTD-U represents distinct clinical entities or the presentation of well-defined connective tissue disease (CTD) is still a matter of debate.

Methods: This is a cross-sectional retrospective study of patient data extracted from the CRD database. Study period:2016-2018. Patients with the clinical diagnosis of undifferentiated features (CTD-U) and/or overlap of two CTD(OCTD) analyzed and presented.

Results: 192 patient records retrieved with diagnosis of CTD-U or OCTD. The basis of diagnosis was predominantly clinical. Mean age was 38 years (range:13-77 years). Male: Female ratio 1:9. Mean disease duration was 6 years (range: 2 months-30 years). Tobacco consumption was in 26 patients whereas 14 patients had a habit of taking regular alcohol. 128 patients had musculoskeletal involvement as initial symptom at onset in which 89% were polyarticular. CTD-U diagnosed in 78 patients; OCTD:55; Mixed-CTD:32; Systemic Lupus Erthyematosus:15; Progressive Systemic Sclerosis:9; Dermatomyositis:2; Autoimmune Thyoiditis:1. Co-morbidity was seen in 32 patients (Diabetes=1; hypothyroidism=20; hypertension=11; asthma=1). Systemic involvement and laboratory features.

Conclusion: Our study shows that there is little doubt that the overlap or undifferentiated diseases represent a diverse clinico-serological spectrum characterized by a mild clinical picture which changes little over time. Also, these patients show limited autoantibody repertoire with a low incidence of disease-specific autoantibodies. Early recognition and unusual associations of symptoms is crucial in management of such patients.


  PC0144: Co-existence of ra and gout: A rerospective analysis from a community referal centre Top


Bharat Veer Manchanda, Anuradha Venugopalan, Abraham Mohan, Arvind Chopra; Rheumatology Fellow

Introduction: Gout and RA are common inflammatory arthropathies, with the prevalence of RA is 0.67(rural) & 0.32(urban) and that of gout 0.13(rural) and 0.06(urban) (Chopra et al,2009). For reasons not understood the RA-Gout coexistence in published literature is UNCOMMON when both disorders are not uncommon in the community.

Methods: Data extracted from referral database in CRD maintained since 1996. This was a cross-sectional retrospective study with prospective follow-up. Study period was 2001-2018. The basis of diagnosis for RA and gout was clinical. Synovial fluid aspiration done to demonstrate crystal in few difficult cases.

Results: From 53498 case records during study period, RA alone was diagnosed in 17480(32.7%); 784(1.5%) only gout and 54(0.1%) diagnosed with co-existence of Gout & RA; Male:Female=5:1; mean age 52years (range:18-77years). 32 patients fulfilled ACR Criteria(2010) for Rheumatoid Arthritis and ACR criteria(2015) for gout. 31.5% used tobacco; 25.9% consumed regular alcohol. 7.4% reported classical podagra. Classical tophi seen in 9 patients; single case presenting chalky white discharge from tophus. Articular pattern was pre-dominantly polyarticular. All had significant early morning stiffness(>30-45 minutes). MTP were commonest joints involved followed by ankle. Radiological evidence of gout seen in 28(51.9%) & of RA in 9(16.7%); 21 had mild-to-no-erosive disease on x-ray. Hyperuricemia in 75%(mean serum uric acid=8.3 mg/dl); RF &/or Anti-CCP positive in 51%. Two had renal calculi; none had renal insufficiency/failure; co-morbidity in 12 patients (Hypertension:6;IHD:1;Obesity:4;Diabetes Mellitus:1). Standard RA treatment and Allopurinol was the treatment in all cases. 52 cases have been followed-up (mean follow-up period:4years).

Conclusion: Coexistence of gout & RA was often clinical but satisfied ACR criteria for both in 2/3rd patients. It may be prudent to speculate that this is often a missed combination by rheumatologists. Whether co-existence of gout in RA increases bone damage & kidney, needs to be evaluated prospectively in a larger cohort.


  PC0145: Early rheumatoid arthritis is an advantage with community based rheumatology centers: Lesson from CRD, Pune Top


Rahul Patil, Anuradha Venugopalan, Arvind Chopra; Rheumatology Fellow

The need for early diagnosis and early treatment in Rheumatoid arthritis (RA) is critical and this opportunity is usually lost in tertiary rheumatology centres. As a community based referral centre we were keen to know that with popularity and time, are we seeing an increase in the proportion of early RA (ERA) cases.

Methods: The current study is a retrospective study of patient records from Jan 2015-Dec 2018 extracted from a comprehensive database, maintained since 1996. The first visit profile of patients diagnosed with early RA(ERA) within 12 months of onset of symptoms is being presented.

Results: Among 64,689 first(initial) visit patient records maintained since 1996, 30,878(47.7%) have been diagnosed as RA. Since 2015–2018, among 4140 RA, 915(22.1%) have been diagnosed within 12 months of disease onset.

Overall Female:Male ratio in the early RA(ERA) cohort was 4.6:1. Median age 45years. Although almost equal proportion of patients came from the rural & urban areas, the larger percent of patients were walk-in(87.8%) as compared to those who were referred by physicians. The year wise data, baseline clinical and laboratory features.

Conclusion: Early RA needs immediate specialist assessment and care. The proportion of ERA in our center has been increasing over the years; a lesser proportion showing presence of erosions at their first visit to our center. A significant number of patients were walk-in rather than being referred by primary care physicians. The awareness of the disease and the necessity to approach specialists or tertiary care was as good in rural as among the urban population.


  PC0146: A comparative study between low dose versus standard dose rituximab in patients of rheumatoid arthritis in a tertiary care center of North Bengal Top


Saikat Singh, Pasang L. Sherpa, Biswadip Ghosh; North Bengal Medical College and Hospital, Siliguri, West Bengal, India

Background: Rheumatoid arthritis is a very common chronic poly-arthritis which can lead to joint deformity and restriction of activities. Rituximab is a chimeric monoclonal antibody directed against CD20 molecule present on mature B cell surface.

Objective: In this study, we aimed to prove non inferiority of low dose rituximab(2*500mg) compared to standard dose(2*1000mg) as assessed by treatment response at 6 weeks and 12weeks using 2 parameters DAS28ESR and CDAI.

Methods: 30 patients, who failed to respond to methotrexate alone or a combination of DMARDs, were included in the study. They were divided in 2 groups randomly. 1 group received 2 doses of rituximab 500mg each and the other group received 2 doses of 1000mg each at 2 weeks interval.

Results: We found that in low dose group mean DAS28ESR scores at beginning, 6 weeks and 12 weeks were 6.89, 4.58 and 3.25 respectively; CDAI scores for the same group were 53.06, 25.2 and 14.13 respectively. Both parameters showing significant improvement; p value for DAS28ESR scores were 0.00001 for 6 weeks and 0.0001 for 12 weeks and p value CDAI scores were 0.01 for 6 weeks and <<<0.05 for 12 weeks. In the standard dose group, DAS28ESR scores at beginning, 6 weeks and 12 weeks were 7.0, 4.85 and 3.53 respectively; CDAI scores for the same group were 53.33, 26.33 and 15.06 respectively, showing significant improvement with p values of 0.003 and 0.00007 for DAS28ESR for 6 weeks and 12 weeks respectively and 0.006 and 0.007 for CDAI for 6 weeks and 12 weeks respectively. However, there was no statistically significant difference among the two study groups as indicated by p value >0.5(0.67 for DAS28ESR and 0.12 for CDAI).

Conclusion:

Low dose rituximab is non inferior to standard dose rituximab in efficacy in rheumatoid arthritis upto atleast 12 weeks.


  PC0147: Outcome at a median period of 5 years on methotrexate and prednisolone therapy in patients with Takayasu's Arteritis Top


Koshy Nithin Thomas, Avinash Jain, Durga Prasanna Misra, Amita Aggarwal, Able Lawrence, Vikas Agarwal, Latika Gupta, Ramnath Misra; Department of Clinical Immunology and Rheumatology, SGPGI, Lucknow, Uttar Pradesh, India

Background: There is paucity of data on long term outcome of TA patients on conventional immunosuppressants. We aimed at analysing outcome (response, remission and refractory disease) of TA patients on Methotrexate using validated outcome measures.

Methods: Patients who fulfilled ACR criteria of TA with a minimum 1-year follow up on Methotrexate and prednisolone were retrospectively analysed. ITAS, ITAS-A and TADS score were retrieved at baseline and last follow up. ITAS ≥ 2 and ITAS-A ≥ 3 (with ITAS at least 1) were defined as active disease. Refractory disease was defined as no response or angiographic progression.

Results: Thirty-four TA patients (24 female) with median (IQR) age of 27 (20.75-39) years and disease duration of 18 (11-49) months were included. The median(IQR) follow-up period was 4 (2-5) years. Methotrexate therapy resulted in remission in 23/34 patients. Median (IQR) ITAS, ITAS-ESR(A) and TADS at baseline were 13 (7.75 – 16.25), 15 (9.5 – 18) and 1(0-3.25) respectively, and were 0, 2 (1-3) and 7.5 (6-9.25) at last visit respectively. All but 4/34 patients showed accrual of damage with median change in TADS 6 (3-8). Median dose (IQR) of Methotrexate at baseline was 15 (10-15). Methotrexate was switched to Azathioprine and MMF in 6 and 1 patients respectively due to unresponsiveness after median period of 2 (1-2 years). Six out of these 7 patients went into remission. Three patients had refractory disease (including 1 in whom Methotrexate was changed). Two patients had relapse on Methotrexate after 1 and 2 years of treatment. Six patients were off steroids at last visit. Radiographic progression was seen in 9/10 patients. Revascularisation procedures were done in 6 patients. No predictors for remission, refractory disease could be identified.

Conclusion: Methotrexate and corticosteroid effectively provide remission in the majority (67%) of patients with Takayasu' arteritis.


  PC0148: Assessment of safety and efficacy of apremilast in psoriatic arthritis patients: Single centre study Top


Rahul Babu, Reshma Reji, K C Shanoj, Padmanabha Shenoy; Dr Shenoy's Care, Centre for Arthritis and Rheumatism Excellence, Chennai, Tamil Nadu, India

Background: Psoriatic arthritis (PsA) is a chronic inflammatory arthritis seen in 30 to 40 % cases of psoriasis. Treatment with conventional disease modifying anti rheumatic drugs (cDMARDs) as monotherapy or in combination therapy have not yielded satisfactory. Results: Our study analyses the effects of the novel agent, Apremilast, in both skin and joints.

Objectives: To analyse the efficacy and safety of Apremilast in patients with PsA.

Methods: A retrospective observational assessment of experience with Apremilast in PsA patients from a single centre. Data were collected during the period from Feb 2018 to July 2019 using electronic medical records. All patients initiated on Apremilast 30 mg BD were included in the study. Their demographic details, co morbidities, baseline disease activity (tender joint count (TJC), swollen joint count (SJC) and Psoriatic arthritis severity index (PASI)) were collected. Their current therapy status i.e., co prescribed DMARDs/Steroids were also included.

Results: A total of 157 patients were selected. Apremilast was discontinued in 13 patients due to intolerance. 26 patients had low disease activity, 7 had insufficient data and 2 had only psoriasis. So in the effective sample size of 109 patients, 42 (38.5%) were found to be females. Baseline mean age and disease duration were 48.84±10.30 & 50(42, 55) respectively. A significant change from baseline disease activity was found at the end of 3 months.The change in TJC, SJC and PASI were not significantly different in the early PsA and late PsA groups. A total of 25 patients were not tolerating optimum dose of Apremilast. The most common adverse events were gastric intolerance and diarrhoea. Detailed Results: of the study are depicted.

Conclusion: Apremilast was found to be an effective option for controlling inflammation in both skin and joints in PsA patients.


  PC0149: Clinical profile of patients with anti synthetase syndrome admitted to Dept. of Rheumatology IPGME&R and SSKM Hospital, Kolkata Top


Basil Paul Kunnathu, Alakendu Ghosh; Department of Rheumatology, IPGME&R and SSKM Hospital, Kolkata, West Bengal, India

Introduction: Anti-Synthetase Syndrome (ASS) is an uncommon autoimmune disease characterized by the presence of antiaminoacyl tRNA synthetase (anti ARS) antibodies along with interstitial lung disease, inflammatory myositis, arthritis, fever, mechanics hand and Raynaud's phenomenon. The clinical presentation of ASS is variable and partly depends on the type of antiARS antibody present.

Methods: A retrospective analysis of the case records of patients with Anti Synthetase Syndrome, who were admitted to the Department of Rheumatology, IPGME&R and SSKM Hospital, Kolkata between October 2014 and February 2019.

Results: Nine patients diagnosed with Anti Synthetase Syndrome were studied. There were five females and four males. The mean age at diagnosis was 34.4 years. Four patients were Jo1 positive, while three were PL 7 positive, one with PL 12 positivity and another one with OJ positivity. ILD was seen in six of the patients and all of them had NSIP pattern. Mechanic's hand was seen only in one patient. Typical rash of dermatomyositis was seen in five patients. Arthritis was present in four patients and fever in six patients at diagnosis. Only one patient had Raynaud's phenomenon. Eight out of the nine patients had clinical proximal muscle weakness, whereas one patient had subclinical myositis. Bulbar involvement was seen in one patient.

Discussion: In our cohort of patients with Anti Synthetase Syndrome, anti Jo 1 was the most common anti ARS antibody detected, followed by anti PL 7. ILD was seen in 66.6% of patients and all of them had NSIP pattern. All the patients had inflammatory myositis, with one patient having subclinical myositis.

Conclusion: There is much heterogeneity among different patients in the presentation of anti-synthetase syndrome and this variability can be associated with various autoantibodies.


  PC0150: Incidence and predictive factors of restrictive lung disease in rheumatoid arthritis patients Top


Debanjali Chakrabarti, Chiranjit Bal, Kaushik Basu; Departments of Physiology and1Medicine, In-Charge Rheumatology Unit, Medical College and Hospital, Kolkata, West Bengal, India

Background: Rheumatoid Arthritis (RA), a chronic inflammatory disorder of unknown etiology characterised by symmetrical peripheral polyarthritis often resulting in joint damage and physical disability. Several extra-articular manifestations are also associated with RA out of which pleuro-pulmonary involvement is the most important as it bears clinical significance in terms of increased morbidity and mortality. Pulmonary Function Tests (PFTs) are widely used to provide objective measure of lung function for detecting and quantifying such lung diseases.

Objectives: The primary aim of this work is to evaluate and characterize the PFT patterns in RA cases compared to normal controls and find the predictive factors of restrictive lung disease in these cases.

Methodology: Detailed history taking and clinical examination done after informed consent. Pulmonary Function Tests conducted in 50 patients of either sex and above 16 years of age with active RA and also in 50 age and sex matched controls.

Data mining and statistical analysis done.

Results: 38 (76%) out of 50 of cases had evidence of lung disease on the basis of abnormal PFT parameters. The PFT parameters like FVC (%), FEV1(%), FEF25-75% (%), PEFR (L/min) and MVV(L/min) were significantly reduced in cases as compared to controls. 34% of cases had Restrictive lung disease, 4% had obstructive lung disease, 22% had small airway disease and rest 16% had mixed airway disease based on their PFT patterns. Mean duration of disease in RA patients having restrictive lung disease was 4.72 years (SD = 1.2 years) and had high Anti-CCP titers.

Conclusion: Pulmonary Function Tests can be regarded as an effective diagnostic, prognostic and research tool to detect and categorize lung diseases associated with Rheumatoid Arthritis and ultimately guiding treatment protocols.


  PC0151: Osteoporosis in scleroderma: Prevalence, clinical characteristics and correlation with disease phenotype, organ involvement, and vasculopathy Top


Dhaval Tanna, Shounak Ghosh, Lucky Sharma, Wasim Kazi, Shruti Bajad, Rohit Bajaj, Vinay Singal, Rajiva Gupta; Medanta-The Medicity, Gurgaon, Haryana, India

Background: Inflammation is considered to be the causal factor for osteoporosis in rheumatic diseases. However, apart from inflammation, scleroderma is uniquely characterized by vasculopathy, for which clinical features such as digital gangrene and pulmonary arterial hypertension are considered to be manifestations. Studies have shown that in scleroderma patients, digits having severe digital gangrenes have higher prevalence of bone loss in the form of acro-osteolysis. We therefore extrapolate that scleroderma may have higher prevalence of osteoporosis because of systemic vasculopathy apart from inflammation.

Methods: All patients classified as having Scleroderma as per the American College of Rheumatology/European League Against Rheumatism 2013 criteria were included. After written informed consent, demographic profile, clinical features, laboratory and radiology parameters were recorded. Presence of osteoporosis was assessed by two-site dual-energy X-ray absorptiometry (DEXA) scan (hip and lumbar spine) and was correlated with disease phenotype and vasculopathy. The study was approved by the Ethics Committee (MICR- 771/2017).

Results: From June 2017 to May 2019, 115 patients were diagnosed with scleroderma. 90 patients underwent DEXA scan and were included in the present analysis, with 45 healthy controls taken for comparison. Out of the 90 patients, 31 (34.4%) and 3 healthy controls (6.66%) had osteoporosis [p<0.001]. No association was found between osteoporosis and interstitial lung disease, duration of disease and type of serology. In univariate analysis, old age, low BMI, presence of menopause, presence of digital ulcers, PAH, GI involvement and diffuse cutaneous phenotype were significantly associated with presence of osteoporosis (P < 0.05) [Table 1]. No association was found between glucocorticoid intake and prevalence of osteoporosis [Table 2].
Table 1: Comparison of clinical and serologic characteristics in patients with and without osteoporosis

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Table 2: Multivariate analysis was applied with the help of regression models to determine independent predictor of osteoporosis in systemic sclerosis

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Conclusion: Scleroderma is associated with an increased prevalence of osteoporosis. Vasculopathy may be an important pathophysiologic mechanism causing bone loss as shown by higher prevalence of digital ulcers, pulmonary arterial hypertension, and diffuse cutaneous phenotype in patients with osteoporosis.


  PC0152: A study of long term disease outcome in patients having rheumatoid arthritis with disease duration greater than 10 years Top


Wasim Kazi, Kaustubh Telang, Lucky Sharma, Dhaval Tanna, Shruti Bajad, Durgarao, Vinay Singhal, Rajiva Gupta; Medanta- The Medicity Hospital, Gurgaon, Haryana, India

Background: Rheumatoid arthritis is associated with multiple extra articular manifestations. Patients with severe disease &/or poor control of disease activity are more likely to develop erosions, deformities and extra-articular manifestations. Studies have shown that most of the manifestations develop within first 5 years of disease. This observational cross sectional study aimed to study different extra articular manifestations in rheumatoid arthritis.

Methods: This cross-sectional study was conducted at Department of Rheumatology and Clinical Immunology at Medanta- The Medicity Hospital, Gurugram from July 2017 till June 2019.

The study subjects were those diagnosed and followed up in the Rheumatology clinic from July 2017 to June 2019. Demographic profile, clinical features, laboratory data, treatment details were noted. Disease characteristics including deformities or other morbidities and associated complications were recorded.

Study was approved by Institutional Research board (IRB). Ethics committee approval was also taken. (Reference number: MICR-789/2017).

Results: A total of 250 patients of Rheumatoid arthritis were studied.Females comprised the majority of the patients included in the study. Median age of onset of RA was 38 years with median duration of 15 years. All patients underwent DAS28 assessment to know the disease activity. Secondary Sjogren's syndrome was observed in 125 patients. Osteoporosis was observed in 53 patients. Interstitial lung disease was diagnosed in 36 patients, nodule in 33, coronary artery disease in 22 and fibromyalgia in 21 patients. Less common extra-articular manifestations observed in our study were peripheral neuropathy, vasculitis, eye manifestations, malignancy and amyloidosis.
Table 1: Comparison of our findings with various previous studies

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Conclusion: Patients with longer disease duration of more than 10 years were likely to have more extra articular manifestations. Disease duration was found to have direct correlation with these manifestations; Secondary Sjogren's syndrome being the commonest in our cohort followed by osteoporosis and Interstitial lung disease.


  PC0153: Bone density in patients of systemic sclerosis and its relationship with disease related parameters Top


Ankit Patawari, Pradyot Sinhamahapatra, Alakendu Ghosh; Department of Rheumatology, IPGME&R and SSKM Hospital, Kolkata, West Bengal, India

Systemic sclerosis (SSc) is an uncommon connective tissue disease characterized by progressive fibrosis of the skin, vasculopathy, and immune activation. Skeletal manifestations of SSc may include fibrosis of the joint capsule, flexion contractures, thickened tendons, or inflammatory, erosive, and non-erosive arthritis. Recent data suggested an increased risk of osteopenia and osteoporosis in patients with SSc. Risk factors, such as age, low body mass index (BMI), previous fragility fractures, a family history of fractures, use of glucocorticoids, early menopause, systemic inflammatory disease, and active cigarette smoking are classically associated with OP. Before a therapeutic program of research is undertaken, a basic understanding of the epidemiology of low bone density in the setting of SSc is needed, namely the prevalence, determinants of the occurrence of low bone density, and its effect on clinically relevant outcomes.

Objectives:

  1. Evaluation of Bone Mineral Density in patients with Systemic Sclerosis
  2. To evaluate the relationship of low Bone Mineral Density in Systemic Sclerosis patients with disease related parameters


Methods: In this Cross-sectional observational study, 100 patients with Systemic Sclerosis who fulfilled the criteria were analyzed from 01 July 2018 - 31 August 2019.

Results: The average disease duration was 9.5 years. BMD showed T score of less than -1.0 in SSc patients. There was low level of Vitamin D levels, and this correlated with the severity of joint involvement, malabsorption syndrome and positivity for certain auto-antibody like anti-Scl 70. There was no correlation with the severity of skin thickening, ILD or pulmonary artery hypertension.

Conclusion: Systemic Sclerosis patients with low BMD have prolonged disease duration, severe joint involvement, malabsorption syndrome and positivity for certain auto-antibody like anti-Scl 70. Patients of Systemic Sclerosis are at a higher risk of losing bone density, especially when other osteoporosis risk factors are present.


  PC0154: Factors predicting mortality in systemic sclerosis – Hospital-based case control study Top


T G Sundaram, Hafis Muhammed, Sakir Ahmed, Durga Prasanna Misra, Ramnath Misra, Amita Aggarwal, Able Lawrence, Vikas Agarwal; Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Systemic sclerosis is characterized by high morbidity and mortality due to disease-related or treatment-related complications.

Objectives: We evaluated baseline characteristics of patients of systemic sclerosis who experienced mortality, compared with age and sex matched controls, in a case-control design.

Methods: Records of patients of systemic sclerosis who died between 2000 and august 2019 were reviewed and causes of their deaths determined. We then compared their clinical characteristics and laboratory parameters with age and sex matched live controls in the ratio 1:2.
Table 1

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Results: There were a total of 35 deaths. The causes were 12 Interstitial lung disease (ILD) related deaths (34.3%) (11 Lower respiratory tract infections (LRTI), 1 Pulmonary Koch's), 5 Gastrointestinal (GI) sepsis (14.3%), 5 Scleroderma renal crisis (SRC) (14.3%), 3 Cellulitis sepsis (8.6%), 3 severe pulmonary arterial hypertension (PAH) (8.6%), 2 metastatic carcinoma (5.7%), 1 each of myositis with type 2 respiratory failure, viral myocarditis, liver cirrhosis of unknown etiology, 1 disseminated Koch's, 2 unknown sudden deaths probably of cardiac etiology. Median age at death was 49 (Interquartile range, 39-58) years, median disease duration at death was 72 (Interquartile range, 36.75-116) months, and median duration of follow-up was 24 (Interquartile range, 2.5-58.5) months. On univariate analysis. patients who died had greater number of admissions, deranged renal function, higher Aspartate transaminase (AST) levels, lower serum protein and albumin levels. A significantly higher number of patients in the death group were immunosuppressed (58.8%) compared to live controls (41.2%; p= <0.001). There was no significant difference between the two groups in terms of disease manifestations like Modified Rodnan skin score (mRSS), ILD, PAH, arthritis and myositis.

Conclusion: Most deaths in patients of systemic sclerosis were because of infectious complications; more number of hospital admissions, deranged renal functions, higher AST and lower serum albumin at baseline associated with mortality.


  PC0155: Factors influencing the utilization of total knee replacement in osteoarthritis knee: A web-based survey of treating doctors Top


G C Yathish, Yogesh Preet Singh, Lokesh Veerappa1, G. Mallinath1, Hemant Kalyan1; Departments of Rheumatology and1Orthopedics, Manipal Hospital, Bengaluru, Karnataka, India

Background: India, with its aging population is going to face a major healthcare burden due to Osteoarthritis (OA) knee in the coming decades. Total Knee replacement (TKR) which is the most effective way of treating severe OA knee, has wide variation in its utilization. The objective of this survey was to gain insight into the factors that influence the treating doctor in referring a patient to TKR.

Methods: Rheumatologists, Orthopedicians and Physicians were invited to complete a fourteen item web based survey.

Results: Out of the 202 respondents, 140(70%) were Rheumatologists, 47(23.5%) Orthopedicians and 13(6.5%) Physicians. Majority (n=126,63.3%) opined that TKR was underutilized in the treatment of OA knee. Functional demands of the patient were considered as the most important patient factor which influences the decision to refer to TKR by 170 (85.8 %) respondents. Financial status of the patient and the availability of health insurance were considered as important socio economic factors influencing referral to TKR by 100(51.3%) and 70 (35.9%) respondents respectively. High cost of TKR was considered as the biggest barrier for underutilization of TKR by 112(56.6%) respondents. The recent price regulation introduced by governing agencies on knee implants had mixed views with 88(44.2%) saying that it has improved access of TKR to larger population and 84(42.2%) saying it has not done any impact.

Conclusion: High cost and non-affordable population remains the biggest barrier for utilization of TKR in OA knee.


  PC0159: Clinical and angiographic features of adult primary angitis of central nervous system Top


Shyamashis Das, Ashis Datta, A. Shobhana, Sukalyan Purkayastha; Institute of Neurosciences, Kolkata, West Bengal, India

Objective: To observe clinical features, angiographic abnormalities and response to therapy in primary angitis of central nervous system (PACNS).

Methods: Adult patients with PACNS, diagnosed by Calabrese's criteria, were recruited for this longitudinal open label study. All of them had abnormalities in digital subtraction angiography (DSA) highly suggestive of vasculitis (narrowing, occlusion or dilation in a segmental pattern affecting multiple cerebral arteries in absence of changes consistent with atherosclerosis) with raised protein in cerebrospinal fluid. All patients were treated with methyl-prednisolone pulse followed by oral prednisolone and mycophenolate and standard care for stroke.

Results: From November 2016 to May 2019 twenty-three patients (15 females) were recruited. Median age was 45 years (range 32-72 years) and median follow up period was 16 months (range: 3-29 months). Most common clinical feature was headache (86.9%) followed by recurrent stroke (56.5%), speech difficulty (30.4%), loss of consciousness (21.7%), balance problem (17.3%), abnormal sensation (13%) and photophobia (4.3%). Brain MRI showed infarcts in 85.7% and hemorrhage in 14.3%. DSA showed bilateral involvement of vessels in 71.4%, medium vessel involvement in all and both medium and small vessel involvement in 57% patients. Narrowing or occlusion of arterial lumen were multifocal in all. DSA demonstrated typical arterial beading pattern, collateral vessels, sharp cut-off and aneurysm in 52%, 40%, 40% and 4% respectively. Follow-up DSA was done in 8 patients after a median period of 12 months and 3 patients showed definite angiographic improvement and rest did not show any significant changes from initial angiogram. Mean clinical improvement on disability, measured by Rankin disability scale, was 2.5 (range 2-4) from baseline. There was no recurrence or worsening of symptoms in any patient till last follow up.

Conclusion: Combination of mycophenolate and corticosteroid resulted in significant clinical improvement and prevented angiographic worsening in PACNS.


  PC0160: Validation of simplified disease activity score forankylosing spondylitis Top


T G Sundaram, Hafis Muhammed, Amita Aggarwal, Latika Gupta; Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background and Objective: Currently available disease activity measures in Ankylosing spondylitis (AS) are complex and cannot be assessed in a busy out-patient clinic without a calculator. The Simplified Ankylosing Spondylosis Activity Score (SASDAS) was devised to obviate this need. We prospectively validated SASDAS and studied whether juvenile onset (<18 years), early disease (<2 years), and pattern of disease (axial or peripheral) impacted these scores.

Methods: Patients with AS (New York Criteria) were assessed for Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Ankylosing Spondylitis Disease Activity Score (ASDAS), with ESR and CRP and various AS disease parameters at baseline and at 3 months. SASDAS-ESR and SASDAS-CRP were derived by simple addition of individual components of the ASDAS-ESR and ASDAS-CRP score. PGA31 was defined as active disease. Those with change in ASADAS31.1 were classified as responders. Fisher's r to Z transformation was used to compare correlations.

Results: 107 patients (96 male and 11 female) with median age of 29 years (Interquartile range- 22-38) and median disease duration of 6 years (Interquartile range- 2.5-12) were enrolled of which 84.1% were HLA-B27 positive and 38.3% had juvenile onset. Thirty-four patients were followed-up at 3 months. SASDAS (ESR, CRP) showed high correlation with BASDAI (r=0.85, r=0.82), ASDAS-ESR (r=0.95, r=0.75), ASDAS-CRP (r=0.80, r=0.89); all with p<0.001. The SASDAS CRP but not ESR showed good internal consistency (Cronbach's α 0.68 vs 0.18) while both had discriminative validity and sensitivity to change.

Based on previously proposed cut-offs of disease activity defined for ASDAS-CRP, 4.8, 12.8, 22.8 were optimum cut-offs for classifying inactive, moderate, high and very high disease activity by SASDAS CRP. SASDAS-CRP performed better in late disease (Z=3.04; p=002) and those with adult onset (Z=2.18; p=0.03).

Conclusion: SASDAS is a valid score for measuring disease activity in AS including those with juvenile-onset AS.


  PC0161: Using clinical disease activity index score for assessment of efficacy of abatacept in biological disease-modifying anti-rheumatic drug naïve rheumatoid arthritis Top


A T Atal, S. Kartik; Command Hospital, Pune, Maharashtra, India

Background: The practicability of CDAI score over Disease Activity Score (DAS-28) in assessing disease activity in RA is apparent in Indian OPD setting, obviating the need for acute phase reactant values and complicated calculations. Costimulation blockade with Abatacept in bDMARD naïve patients with Rheumatoid Arthritis refractory to conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs),is effective.

Objective: To assess the efficacy of Abatacept in bDMARD naïve Indian patients with RA using CDAI score.

Methods: This prospective study included 32 patients with CDAI of >10, who had failed conventional therapy with at least 2 csDMARDs, optimized over a period of at least 1 year. They received Abatacept for 12 months. CDAI was measured at baseline, 3, 6, and 12 months. Primary endpoint was the achievement of Low Disease Activity as defined by a CDAI of ≤ 10.

Results: 32 patients (Males- 05, Females-27) were followed up prospectively. 19 were on triple csDMARDs and remaining 13 on two csDMARDs. 31 patients completed 6-month follow-up: 1(3.1%) was in CDAI remission, 13(40.6%) had low disease activity, 16 (50%) had moderate disease activity and 1(3.1%) had high disease activity. One patient defaulted and was lost to follow up after her 3 month review. The patient with high disease activity underwent a switch to Tocilizumab after 6 months. 30 patients completed 12 months follow up, 8(25%) achieved CDAI Remission while 21(65.6%) had CDAI Low Disease Activity. The remaining 1(3.1%) patient had moderate disease activity. Adverse effects eported during the study period were headache (1 patient) upper respiratory tract infection (1 patient), tinea corporis (1 patient).

Conclusion: CDAI is a convenient tool in assessing response to effective therapy with bDMARD Abatacept.


  PC0162: Efficacy of baricitinib in Indian patients with moderate-to-severe rheumatoid arthritis: Subgroup analysis from RA-BEGIN and RA-BUILD Top


Syamasis Bandyopadhyay, Jyotsna Oak1, Sundeep Upadhyaya2, Anurag Agarwal3, Rohit Arora3, Subhashini Arthanari4; Apollo Gleneagles Hospital, Kolkata, West Bengal,1Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra,2Indraprastha Apollo Hospital, Delhi,3Eli Lilly and Company, Gurgaon, Haryana, India,4Eli Lilly and Company, Singapore

Background: Baricitinib, an oral selective inhibitor of Janus kinase (JAK) 1 and JAK 2, is approved in more than 50 countries for the treatment of active rheumatoid arthritis (RA) in adults. RA-BEGIN and RA-BUILD studies have shown clinical efficacy of baricitinib in disease-modifying antirheumatic drug [DMARD]-naive patients and in patients with inadequate response to conventional DMARDs.

Objective: To evaluate the efficacy of baricitinib in Indian patients with moderatetosevere active RA.

Methods: This subgroup analysis included Indian patients from 2 international, multicenter, phase 3, randomized controlled trials: RA-BEGIN (N=47) and RABUILD (N=58). In both studies, the primary efficacy outcome was improvement in ACR20 and secondary efficacy outcomes were improvement in ACR50/70, change from baseline in DAS28-hsCRP, change from baseline in HAQ-DI score, SDAI, CDAI, patient's assessment of pain, and patient's global assessment of disease activity. Treatments were compared across all arms including baricitinib 4-mg, methotrexate (MTX), and baricitinib 4mg+MTX in RA-BEGIN and baricitinib 2-mg, baricitinib 4-mg, and placebo in RA-BUILD using logistic regression and analysis of covariance.

Results: Patients who received baricitinib had greater ACR20 response rates at Week 12 in RA-BEGIN (vs MTX) and at Week 24 in RA-BUILD (vs placebo). Similarly, better improvements were observed in ACR50/70 response rates, HAQ-DI, DAS28-hsCRP, CDAI, SDAI, pain, and disease activity in patients who received baricitinib at Week 12 in RA-BEGIN (vs MTX) and at Week 24 in RA-BUILD (vs placebo) [Table 1].
Table 1: Efficacy measures up to week 24 in RA-BEGIN and up to week 12 in RA-BUILD

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Conclusion: The Results: demonstrated greater improvements in all efficacy outcomes with baricitinib treatment at Week 12 (vs placebo; RA-BUILD) and Week 24 (vs MTX; RA-BEGIN) in Indian patients. These Results: were consistent with those observed in the global population of RA-BEGIN and RA-BUILD studies.


  PC0163: An etiological and clinicopathological study of adult onset nephrotic syndrome in a tertiary care centre of North Bengal Top


Saikat Singh, Debadyuti Mahapatra, Biswadip Ghosh, Pasang L. Sherpa; North Bengal Medical College and Hospital, Siliguri, West Bengal, India

Background: Nephrotic Syndrome describes the clinical condition of heavy proteinuria (>3.0 g/24 h), hypertension, hypercholesterolemia, hypoalbuminemia, edema/anasarca, and microscopic hematuria. It can either be primary(idiopathic) or secondary.

Objective: To study the etiological and clinical and histo-pathological aspects of adult-onset Nephrotic Syndrome and their correlation in a tertiary care centre of North Bengal.

Methods: It is an observational, cross-sectional study. 50 patients were included in the study after meeting the inclusion criterion and taking informed consent. Detailed history was taken followed by clinical examination. After routine and specific investigations were done, patients were sent for USG guided percutaneous renal biopsy and samples were tested using light microscopy and immune-fluorescence study.

Results: Out of 50 patients, 26 were males and 24 were females. Most of them belong to the 25-34 years age group. Most patients were from low socio-economic group. Pedal edema was the most common presenting complaint followed by facial puffiness. According to renal histopathology most common type was FSGS (28%) followed by MGN (22%) and LN (20%). In our study, males dominated in all histologic subtypes except Lupus nephritis, where females predominated. Systemic hypertension was present in 50% of cases. Hematuria was present in 26%. Both the cases of IgAN and MesPGN and all the cases of LN presented with hematuria. 24 hours urine protein excretion or ACR ranged from 3 .5 g to 9 g/ day with average 5.06±1.23. Majority of patients of nephrotic syndrome did not have significant renal dysfunction. Renal dysfunction was seen in 34% of cases but it was 40% among patients of lupus nephritis. Nephrotic syndrome was primary or idiopathic in 80% of cases and secondary in 20% of cases.

Conclusion: FSGS is the leading cause of nephrotic syndrome in adults in North Bengal along with MGN, LN, MCD.


  PC0164: Prospective clinical study in patient with viral illness with arthritis in a tertiary hospital Top


Dharshan Gowda, V P Pandey, D R Ashok Thakur Resident, Professor and Head, Associate Professor

Background: Epidemics of viral fever like chikungunya presenting with fever, rashes and polyarthralgia lasts for 7-10 days. However chikungunya can cause severe chronic arthralgia that can last for months to years. Viral infection show rheumatologic symptoms by immune complex formation and immune dysregulation.

Objectives:

  • To study the pattern of fever and arthritis in patients attending MYH OPD
  • Follow-up of the patients at 3 months interval for 18 months (from December 2017 to May 2019)
  • To evaluate the patients with preexisting morbidity and course of arthritis
  • To know prevalence rheumatoid arthritis like condition.


Methods: Observational prospective study involving 262 patients with fever and arthritis presenting to the Maharaja Yashwantrao hospital.

Results: Females 139[53%] affected more than Males 123[47%]

  • Age ranging from 18 to 80 years with mean age of 39.68 years. Most of the patients were in the age group of 20-40 years [137 patients; 52.3%].
  • Arthralgia 100%, history of fever 53%, fever 47%, rashes 11.8%, headache 27%, family history 7%.
  • Joint tenderness 68%, stiffness 35%, movement restriction 40%, edema 22%.
  • CRP positive in 148(56.5%), RA factor was positive in 13 (5.0%) patients, Chikungunya IgM antibody was positive in 61 (23.3%) patients, equivocalresult in 5 (1.9%) and Dengue IgM antibodies were positive in 4 (1.5%) patients.
  • Joint involvement at the beginning, 3rd month and 18th month are 100%, 80% and 51% respectively.
  • EULAR criteria positive in 33 patients in 1st visit, 16 patients in 2nd visit.
  • Mean number of joints involved at 1st visit 6.25, 2nd visit 4.05 and 3rd visit 1.9.
  • Joints involved-Knee [81%], wrist [78%], small joints [58%], ankle [47%], elbow[52%] and axial[23%].


Conclusion: Chikungunya has high prevalence of persistent rheumatic symptoms. Study shows the pattern of joint involvement, duration of arthralgia in viral arthritis.
Figure 1: Comparison of patient having EULAR SCORE more than 6 at different visits

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  PC0165: Experience of rituximab in idiopathic inflammatory myositis: Data from Myo-IN registry Top


Ramya Janardana, Aneesa Kapadia1, Sanjiv Amin1, Liza Rajashekhar2, Latika Gupta3, Vineeta Shobha, Ramnath Misra3; Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, Bengaluru, Karnataka,1Consultant Rheumatologist, Mumbai, Maharashtra,2Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana,3Department of Clinical Immunology, Sanjay Gandhi Post Graduate Institute of Medical Sciences (SGPGIMS), Lucknow, Uttar Pradesh, India

Objectives:

  1. Study the prescribing pattern and outcome of idiopathic inflammatory myositis (IIM) patients managed with Rituximab
  2. Study the safety profile of Rituximab


Methodology: Myo-In registry (4 centers) based retrospective data on IIM patients who were given Inj Rituximab in a tertiary care setup over last 5 years. Rituximab was administered either as 500mg or 1g dose 2 weeks apart. All patients were continued on concomitant immunomodulators.

Results: Fifty-five patients of IIM, 42 of whom were females, with a mean (SD) age group of 38(±13.6) years were administered Rituximab. Three patients had juvenile dermatomyositis (JDM), 24 were dermatomyositis (DM), 12 were anti-synthetasesyndrome (ASS), 9 were overlap myositis and 7 were polymyositis [Table 1].

Information on outcome was available at last followup in 40 patients, at 6 months in 37 patients. Median (IQR) duration of follow-up post Rituximab was 24(11,36) months, majority received single course. All patients except one had at least partial improvement by 6 months. Fifteen patients had complete improvement by 6 months and 30 patients had complete improvement by the last follow-up. No significant difference was observed between partial and complete responders at 6 months.

In the subset with available follow-updata (n = 40), infusion reaction was not reported with any of the infusions. Two patients developed serious infection in the form of lower respiratory tract, both survived. No deaths have been reported during the follow-up period.

Conclusion: Rituximab is safe and efficacious in IIM in this cohort. No specific clinical or laboratory factors are associated with early complete response.


  PC0166: Profile of critical illness and its outcome among children with pediatric SLE, admitted in PICU in a tertiary care centre in South India Top


S Indhuumathy Thayammal, T K Shruthi, Mahesh Janarthanan, P S Rajakumar, S Shubha; Department of Rheumatology, Division of Pediatric Rheumatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Background: SLE is an autoimmune disease. The outcome of critical illness in adults requiring ICU admission is well established but pediatric counterpart is lacking. Hence we decided to present this study.

Objective: To study the profile of critical illness and its outcome in pediatric SLE.

Methodology: This is a retrospective observational study at PICU, SRMC& RI, SRIHER over 2 months. Pediatric SLE children admitted to PICU with critical illness during Jan 2010 to May 2019 were included. The demographic details, complication and management data were collected from case sheets and studied.
Table 1

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Results: Out of 285 admissions among 87 SLE children, 14 developed critical illness needing PICU admissions (16 admissions).The median age at PICU admission was 13yrs (1.5-17), showing a female preponderance 11:3. Average length of PICU stay was 8 days. ARDS(12.5%), pulmonary edema(12.5%), pulmonary thromboembolism(6.25%), pulmonary hemorrhage(6.25%), H1N1 pneumonia(6.25%), Seizures due to CNS vasculitis (18.75%) and cerebral hemorrhage leading to hemiparesis(6.25%) refractory hypertension(12.5%), cardiac tamponade with obstructive shock and severe pulmonary hypertension(6.25%) were the reason for PICU admission. Immunotherapy and supportive therapy given as per unit Protocol. Out of 14 children, 4 succumbed to the complications. Rest 10 are under follow up with medications.

Conclusion: we observed that mortality was highest among younger age group and children presenting late to PICU with complication.


  PC0167: Predictors of early atherosclerosis in children with juvenile idiopathic arthritis Top


Sneha Agarwala, Suparna Guha, Priyankar Pal, S R Pal; VIMS and ICH, Kolkata, West Bengal, India

Introduction: Multiple amenable risk factors affect the initiation of atherosclerosis.Autoimmune diseases like lupus and vasculitis are known risk factors. This study wasundertaken to determine if there is any statistical association between juvenile idiopathic arthritis (JIA) and increased risk of atherosclerosis.

Methods: This is a combined data (serum HDL level, serum cholesterol level and carotid intima-media thickness) of two case control study, conducted independently, comprising of 75 diagnosed cases of JIA, which includes 3 new cases who were diagnosed and followed up since 6 months and rest of the cases were old cases on DMARDs at the time of recruitment, and 50 age and sex matched controls, attending the Pediatric Rheumatology Unit, Institute of Child Health, Kolkata and Vivekananda Institute of Medical Sciences, Kolkata during February 2018 – August 2019.

Results: We found significantly higher serum levels of total cholesterol (p <<< 0.05) and lower serum levels of HDL (p <<< 0.05) as compared to controls. Difference of mean carotid intima-media thickness (CIMT) between cases & controls was significant (p << 0.05). CIMT (mean) of JIA patients was 0.4966. In controls, the mean CIMT (mean) was 0.3774.

Conclusion: Clinical consequences of the atherosclerotic process, in the form of ischemicheart disease, disorders of cerebral circulation, or circulatory disorders of peripheral arteries occur in the adult population, however atherosclerotic changes have their beginning in childhood. With the advent of increasingly effective drugs for treating chronic inflammatory diseases, we may also consider broadening our strategy for early aggressive management of associated co-morbidities. In this study we find convincing evidence for early onset of atherosclerosis in JIA but further follow up studies with extensive population research are needed for validation in pediatric population.


  PC0168: Case series of patients presenting with peripheral ulcerative keratitis at a tertiary care hospital in Western India Top


Prashant Chotalia, Dhaiwatshukla, Rutwiz Mistry, Sapan Pandya, Puja Srivastava; SMT NHL Municipal Medical College and its Affiliated Hospitals, Ahmedabad, Gujarat, India

Objective: To study profile of patients presenting with peripheral ulcerative keratitis (PUKs) to our OPD.

Methods: Patients referred to with Peripheral Ulcerative keratitis (Idiopathic or associated with connective tissue diseases) were included. Their demographic, clinical, serologic al and treatment details were evaluated. Follow up data wherever available was also analyzed.

Results: Three out of 13 were male. Median age was 62 (IQR-40-65). Mean duration of illness was 1.63 ±1.9 years. Demographics and other features in table.

Seven out of 12 were RF, 3 were ANA and 3 were ANCA positive.

Nine patients received Injectable CYC with oral corticosteroids, 2 received Oral Methotrexate and oral corticosteroids. 1 patient received only oral corticosteroids.

Improvement in vision after treatment was recorded in 4 patients, 1 had persistent low vision despite treatment with CYC and Corticosteroids. One patient lost vision in both eyes. Four patients had more than 70% global improvement.

Conclusion: While most cases of PUK were idiopathic, they responded to immunosuppression as it is believed to be an autoimmune manifestation. More follow up data is needed to ascertain which drugs would be best in controlling this disease.
Table 1

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  PC0169: Efficacy of Vitamin E in methotrexate induced transaminitis in rheumatoid arthritis: A prospective randomized open-label case-control study Top


Binit Vaidya, Manisha Bhochhibhoya, Shweta Nakarmi; National Center for Rheumatic Diseases, Kathmandu, Nepal

Background: Derange aminotransferases are one of the commonest adverse reaction seen in management of RA with Methotrexate (MTX). Vitamin E was effective in the prevention of hepatotoxicity induced by MTX in the animal.

Objective: To examine the efficacy of vitamin E in MTX induced transaminitis in RA patients.

Methods: A prospective study conducted at a tertiary center for 6 months. RA patients on MTX treatment with deranged aminotransferases <3 fold were included. Patients with previous liver diseases, alcohol intake, muscle diseases, under hepatotoxic drugs and aminotransferases >3 times UNL were excluded. Simple randomization technique used to divide patients with altered LFT treated weekly with oral MTX (7-20mg/week) into treatment (Vitamin E 400mg bid for 3 months) and control group (no vitamin E). Follow up was after 3 months. Statistical analysis done using SPSS. Paired t-test and student t-test were done to compare mean differences. Ethical approval was obtained from Nepal Health Research Center.

Results: Total of 174 cases had increase aminotransferases, 87.5% were female, housewives (69.8%) and the mean BMI was 25.4±4. Out of 174, 80 patients showed deranged aminotransferases under MTX at a dose of 20mg/week. In the treatment group (88), SGPT (IU/L) and SGOT (IU/L) at baseline were 93.8±12.5 and 67.2±8.9 respectively and at follow up 39.4±5.2 and 26±3.5 respectively. There was a significant decrease in the level of aminotransferases (p value < 0.001). In the control group (86), SGPT and SGOT at baseline were 63.0 ± 2.3 and 46.5±1.8 respectively, at follow up 55.7±6.6 and 44.1±4 respectively with p-value >0.569. In subsequent follow-up, 29 patients were given vitamin E, SGPT decreased from 113 to 43.3 and SGOT from 76.3 to 29.3 (p value < 0.05).

Conclusion: Vitamin E significantly attenuates the MTX-induced hepatotoxicity resulting in a decrease in major adverse effect encountered during the treatment of RA.


  PC0170: Clinical profile of neuropsychiatric manifestations in lupus in South India Top


Benzeeta Pinto, C S Sumatha, Ramya Janardana, Kodishala Chanakya, Vineeta Shobha; Department of Clinical Immunology and Rheumatology, St. John's Medical College, Bengaluru, Karnataka, India

Introduction: Neuropsychiatric manifestations (NPSLE) are one of the least understood aspects of SLE. There are very few reports of NPSLE from India.

Objective: To study the prevalence of different NPSLE manifestations and in our cohort and to compare clinical and immunological features of NPSLE with non NPSLE patients.

Methods: This was a retrospective study in a tertiary care referral centre. All patients of SLE diagnosed in our centre for the last 5 years with neuropsychiatric manifestations as per the ACR definitions were included in the study. Consecutive patients of SLE over last 2 years were taken as controls. The clinical and immunological features were compared with controls without NPSLE.

Results: Ninety patients (85 females) with NPSLE and 137 (130 females) consecutive controls were included in the study. All patients fulfilled the SLICC criteria for SLE. Mean age at presentation of NPSLE patients was 29.31±11.84 years [Table 1]. The commonest NPSLE manifestation was seizures followed by cerebrovascular accident. Twenty nine patients had more than one NPSLE manifestation. Antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibody) was tested in 82/90 patients and was positive in 22 (26.83%). APLA positivity was associated with cerebrovascular disease (p 0.021) and cognitive dysfunction (p 0.018). The other NPSLE syndromes were similar in APLA positive and negative patients. As compared to controls, patients with NPSLE had lower prevalence of mucocutaneous and musculoskeletal manifestations. There was no difference found in anti-ribosomal P, lupus anticoagulant and anticardiolipin antibodies between the two groups. Magnetic resonance imaging was available in 45 patients of which 40 were abnormal. Ischemic changes were the commonest followed by nonspecific T2/FLAIR hyperintensities.
Table 1: Clinical and immunological features of NPSLE versus control

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Conclusion: Seizures and CVA are the commonest NPSLE syndromes in our cohort. The immunological profile was similar in patients with and without NPSLE.


  PM0001: HIV presented with rheumatological diseases Top


Fatma Fayed, May Morsy, Marwa Elkhalifa; Department of Rheumatology and Immunology, Alexandria University, Alexandria, Egypt

Background: Human immunodeficiency virus (HIV) infection is pandemic nowadays, more than 35 million people are infected with HIV, with two-thirds being resident in Africa.[1] The incidence of rheumatic manifestations in HIV infection was reported in about 4 to 71.3% cases in different studies depending on the stage of the disease and musculoskeletal involvements. [2,3]

Objective: The aim of these study to scope the light on HIV associated rheumatic syndromes. Rheumatic disease was a misdiagnosis or not?

Methods: Cross sectional study of patients admitted to rheumatology unit in Alexandria University with a previous diagnosis of autoimmune rheumatic diseases, resistant to treatment, were screened for HIV.

Results: Three patients found to be HIV positive with low CD4+ less than 200 cells mm3 among 130 screened patients. Two patients were diagnosed as Bechet disease due to recurrent oral and genital ulcers. The first one, also had recurrent skin infections associated with bilateral anterior and posterior uveitis. The second one admitted by recurrent oral and genital ulcerations associated with severe oral candidiasis, arthritis, erythema nodosum and positive pathergy test. The third one diagnosed as peripheral spondyloarthritis admitted with low-grade fever, palmoplantar psoriasis as well as acute extensive anterior and posterior uveitis in left eye and chronic anterior and posterior uveitis in right eye with CMV positive.

Conclusion: HIV infection might be misdiagnosed as a rheumatic disease. It is important to screen patients with inflammatory autoimmune rheumatic manifestations for HIV infection for its implications in the diagnosis and management.


  PM0002: Adult onset still's disease with lymphadenopathy mimicking lymphoblastic lymphoma: A case report Top


S Sivansuthan, Narani Aravinthan, M Prieyanka Krishanthi; THJ, Jaffna, Sri Lanka

A 21 years old female patient presented with high grade fever with constitutional symptoms and inflammatory arthritis which last for 4 weeks.

On examination, she was febrile, tachypneic and had features of active, symmetrical, inflammatory arthritis involving large and small joints of hands. Moreover, she had multiple right sided cervical lymphadenopathy.

Her complete blood count showed neutrophilic leukocytosis and anemia (Hb%-8.6g/dl, WBC-20,000/mm3, with-80%neutrophils, 15%lymphocytes). Her inflammatory markers were high (ESR -128 mm/1sthr, CRP 281mg/dl). Her serum ferritin was >1000 and LDH was 1202 U/l. Her Contrast Enhanced Computer Tomography of neck, chest, abdomen and pelvis revealed generalized lymphadenopathy involving right side cervical, bilateral axillary, right side external iliac and inguinal areas. Right cervical lymph node biopsy revealed a high-grade Lymphoma morphology favor for a diagnosis of lymphoblastic lymphoma. However, her Immunohistochemistry study for right cervical lymph node revealed negative CD-20, Nuclear TDT. Bone marrow biopsy revealed no evidence of infiltration and immunohistochemistry study of bone marrow biopsy was not suggestive of Lymphoma. Meanwhile, she was initially treated with non-steroidal anti-inflammatory drugs and has been treated with high dose of systemic steroids later. She responded quickly to steroid treatment and completely resolved within a few days. She has been followed up at rheumatology clinic.

Discussion: Adult Onset Still's Disease is a rare systemic inflammatory condition. Inaddition toclinical features, histological findings of lymph node biopsy may also mimic lymphoma and some authors reported scattered cases leading to confusion with malignant T-cell lymphoma.[3] Immunohistochemistry isimportant in the diagnosis of AOSD in which histologicalfindings, if taken alone, might be misleading.[4]

Conclusion: In a patient with lymphadenopathy, joint involvement and constitutional symptoms eventhe lymph node biopsy shows the features of T-cell lymphoma we need to consider Adult onset ofStills Disease and which should be confirmed with immunohistochemistry.


  PM0003: Rituximab induced lung injury in a young girl with juvenile dermatomyositis: A clinicopathological conference Top


Ankur Kumar Jindal, Mayur Parkhi1, Kirti Gupta1, Gargi Das, Anju Gupta, Amit Rawat, Amanjit Bal1, Dharmagat Bhattarai, Ashim Das1, Surjit Singh Department of Pediatrics, Advanced Pediatrics Centre, Allergy Immunology Unit,1Department of Histopathology, PGIMER, Chandigarh, India

Case Report:

A 13-year-old girl was apparently well till the age of 11 years when she was diagnosed to have juvenile dermatomyositis in view of progressive proximal muscle weakness, heliotrope rash, Gottron papules, elevated muscle enzymes and suggestive magnetic resonance imaging (MRI) changes. She was treated with corticosteroids, intravenous immunoglobulin, intravenous cyclophosphamide, and mycophenolate mofetil. In view of the chronic relapsing course, she was treated with rituximab at 13 years of age. Two weeks after receiving the second dose of rituximab, she presented with a history of cough and fever. At presentation, she was noted to have crepitations in the chest. Laboratory investigations revealed persistent anemia, normal leukocyte counts with lymphopenia and normal platelet counts. She had absent CD20+ B cells on flow cytometry but immunoglobulins (IgG, IgM and IgA) were within normal range. Initial chest X-ray showed a non-homogeneous opacity in the right lower lobe). Subsequent X-rays showed increasing infiltrates in bilateral lung fields and pneumothorax. A clinical possibility of pneumonia with sepsis with multiorgan dysfunction or rituximab induced lung injury was considered. She succumbed to her illness after one month of hospital stay and a partial autopsy was carried out. Histology of lungs revealed changes of diffuse alveolar damage (organizing phase) in the form of hyaline membrane formation, type II pneumocyte hyperplasia, prominent intra-acinar arteries and inter- and intra-alveolar organization. No organism could be identified. The findings were consistent with a diagnosis of rituximab induced lung injury.

Discussion: Rituximab induced lung injury has mostly been reported in adult patients following treatment of malignancies and is much less common in patients with rheumatological disorders.

Conclusion: Rituximab is increasingly being used in various rheumatological disorders and one must always be aware of its side effects and potentially life-threatening complications.


  PM0004: Complete heart block: A rare presentation in rheumatoid arthritis Top


Ganganpalli Dattaprasad, V P Pandey, Sanjay Dubey; Department of Medicine, MGM, Medical College, Indore, Madhya Pradesh, India

Rheumatoid arthritis is a chronic inflammatory disease of unknown etiology characterized by inflammatory polyarthritis with systemic involvement. Cardiac manifestations involve pericardium, cardiomyopathy and rarely conduction defects. Here we report a rare presentation of Rheumatoid arthritis as complete heart block which has incidence of <0.1%.
Figure 1: ECG suggestive of complete heart block

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Table 1: Investigation details

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Case Report: A 44 year male with no significant past and family history presented with syncope, palpitations and exertional dyspnea of 1-month duration. Pain and swelling of multiple small joints of hands for 5 months.

On examination, pulse rate was 35 beats/minute, BP was 110/70 mm of hg, respiratory rate was 16 cycles/minute. No abnormality detected on cardiovascular and respiratory system examination. Left 2nd MCP and PIP joints were swollen and inflamed. ECG showed complete heart block. Treated with Hydroxychloroquine, methotrexate and anti-inflammatory drugs. Permanent pacemaker was inserted and symptoms improved.

Discussion: Cardiac manifestations in Rheumatoid arthritis include cardiomyopathy, pericardial effusion, valvular involvement, coronary artery disease, and conduction defects. Conduction block manifests as RBBB, hemiblocks or AV blocks of variable degree like Complete heart block which is extremely rare.

Mechanisms involved are 1) granulomatous invasion and fibrosis of conduction system,2) vasculitis of the arterial supply of conduction system,3) hemorrhage into the rheumatoid nodule, 4)inflammatory lesion extension from the aortic or mitral valve, 5) amyloid deposition.

Conclusion: Rheumatoid arthritis involving the conduction system is atypical presentation and manifestation as complete heart block is extremely rare with an incidence of <0.1%. only a few case reports are reported worldwide.


  PM0005: Case of Bechet's syndrome with large longitudinal esophageal ulcers, bilateral sacroiliitis and oral, scrotal and penile ulcers Top


Varghese Koshy, Amit Kumar; Command Hospital (Central Command), Lucknow, Uttar Pradesh, India

Case Report: Patient is a 40-year, male who initially became symptomatic in March 2019 with fever, oral and penile and scrotal ulcers.He also developed dysphagia to both solids and liquids in May 2019.His UGIE revealed 2 large longitudinal ulcers in the esophagus.Biopsy of his scrotal ulcer revealed neutrophilic vasculitis.

MRI of his L-S Spine & SI joints revealed bilateral Sacroiliitis.

Patient showed an excellent response to Inj. Methylprednisolone pulse 1gm OD for 3 days followed by tapering oral corticosteroids and Tab Methotrexate.

There was complete resolution of his oral, penile , scrotal and esophageal ulcers.

Discussion: Esophageal ulcers are uncommon in BD and since their first description by Brodie and Ochsner in 1973, less than 50 cases have been reported worldwide till date.(1) Esophageal lesions include ulcerations, fistulae, strictures and varices. Esophageal ulcerations can be single or multiple and are often associated with ulcerations elsewhere in the GI tract. Rare gastrointestinal manifestations include portal vein thrombosis and Budd Chiari syndrome. (2) Sacroiliitis is an uncommon manifestation (7.5% cases in one study) (3). The presence of both Sacroiliitis and esophageal ulceration in the same patient has possibly never been reported.

Conclusion: Since Bechet's Disease is a clinical diagnosis, a high index of suspicion is a prerequisite to diagnosing the condition and reporting of cases with its varied and myriad manifestations.

References

  1. Yi SW, Cheon JH, Kim JH, Lee SK, Kim TI, Lee YC, et al. The prevalence and clinical characteristics of esophageal involvement in patients with Behçet's disease: a single center experience in Korea. J Korean Med Sci 2009;24:52-6.
  2. Bayraktar Y, Ozaslan E, Van Thiel DH. Gastrointestinal manifestations of Behcet's disease. J Clin Gastroenterol 2000;30:144-54.
  3. Ambrose NL, Haskard DO. Differential diagnosis and management of Behçet syndrome. Nat Rev Rheumatol 2013;9:79-89.
Figure 1

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  PM0006: Arthritis in sarcoidosis: Clinical profile and outcome of 11 patients from Nepal Top


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

Background: Sarcoidosis is a Multi-system Disease of unknown etiology. Ten to 15 of Patients with Sarcoidosis have associated Arthritis. Aim of this Article is to Delineate Articular Manifestation & Clinical Profile of Sarcoidosis from Nepal.

Methods: All Cases Records of 11 Adult Patients with Sarcoidosis between 2011 & 2019 were Prospectively Reviewed. Joint Involvement was assessed Clinically, Classified as Acute or Chronic respectively. All Patients were Follow Up every 2-3 weeks and Monitored Joints Involvement and Clinical Profiles. Their Treatment and Outcome were Recorded.

Results: Joints Involvement at the Time of Diagnosis was 81.81%. The pattern of Joint Involvement Revealed the Ankle and Wrist to be the most Commonly Affected in Both the Groups. Shoulder, Elbow, Metacarpophalangeal, Proximal Interphalangeal Joints of Hands and Knee Joint Involvement were Significantly more Common in Chronic Sarcoid Arthritis. Other Clinical Features were Fever 83%, Uveitis 36.36%, Peripheral Lymphadenopathy 54.54%, Difficulty in Breathing 45.45%, Skin Lesion (Erythema Nodosum) 54.54%, Hepatosplenomegaly, Weight Loss and Arthralgia. Imaging Findings included: Hilar Adenopathy 36.36%, Pulmonary Infiltrate 45.45%.

Four out of 11 Patients with Acute Oligoarthritis followed over a Median of 2.5 Years had achieved complete Remission. Five patients of Chronic Sarcoid Arthritis associated with Pulmonary Infiltration and Skin Lesion had been Treated with Steroid, Methotrexate and Hydroxychloroquine Respectively. All Patients has assessed by Resolution of Clinical Symptoms & Improvement in Erythrocyte Sedimentation Rate, C-Reactive Protein, Angiotensin-Converting Enzyme Levels and Spirometry Parameters.

Conclusion: Adult Sarcoidosis with Predominant Arthritis seems to Respond well to Systemic Steroid &Methotrexate. The Ankle Joints being the Most Commonly affected in Acute Oligoarthritis Patterns. Knee & Shoulder Joint Involvement were more common in Chronic Sarcoid Arthritis. Uveitis, Peripheral Lymphadenopathy and Skin Lesion were more common in Both Acute and Chronic Sarcoid Arthritis. Delay Diagnosis, Ocular and Pulmonary Involvement are Probably associated with Poor Outcome.


  PM0007: Idiopathic C1 esterase inhibitor deficiency: A rare cause of acquired angioedema Top


Sanjana R Badami, Sudhir Mehta, R K Bhimwal; SMS Medical College, Jaipur, Rajasthan, India

C1 esterase inhibitor deficiency (C1INH-AAE) is a rare self-limited disease of recurrent episodes of angioedema without urticaria. Idiopathic cause accounts to less than 10 percent of C1INH-AAE. A forty-year-old male presented with self-limiting recurrent episodes sudden onset of facial puffiness, difficulty in breathing and pain abdomen occurring at an interval of three to four months for last 1.5 years without any precipitating etiology lasting for 48 to 72 hours. There was no history of fever, rashes, pruritus, personal or family history for allergic disorders and no exposure to environmental allergens or any drug allergy. Routine investigations were negative. Lymphoproliferative disorders, B cell malignancies, autoimmune disorders were ruled out. Low serum complement C4 levels, c1q and C1INH levels established the diagnosis of C1 Esterase inhibitor deficiency. No treatment was initiated as symptoms recovered spontaneously. C1 INH- AAE should be considered in a patient who presents with isolated angioedema (without urticaria) in the fourth decade of life or later without family history of angioedema. All patients with acquired angioedema should be evaluated for an underlying B cell lymphoproliferative disorder at the time of diagnosis and repeated annually.
Figure 1

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  PM0008: A tetrad of fever, pain abdomen, diarrhea and anasarca- unraveling the enigma Top


Arka Bairagya, Kaushik Basu, Anupam Sarkar, Debarup Das; Medical College and Hospital, Kolkata, West Bengal, India

Case Report: 31 years old female presented with fever, loose stools and abdominal pain for 1 month along with gradually progressive bilateral pedal swelling followed by abdominal swelling for 3 weeks. Fever was associated with myalgia, arthralgia and colicky abdominal pain; poorly localized and non-radiating. She also complained of vomiting and loose stools, 3-4 times per day. Stool contained only mucus and no blood. On examination there were mild pallor, bipedal pitting edema, moderate ascites and left sided pleural effusion. Investigations revealed anemia, moderate proteinuria, hypoalbuminemia, high fecal calprotectin and normal CRP. Infective etiologies ruled out from ascitic fluid study, urine culture, blood culture and hemogram. CECT abdomen showed small intestinal wall (jejunal) edema, thickening, target sign and comb sign. UGI endoscopy: nonspecific duodenitis. Colonoscopy: nothing significant. ANA (hep2) 4+ coarse speckled (1:160), 3+ anti Sm, low C3 and C4, high anti ds-DNA. Other causes of chronic diarrhea were ruled out. Finally a diagnosis of lupus enteritis was made. Inj. methylprednisolone 1 gm i.v. was given for 3 days followed by tab prednisolone 1mg/kg/day and inj. Cyclophosphamide 1gm i.v. The patient started gradually improving after 1 week.

Discussion: Lupus enteritis is a rare complication of SLE. Patients with SLE can present any time before or after diagnosis of SLE. If diagnosis is delayed intestinal necrosis and perforation can occur. One should rule out other common and infective causes of pain abdomen first. No CT feature is specific for lupus enteritis. Biopsy is usually not required for confirmation but can be done to exclude other possibilities. Intravenous steroid is an effective therapy.

Conclusion: Lupus enteritis is a poorly known cause of abdominal pain in SLE patients with distinct clinical features and therapeutic outcome. High index of suspicion is required to make a timely diagnosis in order to prevent further complications.


  PM0009: Not so uncommon cause of nephropathy in a case of Spondyloarthropathy Top


Paras Kathuria; VMMC and Safdarjung Hospital, New Delhi, India

Case Report: I am presenting a case of a 35-year-old male who presented to us with complaints of low backache for 3 months which used to get increased on resting along with early morning stiffness. His inflammatory markers were raised. On further evaluation he was found to be having bilateral sacroilitis with HLA B27 positivity and a diagnosis of spondyloarthropathy was made. On reviewing old records patient had history of reduced urine output along and pedal edema 1 year back with deranged KFT (62/1.5). Urine examination revealed significant proteinuria and hematuria with active sediments. Kidney biopsy showed IgA nephropathy. He was managed with ACE Inhibitors and steroids to which he responded. His KFT and urine examination were normal when he presented to us. So a diagnosis of Spondyloarthropathy with Ig A nephropathy was made and patient was started on NSAIDs to which he partially responded, we could not start TNF inhibitors in this patient due to financial constraints.

Discussion: Renal abnormalities in ankylosing spondylitis is seen in approximately 5-8% of cases, with etiologies such as amyloidosis being most common followed by drug induced. IgA nephropathy being the third most common cause with prevalence ranging from 20-30% of all causes of renal abnormalities. Most of the cases described in literature were diagnosed in established case of ankylosing spondylitis, however in this case Ig A nephropathy preceded the onset of ankylosing spondylitis which is very rare.

Conclusion:

  1. Renal evaluation including urinalysis should be done in all cases of spondyloarthropathyand if found abnormal should be worked up for the cause.
  2. Ig A nephropathy is not an uncommon cause of nephropathy in rheumatological disorder and if present should be appropriately managed.
  3. Ig A nephropathy may even be present before the onset of spondyloarthropathy.



  PM00010: Adult onset still disease – An uncommon etiology of nonresolving pneumonia: A rare case entity Top


Anil Kumar Behera, Sarat C V Talluri, K Bchetanreddy; Care Hospital, Banjara Hills, Hyderabad, Telangana, India

Introduction: Adult onset stills disease is a rare systemic inflammatory disorder of unknown etiology and pathogenesis with high spiking fever accompanied by several systemic manifestation. A few reports exist in medical literatureexplaining pulmonary manifestation of(AOSD).one rare case entity of(AOSD) with non-resolving pneumonia is being reported.

Case Report: A 33-year-old male, known case of pulmonary tuberculosis and TB lymphadenitis presented with high spiking fever, dry cough, dyspnea and pleuritic chest pain. On examination, patient was tachypneic, hypoxic, with bilateral basal crepitations.hemogram showed leukocytosis, high erythrocyte sedimentation rate and c- reactive protein. Chest imaging showed bilateral mild pleural effusion with mediastinal lymph nodes. Pleural fluid analysis showed transudate, sputum culture negative and other laboratory investigation. Procalcitonin and autoimmune workup was negative. Suspecting stills disease, serum ferritin was very high. Patientdeveloped polyarthritis and transaminitis. Patient did notrespond to any antibiotics. Patient was diagnosed to have AOSD using yamaguchicriteria. Patient was started with naproxen, hydroxychloroquine and IV steroids. Patient improved symptomatically and followed up with oral steroids.

Conclusion: AOSD is an uncommon immunological disorder of unknown etiology with recognized increased frequency, diagnosed by yamaguchicriteria. Infectious diseases, malignancy and rheumatic disorders must be kept in mind as an exclusion for those patients. In our patient, diagnosis of AOSD was made using yamaguchi criteria with following clinical and laboratory values like spiking fever, arthralgia, leukocytosis and elevated levels of aminotransferase and high serum ferritin.


  PM0011: Antineutrophil cytoplasmic antibody-associated vasculitis and systemic lupus erythematosus overlap syndrome: Case report Top


Lapsiwala Mehul, Patel Krunal, Goel Anshul1; Shalby Multi-Specialty Hospital, Surat, Gujarat,1Vivekanand Medical Institute, Palampur, Himachal Pradesh, India

Case Report: We report case of 50yrs(F), presented with fever, arthritis, maculo-papular rash, photo sensitive rash, hematuria, cough. On evaluation, ANA-1:320 (homogenous), anti-dsDNA++, S.Creatinine-2.3 mg7dL, CRP-110mg/L, B/L non-homogenous opacities in lung-fields on X-ray, Urine routine: Proteinuria 3+, >100 RBCs/hpf and casts, protein-creatinine ratio 3.97, ↓S.C3(60mg/dl), c-ANCA-1:40, MPO-positive(>200U/ml). CT-chest: B/L sub-centimetric nodules, tree-in-bud appearance, scattered consolidation patches, multiple enlarged hilar, mediastinal LNs. Kidney biopsy: focal necrotizing glomerulonephritis, cellular crescents and glomerular thrombosis, interstitial inflammation without immunofluorescent deposits. The diagnosis of SLE with AAV was based on clinical, serological, histo-pathological and CT scan abnormalities according to the ACR criteria. She received non-invasive ventilation, 3-pulses prednisolone and supportive therapy. Once stabilized, she received cyclophosphamide 1000mg monthly for 6 months with tapering prednisolone dose. Follow-up 6 months CT-chest showed resolved abnormalities, urinalysis and urine protein/creatinine ratio-negative for proteinuria/hematuria. She is asymptomatic for 9-months.

Discussion: SLE and AAV share clinical features, like arthritis, cutaneous lesions, renal involvement. Patients meeting classification criteria for both SLE/AAV are categorized as SLE-AAV overlap syndrome. They are predominantly women. In these cases, renal histopathology distinguishes AAV from SLE vasculitis. ANCA prevalence is 31% in lupus patients and vasculitis prevalence in SLE is 11-36%. Lupus nephritis is immune complex-mediated glomerulonephritis. Whereas, renal involvement in AAV is pauci-immune crescentic glomerulonephritis. However, there are LN patients (class-IV-segmental, class-III) who develop extensive segmental necrosis, crescent formation without subendothelial immune deposit but have positive P-ANCA+MPO antibodies. They resemble AAV glomerulonephritis. In SLE/AAV syndrome reported so far, renal pathology is classified either LN or pauci-immune GN according to the predominant type lesion. Majority (75–80%) have mesangial deposits and interstitial inflammation. Our patient had pure AAV nephritis.

Conclusion: This case suggests, SLE-AAV overlap syndrome should be considered in differential diagnosis of subjects with LN or AAV, renal biopsy should be considered in any AAV or LN patients.
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  PM0012: Case of SLE presenting as SJS-TEN overlap Top


Varghese Koshy, Asmita Sinha, Rahul Rai, Aditya Bakshi, Pooja Devi; Command Hospital (Central Command), Lucknow, Uttar Pradesh, India

Patient is a 62-year, female, who initially became symptomatic in April 2019 with fever, and loss of appetite. There is history of consuming alternative systems of Medicine, temporally, following which she developed extensive generalized maculopapular rash with oral mucosal ulcerations and subsequently bullae formation and degloving of skin. She was initially considered to be a a case of SJS-TEN overlap with DILI (Drug induced Liver injury) and Pancytopenia and polyserositis.

She was managed on the 1st admission in April/May 2019 with IVIg followed by oral corticosteroids. She recovered well. Her Serology was suggestive of SLE ( ANA by IIF +ve Homogenous, Anti dsDNA +ve, Anti Sm +ve, Anti Nucleosome +++, Anti Histone +ve, SSA +ve) with decreased C4 level.She developed a recurrence of SJS-TEN overlap in June 2019 and was then managed with Pulse dose of Inj Methylprednisolone 1gm OD x 3 days and was also started on Tab MMF. She was administered 2 doses of Inj Rituximab 15 days apart.

Discussion: Although SJS and TEN are almost invariably due to medications, they may, rarely, be an initial presentation of lupus.

Conclusion: SLE presenting as SJS-TEN overlap is rare and the management of such a patient is challenging requiring a team approach and b decision making.
Figure 1

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References

  1. Lee HY. SLE presenting as SJS & TEN: A report of 3 cases. Lupus 2011;20:647-52.



  PM0013: H syndrome: A novel genodermatosis mimicking IgG4 related disease Top


Benzeeta Pinto, Gautham Arunachal1, Rahul Sethia2, Ashish Aggarwal2, Vineet Ahuja2; Department of Clinical Immunology and Rheumatology, St. John's National Academy of Health Sciences,1Department of Human Genetics, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka,2Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India

Case Report: 24-year-old male was symptomatic for the last five years with abdominal pain and loose stools alternating with constipation. He noticed thickening and darkening of skin on both his lower limbs and dryness of mouth and eyes with caries of multiple teeth. Hyperpigmentation and induration was noted on both lower limbs extending till lower abdomen prominent on inner thighs and sparing both the knees [Figure 1]. A firm mass was noted in the pelvic region. Colonoscopy revealed superficial ulcers with mild luminal narrowing in the distal ascending colon with dense inflammatory infiltrate on biopsy. PET CT revealed FDG avid multiple parenchymal opacities in both upper lobes and sheet like soft tissue thickening with mild FDG uptake involving thoracic aorta, retroperitoneum and pelvic fascia with skin and subcutaneous involvement in inguinal and thigh regions. FDG uptake was also noted in the hepatic flexure with thickening of the wall. Double inferior vena cava was seen. Biopsy of pelvic mass revealed extensive fibrosis. Immunostaining for IgG4 was negative. IgG4 levels were within normal limits. A diagnosis of H syndrome was considered in view of characteristic cutaneous findings. Clinical exome sequencing revealed a previously reported pathogenic variant c.1330G>T [p. E444Ter; HGMD ID- CM093097] in homozygous state in SLC29A3 confirming the clinical diagnosis of H syndrome.1 He is started on tocilizumab with symptomatic response.
Figure 1

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Discussion: H syndrome is a rare autosomal recessive disease characterized by cutaneous hyperpigmentation, induration and hypertrichosis along with numerous systemic manifestations The clinical manifestations are heterogenous and include short stature, diabetes mellitus, hypogonadism, flexion contractures and heart anomalies. Only 10 cases are reported from India.

Conclusion: Tocilizumab may be an effective treatment for H syndrome.


  PM0014: An unusual case of gouty arthritis in a young woman with pregnancy Top


Lubna Khurshid, Suraj Chaudhary, Sanjiv Kapoor, Anand N Malaviya; Department of Rheumatology, ISIC Superspeciality Hospital, New Delhi, India

Case Report: A 39-year-old woman presented with acute inflammatory arthritis of the right knee in her 8TH week of pregnancy. She had a history of episodic paraparesis 20 years ago. Initially treated as polymyositis with high dose of steroids. A few months later she was correctly diagnosed as hypokalemic periodic paralysis and treated with fluids and potassium supplements. 3 years later she developed episodic intermittent pain and swelling involving both knees and both the shoulders. She was diagnosed as inflammatory polyarthritis and treated with DMARDs with some relief. However she had a flare of right knee arthritis in March 2019. Synovial fluid analysis showed monosodium urate (MSU) crystals. Serum uric acid (SUA) was 9.8mg/dL. She was managed with allopurinol, colchicine and glucocorticoid (GC). She improved but had another flare of arthritis during pregnancy. Allopurinol was discontinued and she was maintained only on colchicine and GC.

Investigations: ESR 109 mm 1st hr, Hb-9.8 gm/dL, WBC 15000/cmm, platelets 3, 85, 000/cmm. KFT and LFT were normal, SUA 9.8mg/dL, Synovial fluid from the right knee joint showed negatively birefringent MSU crystals under polarized light microscopy. Test for the enzyme HPRT and PRPP was not available; could not be done.

Discussion: Gout is rarely diagnosed in young, premenopausal women. Its protective effect is attributed to estrogen therefore if found in a young woman, secondary causes of hyperuricemia should be investigated (Table 1). Diagnosis is based upon clinical findings and demonstration of MSU crystals in synovial fluid. Treatment should be targeted to keep SUA < 5 mg/dL.

Conclusion: Asymmetrical seronegative polyarthritis with acute intermittent attacks should be considered to be due to gout even in persons in young age group and carefully investigated as it is a treatable condition. Due to its rarity in young woman its treatment in pregnancy remains a challenge.


  PM0015: An elderly patient of Takayasu Arteritis presented with hypertensive heart failure Top


Bharat Kumar, Rohit Mathur, Sonu Pandit, S Veena, N I Galib Mirza, Mahendra Kumar Garg; All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

Introduction: Takayasu arteritis or pulse-less disease is a rare chronic inflammatory large vessel vasculitis involves primarily aorta and its branches; affecting the females in the second and third decade. The main pathological changes in vessel wall are thickening, fibrosis, thrombosis, stenosis, formation of aneurysm and these changes are due to cellular immunity against the endothelial cell. Manifestations range from asymptomatic pulseless patient to catastrophic neurological or renal involvement. Lack of specific treatment and delayed diagnosis causes high morbidity and mortality of the disease.
Figure 1

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Case Report: A 59 years old female from Barmer, Rajasthan came to emergency with acute shortness of breath of 2 hours duration. She also complained of headache, decreased vision and decreased urine output for 7 days. On examination, the patient was conscious, disoriented and had findings suggestive of heart failure. Patient left radial &brachial pulse was absent and had blood pressure of 240/160 mmHg which was treated as hypertensive emergency with NTG and labetalol infusion in A & E. Patient was further investigated. MRI brain with angiography shows posterior reversible encephalopathy syndrome (PRES), complete occlusion of left external carotid and 3rd part of subclavian artery and saccular pseudo-aneurysm of left ICA. Renal Doppler suggests bilateral renal artery stenosis. Fundoscopy suggestive of grade 2 Retinopathy (Takayasu retinopathy). All the findings were consistent with Takayasu arteritis complicated by renal, neurological, eye and cardiac involvement. Steroids were started and blood pressure was maintained with antihypertensive. Despite optimal treatment, the patient had progressive renal failure which leads to mortality.

Conclusion: Takayasu arteritis is rare in the elderly but it can be considered in a female with malaise, fever, pain abdomen, and hypertension with asymmetrical or absent pulses. Though it rapidly involves multiple organs and can be devastating but early diagnosis and prompt treatment with steroids can prevent further complications and mortality.


  PM0016: A patient with swollen hands Top


Lubna Khurshid, Suraj Chaudhary, Sanjiv Kapoor, Anand N Malaviya; Department of Rheumatology, ISIC Superspeciality Hospital, New Delhi, India

Case Report: 75 years man presented with Swelling of both the hands x 4 months. He was being managed as carpel tunnel syndrome by an orthopaedician without relief.On examination the hands were grossly swollen (dorsal and planter aspect) [Figure 1]. Additionally, there was marked restriction of cervical, dorsal and lumbar spinal movement in all planes. There was past history of CAD, episodic monoarthritis treated as gouty arthritis intermittently, and tubercular lymphadenopathy – taken ATT for 2 years 18 years ago.
Figure 1: Swelling of hands

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Investigations: ESR 33 mm 1st hr., Normal hemogram. The X Ray of the cervical and dorsolumbar spine showed loss of lumbar lordosis, marked syndesmophytes and grade 3 sacroiliitis respectively [Figure 2]. HLA B27 test was negative. USG hands suggested synovial hypertrophy at radiocarpal joints with chronic tenosynovitis and subcutaneous tissue hypertrophy of bilateral hands and wrists.

He was managed as a case with axial and peripheral SpA associated with CAD, treated with methotrexate, sulphasalazine, and NSAIDs. If he does not respond, TNFi may be considered.

Discussion: SpA as a group are a common rheumatic disease with a prevalence of 0.5-1.9% making them at least as common as rheumatoid arthritis. SpA is a condition with broad spectrum of clinical manifestations, laboratory abnormalities and imaging features. As a result, it is difficult to recognize all of its protean manifestations in clinical practice. The pessimistic scenario of a late case has changed dramatically since the advent of TNFi drugs.The early and correct diagnosis, although still remains a challenge, is important with the availability of a number of highly effective drugs.
Figure 2: Radigraphs showing Grade 4 sacriiliitis and lumbar lordosis, syndesmopytes and possible compression fracture with anterior wedging of L1 vertebra

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  PM0017: Monoarthritis with skin rash in a middle aged male: An unusual case Top


Suraj Chaudhari, Lubna Khurshid, Sanjiv Kapoor, Anand N Malaviya; Department of Rheumatology, ISIC Superspeciality Hospital, Vasant Kunj, New Delhi, India

A 45 year old man with 6 years of T2DM presented with mildly itchy rash over the trunk and scattered areas over arms. The lesions were erythematous, well defined, slightly scaly papules and plaques, with hypopigmented borders in some lesions. He had swelling below his ears and on the lateral side of his neck since 2018. There was right elbow swelling, pain worst in last 3-4 days treated with aspiration and IAS in another hospital, without relief. He consulted a dermatologist; a skin biopsy was performed (reported as sarcoidosis). He had bilateral parotid and cervical lymph node enlargement and broad nodular nose. The examination of the other systems was unremarkable.

Investigations:

ESR 50 mm 1sthr, CRP 156, Hb10.70gm/dl, WBC 5500 /cmm, Platelets18800/cmm, ACE levels 60 u/l, USG abdomen - hepatosplenomegaly & retroperitoneal lymphadenopathy. CECT chest – lymphadenopathy without necrosis suggesting sarcoidosis.

Final Diagnosis: Extra-pulmonary sarcoidosis with skin lesions and right elbow monoarthritis.

Treatment prescribed: methotrexate 20 mg weekly, hydroxychloroquine 400 mg/day and methylprednisolone 4 mg daily.
Figure 1: Before treatment (a) Lupus perino (b) Trunk of the patient erythematousslightly scaly papules and plaques over the trunk. After treatment (a) A significant decrease in lupus perino (b) A significant decrease in plaques and papules over the trunk

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Follow-up: after 4 weeks of treatment joint symptoms had disappeared with marked clearing of the skin lesions.

Discussion: Sarcoidosis is a chronic multisystem disease of unknown etiology mostly involving lungs, but frequently shows extra-pulmonary manifestations that might be difficult to recognize and treat.

Cutaneous lesion includes erythema nodosum, subcutaneous nodules, maculopapular eruptions, lupus Perino. In MSK involvement any type of arthritis may occur though monoarthritic, as seen in our patient, is uncommon. Acute form of sarcoidosis with bilateral hilar lymphadenopathy and ankle periarthritis with EN is called Lofgren's syndrome.

Conclusion: In the absence of any RCTs for treating sarcoidosis, treatment is based upon the experience of the clinicians who have treated large number of patients. Glucocorticoids and hydroxychloroquine with methotrexate and TNF-alpha inhibitors as steroid-sparing agents are used with satisfactory Results:


  PM0018: Nocardia brain abscess mimicking tuberculoma in a systemic lupus erythematosus patient Top


Mukta Wyawahare, M Nidhish Chandra; Department of Medicine, JIPMER, Puducherry, India

Background: Patients on immunosuppression for Systemic Lupus Erythematosus (SLE) are prone for atypical infections. Here we present a case of Nocardia farcinia brain abscess in an SLE patient on immunosupression who presented with altered sensorium.

Case Details: A 41-yearfemale, housewife,a known case of Systemic Lupus Erythematosus (SLE) on Prednisolone 30 mg once daily, Hydroxychloroquine 200 mg once daily, Methotrexate 10 mg weekly for the past 8 months now presented to us with altered sensorium.Initial MRI showed Multifocal areas of T2 iso to mild hypointense signals in right cerebellum, left temporal periventricular region, right occipital lobe that showed diffusion restriction with ring enhancement. Mild perifocal edema and diffuse leptomeningeal enhancement were also present. MRS showed elevated lactate peak and choline to creatinine ratio>1 suggestive of tuberculosis. Her sensorium worsened despite starting ATT and an external ventricular drain was placed in view of hydrocephalus. CSF-gram stain revealed gram positive, filamentous organism and subculture grew Nocardia farcinia identified by MALDI TOF.Brain heart infusion blood agar showed buff coloured dry, cerebriform colonies. and Modified Ziehl Nielsen filamentous, branching staining demonstrated thin, partially acid-fast bacteria in a Background of many polymorphonuclear leukocytes suggestive of Nocardia. After nocardia was isolated in culture, intravenous trimethoprim-sulfamethoxazole (15mg/kg of TMP divided in three doses), intravenousamikacin (7.5mg/kg every 12 hours) and intravenous meropenem (500 mg every 6 hours) was started. Later patient developed Extraventricular drain related shunt infection during which Acinetobacter was isolated from CSF. Patient developed external ventricular drain infection and septic shock and expired after 2 months of treatment.

Discussion and Conclusion: In an immunocompromised patient presenting with multiple ring enhancing lesion in the brain, we have to think of atypical infections like nocardia as differentials especially when patient is not responding to routine treatment.


  PM0019: A rare case of Behcet's disease misdiagnosed as rheumatoid arthritis/Hansen's disease Top


BaikunthaPanigrahi, Bidyut Kumar Das, Saumya Ranjan Tripathy, Manoj Kumar Parida; S.C.B.Medical College, Cuttack, Odisha, India

Case Report: A 38-year old male was on treatment (methotrexate and sulfasalazine) for RA since 2008. In 2012, he developed episode of choroiditis and left forearm panniculitis (biopsy-proven) which was managed with systemic steroids. Since 2016, he has suffered from recurrent, multiple ulcers over bilateral lower limbs. In 2018, he developed bilateral ulnar claw hand. Although, split skin smear was negative, he was started on MDT for Hansen's by local physician based on tender and thick bilateral ulnar nerves. He was referred to Rheumatology for further evaluation. On examination, he had non-healing ulcers over dorsum of left foot, right 1st, 2nd and 5th toes. Bilateral wrists were tender and swollen. There was a superficial tortuous vein over abdomen with flow downward from epigastrium to umbilicus. There was a dermatomal loss of sensation along left sural nerve, bilateral ulnar claw hands. CBC, RFT, LFT, and urine R/M were normal. ESR:69 mm 1st hour, and CRP:17mg/l were raised. RF, ACPA, ANA and ANCA were negative. NCS: sensory motor axonal polyneuropathy. Doppler ultrasound revealed left sapheno-femoral incompetence. Biopsy of ulcer-margin revealed leucocytoclastic-vasculitis. Sural nerve-biopsy revealed vasculitis and was AFB-negative. CT-angiography revealed right brachiocephalic and SVC thrombosis. APLA was negative, Factor V Leiden mutation absent and serum homocysteine was normal. Pathergy test was negative. However, HLA-B51 was positive. He was started on oral prednisolone (1mg/kg) and azathioprine and has shown marked improvement.

Discussion: The patient had arthritis, vasculitic ulcer, neuropathy and venous thrombosis with history of choroiditis [score=4 as per ICBD-criteria, 2014]. The diagnosis of RA-vasculitis is unlikely, as RF and ACPA are negative. Nerve biopsy was AFB-negative and venous thrombosis does not occur in Hansen's. Although Pathergy is negative, HLA-B51 supports diagnosis of Behcet's disease.

Conclusion: Presence of venous thrombosis is an important clue to diagnosis of Behcet's in a setting of vasculitis.
Figure 1

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  PM0020: Multifocal cutaneous tubercular abscess in a SLE patient presenting as MAS Top


Siddhartha Satapathy, Lavina Patnaik, Rina Tripathy1, Manoj K Parida, Saumya R Tripathy, Bidyut K Das; SCB Medical College,1SVPPGIP, Cuttack, Odisha, India

Case Report: A 18 year old female who was diagnosed case of SLE nephritis mucocutaneous for 1.5 years with history of MAS on corticosteroid presented with fever and multiple swelling over right elbow, medial and lateral aspect of foot. The swellings were tender, fluctuant with purulent discharge.

Laboratory investigations revealed normal complete blood count with elevated ESR and CRP and low C4, normal LFT and RFT.FNAC of the nodule showed inflammatory lesion. Chest radiograph was normal. Pus from the site of lesion showed acid fast bacilli and MTB was detected on CBNAAT with sensitivity to rifampicin. Based on these observations, a diagnosis of multifocal tubercular abscess was made in this SLE patient and cat I ATT was started and is on follow up.

Discussion: There is a high prevalence of tuberculosis in SLE which can be attributed to multiple immune abnormalities and related to immunosuppressive therapy. Extrapulmonary involvement is more frequent than pulmonary in SLE.

Conclusion: The possibility of tuberculosis must be kept in mind in case of subcutaneous abscess and nodules non responsive to antibiotic therapy and drainage. Awareness of unusual presentation of tuberculosis is important for early diagnosis and proper management.


  PM0021: An unusual presentation of multifocal musculoskeletal tuberculosis Top


Lavina Patnaik, Siddhartha Satapathy, Manoj K Parida, Saumya R Tripathy, Bidyut K Das; SCB Medical College, Cuttack, Odisha, India

Case Report: A 35-year-old man presented with 6 months history of swelling of right wrist and left ankle, and 3 months history of pain over thoracolumbar spine without any significant accompanying symptoms of fever, weight loss, loss of appetite or night sweats. Examination revealed kyphoscoliosis, swelling over left ankle and right wrist, hyperreflexia with bilateral flexor plantar response and no sensory or motor deficit. Laboratory reports revealed normal complete blood count with high ESR and CRP, elevated urea (51mg/dl) and creatinine (2.4mg/dl) and normal LFT; chest radiograph showed homogenous opacity adjacent to right hilum and HRCT thorax showed multiple paravertebral abscess. Ultrasonography of abdomen and pelvis showed features of chronic medical renal disease. MRI of right wrist and left ankle showed features of chronic inflammatory arthropathy possibly due to Koch's and MRI spine revealed disseminated spinal tuberculosis with bony erosions at C6, C7, D4, D5, D6, D7, D8, D9, D10, L5 &S1 and paravertebral abscess extending from C6-D12 and L5-S1. Synovial fluid examination from left ankle showed acid fast bacilli. HIV ELISA was non-reactive. Based on these observations, a diagnosis of multifocal skeletal tuberculosis was established and patient was started on Cat I anti-TB chemotherapy and is on follow-up.

Discussion: Extrapulmonary tuberculosis accounts for 15%-20% of all cases of tuberculosis, with skeletal tuberculosis comprising 15% cases of extrapulmonary TB.

The present study is rarest of rare case of non-contiguous multifocal osteoarticular tuberculosis in a healthy 35-year-old male with negative HIV status which should be considered in patients with long standing pain and deformity especially in endemic countries like India to avoid delay in diagnosis.

Conclusion: PMultifocal extensive spinal tuberculosis with tubercular arthritis with chronic kidney disease without any constitutional symptoms is rare and can mimic other benign or neoplastic etiology.


  PM0022: An uncommon case of SLE with cirrhosis and hypersplenism Top


Kishore Kunal, Singh Jasjit, Manoj Kumar, Samandeep Singh; Command Hospital Western Command, Panchkula, Haryana, India

Background: The association of systemic lupus erythematosus (SLE) with gastrointestinal autoimmune diseases is rare, but has been described in the literature, mostly as case reports. We describe here a case of SLE who presented with cirrhosis of liver with hypersplenism leading to life threatening refractory pancytopenia.

Case Report: Our patient is a 30-year-old male, who was admitted with history of high grade,intermittent fever of 15 days duration associated with episodic epistaxis and easy bruisability. Clinically he had pallor with subcentimetric cervical lymphadenopathy, multiple ecchymotic lesions on extremities and moderate splenomegaly. Lab investigations revealed pancytopenia, transaminitis, positive direct Coombs test and polyclonal hypergammaglobulinemia. His infective screen was negative. CECT of abdomen revealed splenomegaly with ascites and bilateral renal infarcts His immunological workup revealed positive ANA ( 4+ speckled, 1:640), ds DNA positivity (ELISA), low complements and Anti-SS-A, SS-B, U1RNP on ENA. His viral markers (HbsAg/Anti-HCV), ASMA/Anti-LKM/AMA was negative. His workup for APS was negative and bone marrow studies was suggestive of trilineage cellularity with megakaryoticthrobocytopenia. He also underwent transjugular liver biopsy and UGI endoscopy. His liver biopsy sample was suggestive of cirrhosis and had mild portal hypertensive gastropathy on endoscopy. He was managed as a case of SLE with secondary cirrhosis of liver and pancytopenia with IV methylprednisolone, IVIG and oral steroids. He had poor response to therapy and continued to have severe neutropenia (ANC – 700/uL). He was also given Inj GM- CSF and Eltrombopag but with no favorable outcome. He finally underwent splenectomy with gradual recovery of cytopenias.

Conclusion: Autoimmune GI manifestations is common in patients of SLE, but rarely serious and life threatening. This case report highlights the rare association of cirrhosis of liver with SLE and hypersplenism leading to life threatening refractory pancytopenia with favorable outcome after splenectomy.


  PM0023: Pseudo-obstruction in SLE: Our experience Top


Sandeep Yadav, C Balakrishnan, Rohini Samant; PD Hinduja Hospital and Research Center, Mumbai, Maharashtra, India

Introduction: Gastrointestinal involvement (GI) involvement is reported to occur in 50 % of patients with systemic lupus erythematosus (SLE) (1). Systemic reviews of literature have yielded not more than 42 cases of Intestinal Pseudo-obstruction (IPO) in SLE (2). We present our experience with 9 patients of SLE with IPO and discuss their epidemiology, clinical features, and clinical outcome.

All 9 patients were female with median age at diagnosis of 28 years. Most common presenting symptoms were abdominal pain, nausea, vomiting and diarrhea. Seven out of nine patient had chronic diarrhea while none had constipation. Median duration of delay in diagnosis was 6 months. Five of the seven patients misdiagnosed at the time of presentation and underwent avertible laparotomies. Ileum was most commonly involved (9/9) followed by duodenum (3/9), stomach (3/9) and large intestine (2/9). Supportively, colonic biopsy was negative in 7 out of 9 patients. All patients were ANA positive with ds-DNA being positive in 6/7.

CT abdomen was done in all cases showing diffuse circumferential wall thickening of the involved bowel part with dilatation with mucosal edema while 2 patients had concurrent uretero-hydronephrosis. 6 /7 patient had associated hematological and renal involvement. Available histopathology typically showed non-specific inflammation and atrophy of the muscularis layer.

All patients were treated with pulse methylprednisolone followed by oral prednisolone. Five patients had concurrent lupus nephritis, of whom three received cyclophosphamide and two received Mycophenolate. All patients had favorable response to treatment with resolution of GI symptoms maximum by six weeks.

Conclusion: Intestinal pseudo-obstruction is a rare, enigmatic manifestation of SLE. Ileal involvement is typical. Dilated thickened bowel loop is seen on imaging. Histopathology shows non-specific inflammation with atrophy of muscularis layer. High clinical suspicion and typical radiology may help in preventing a laparotomy. Response to steroids and steroid sparing agents is usually good.


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  PM0024: Atypical persistent skin eruption and a rare disease journey: Difficult to diagnosis and difficult to treat: Stills her life Top


Biswajit Banik, Debojyoti Ray; R.G.Kar Medical College, Kolkata, West Bengal, India

Adult onset still's disease is a rare peculiar rheumatological febrile illness with persistent fever; rash and arthritis.A 29 yrs female patient presented with persistent fever, persistent pruritic erythematous maculo-papular rash for 10 days; with associated symmetrical polyarthritis mainly in upper limbs joints.
Figure 1: Persistend pruiti c morbiliform maculo-papular skin eruption

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Figure 2: Haemophaghocytes in bone marrow

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After admission in our hospital, after battery of tests, reveled; persistent leukocytosis, high serum triglycerides, mild splenomegaly, multiple lymphadenopathy, high ESR; all routine; infective panel shows, negative result.

After considering, history and routine tests, blood for serum ferritin level and skin biopsy was done. Surprisingly, ferritin 12700 ng/ml; skin biopsy- from lesions, shows, dyskeratotic cells in the epidermis with inflammatory infiltrate in superficial dermis.

RA FACTOR, ANTI CCP, ANA (HEP 2) CELL LINE, ANCA negative.

Neoplastic screening: negative.

Bone marrow: normal study.

Treated with oral prednisolone and NSAIDs. With diagnosis of AOSD (adult onset still's disease).

After 2 months, with treatment, patients, complaining of, persistent fever, arthritis with respiratory distress. Routine investigation revealedpancytopenia, hepatosplenomegaly bilateral fluffy opacities, ferritin 22000 ng/ml; keeping in mind macrophage activity syndrome (MAS); all infected focus are screened, and bone marrow was done.

Tests revealed, hemophagocytes in bone marrow and dengue IgM was reactive; dengue PCR also shows same result. We started pulse methyl prednisolone, intravenous immunoglobulin; for 2 days; further deteriorating, the clinical profile, we started plasmapheresis and prednisolone; after 7 days, patient response to treatment, and discharged with tab cyclophosphamide, tab prednisolone, tab sulfasalazine, tab methotrexate and tab folic acid.

Discussion: AOSD is a rare systemic infection; the diagnosis made by exclusions after possible infective, neoplastic etiology rule out; without typical skin lesions it causes a huge diagnostic diplomacy.

Conclusion: Atypical skin eruption in still's disease causes diagnostic problem with treatment the course may not favorable; can transforms into MAS; dengue can precipitate the course.


  PM0025: Idiopathic necrotizing autoimmune myopathy Top


Chanaveerappa Bammigatti, Deepanjali Surendran; JIPMER, Puducherry, India

Background: We present a case of idiopathic necrotizing autoimmune myopathy, a rare entity in inflammatory myopathy.

Case Report: 32-year-old male, a ward assistant in local hospital from Kanchipuram occasional alcoholic with no comorbidities presented with c/o difficulty in getting up from the sitting and squatting position for 20 days.Later noticed swelling in bilateral forearm and calf region with upper and lower limb body pain. He felt difficulty in lifting the hand above the head and difficulty in combing hairs.no obvious muscle weakness,cranial nerve involvement, skin lesions and joint pain.no h/o drug /native medication intake.O/E power in bilateral shoulder, hip and knee joint is 4-/5 with 80% hand grip. Diminished DTR's noted. Lab investigations shown creatine kinase of 16,628 IU/L(markedly elevated),otherwise normal RFT,LFT and TFT. EMG shown positive sharp waves with fibrillatory potentials,s/o myopathic process.We proceeded with muscle biopsy(left vastus lateralis) and started on prednisolone 1mg/kg/day. Later muscle biopsy reported as necrotizing autoimmune myopathy.HPE shown early necrotic and regenerating fiber [Figure 1]. Other investigations such as ANA is negative, myoblot for anti-SRP is negative and anti-HMGCR was not done.CECT chest and abdomen is normal.Later patient is added on azathioprine 50mg BD.
Figure 1

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Discussion and Conclusion: Immune mediated necrotizing myopathy, new and a rare variant present as acute onset, symmetrical, proximal more than distal weakness.Difficult to treat than DM/PM.Correct diagnosis and aggressive therapy is required.


  PM0026: p-ANCA positivity in NMO-SD patient: Is vasculitis associated with NMO-SD Top


Sumit Kumar Vishwakarma, V P Pandey, Archana Verma; Department of Medicine, MGMMC, Indore, Madhya Pradesh, India

We are presenting 2 cases of NMO- SD with p –ANCA positivity. In about 40% cases, NMO – SD is associated with vasculitis. It gives new horizon of management in NMO -SD patients.

1st case-45-year female presented with recurrent history of paresis;

1st episode as right hemiparesis in 2008; MRI Brain -left mid brain, pons and dorsal cord hyperintensity.

2nd episode as paraparesis in 2011; MRI Brain and Spine - Bilateral Centrum semi ovale , C3-C4 and D4 -12 -T 2 HYPERINTENSITY.

VEP-prolonged

CSF-OCB, NMO-Aquaporin -4, MOG-Negative

ANA And p-ANCA-Positive

C-ANCA-negative

On the basis of criteria of NMO –SD, she was diagnosed as same.

2nd case-17-year girl presented with recurrent episode of paraparesis and seizure since 2011.

MRI Spine (2011) - Diffuse T2 hyperintense signal involving C1 to conus.

MRI Brain (2014)– Multiple new T 2 ,FLAIR hyperintensity in pons, midbrain ,right thalamus and peri and supraventricular white mater, splenium of corpus callosum and bilateral internal capsule.

MRI Brain - (2018) –New onset lesion in similar areas.

CSF-OCB -Negative

VEP- B/L Prolonged

CSF-Aquaporin 4 positive.

ANA and p-ANCA-Positive

Discussion: NMO-SD is associated with vasculitis in 40% cases, like SLE, Sjogren's syndrome and p-ANCA associated vasculitis. In our 2 patient we got p- ANCA positivity without any other clinical manifestation.

Among the CNS demyelinating disorders higher occurrence of ANCA in patient with NMO- SD than in MS, ANCA is a potential marker of autoimmunity in CNS demyelinating disorders.

We treated both the patient with Rituximab and they showed substantial clinical recovery.

Conclusion: We present 2 cases of NMO- SD with p-ANCA positivity which is not much reported in literature .These patients are in close follow up to see for clinical manifestations of p –ANCA associated clinical symptoms.


  PM0027: PUO and persistent disease activity in a child with SLE- time to think of coexisting evils Top


Prem Kumar, Sarala Premkumar, Mahesh Janarthanan; Department of Rheumatology, Division of Pediatric Rheumatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Background: SLE in children requires aggressive treatment to keep the disease under control. But the low immunity state can lead to serious infections.

Objective: We describe a 12 year old girl who presented with persistent fever and disease activity for a year. On investigation she was found to have features of military TB on chest X-ray, granulomas in the spleen and Growth of mycobacterium tuberculosis from the bone marrow and biochemical and lab features of macrophage activation syndrome.

Methods: 12-year-old developmentally normal female child, a known case of SLE with Lupus nephritis was admitted in SRMC with complaints of intermittent fever for one year and significant weight loss in August 2019. Child was diagnosed to have SLE with Lupus nephritis in 2016 at a private hospital and started on steroids but drug compliance is poor. History of Ayurveda medication intake for 2 years. On admission her vitals were stable and clinical examination showed hepatosplenomegaly and hyperpigmented macules over face and abdomen with significant muscle wasting. With a provisional diagnosis of PUO, child was treated with i.v antibiotics and supportive measures. CBC showed pancytopenia and ESR elevated. LFT was minimally deranged. Ferritin was elevated. Blood and urine cultures done showed no growth and hence i.v antibiotics stopped. Chest X-ray done showed reticulonodular opacities in both lung fields. CECT Chest done showed features of Miliary tuberculosis. Sputum smears showed AFB. Bone marrow aspirate culture showed growth of Mycobacterium tuberculosis. TB QuantiFERON test done was positive and ultrasound showed features of splenic granuloma. During the course of stay, child developed fall in hemoglobin, platelets and leucocytes with persistent fever spikes and elevated ferritin levels. Macrophage activation syndrome and disseminated TB were identified as the underlying problems and the child was treated with IVIG and methyl prednisolone and ATT. Fever spikes gradually came down and child showed clinical improvement.

Conclusion: Tuberculosis must be considered in children with SLE on significant immune suppression and persistent fevers.


  PM0028: Wegeners granulomatosis associated with unilateral facial nerve palsy- A rare case presentation Top


Abhishek Agrawal, V P Pandey, Sanjay Dubey; M.G.M. Medical College and M.Y. Hospital, Indore, Madhya Pradesh, India

Case Report: A 55 year old non diabetic non hypertensive female comes with complain of deviation of angle of mouth to the right side and difficulty in closing left eye since 5 days.

  • She has history of recurrent upper respiratory tract infection with rhinorrhea (3-4episode per month) since last 2 years and history of hemoptysis 2-3 episode in last one year
  • No history of fever, weight loss, contact with patient of tuberculosis, hyposthetic patch over body, skin rash, local trauma, or any other chronic illness like DM or hypertension.


On Examination-

  • Pulse- 86/min, BP- 124/80 mmHg, SpO2- 98% on room air, respiratory rate- 18/min, temperature- 98.7 F
  • Neurological examination revealed lower motor neuron type of left 7th nerve palsy. the rest of neurological and other systemic examination was normal
  • Otoscopy examination- normal.


Laboratory Investigations-

  • Haemoglobin-9.9 g/dL, TLC- 7000/Cumm, platelet-2.8 lacs/Cumm, Peripheral smear- normocytic normochromic picture
  • ESR- 78mm
  • S. creatinin-0.79mg/dL, s. urea-32mg/dL
  • S.total bilirubin 0.20mg/dL, s. protein-6.5mg/dL, s. albumin- 4.0 m/dl, lipid profile- normal
  • URIN R/M- 8-10 RBC/hpf
  • HIV/HBSAg/HCV- negative
  • C-ANCA- positive (>100 U/ml)
  • RA FACTOR/ ANA- Negative
  • C3 & C4 level- normal
  • Sputum Afb- Negative
  • X-RAY CHEST-Small nodules present in left lung, wedge shaped opacity in middle zone of right lung
  • CT CHEST- Suggests- small nodular opacity in left lung parenchyma
  • MRI BRAIN- No abnormality detected.


Patient refused for any biopsy.

Patient was managed initially with pulses of daily iv methylprednisolone and followed by iv cyclophosphamide. we also put her on azathioprine.

Discussion: Our patient presents with lower motor neuron type facial nerve palsy and after ruling out other causes of facial nerve palsy we explore the history of patient and reached the final diagnosis of wegner granulomatosis.

Conclusion: Wegener's disease can present with facial nerve palsy also so always look for features of this disease for timely diagnosis.


  PM0029: Familial hypercholesterolemia with arthritis Top


Venkatesh Yellapu, Prasanta Padhan, Sakir Ahmed; Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India

Familial Hypercholesterolemia is a disorder of lipoprotein metabolism characterized by elevated cholesterol, low density lipoprotein cholesterol, xanthomas and early onset atherosclerosis. Arthritis and tendinitis were rare in familial hypercholesterolemia. So there were often confused with reactive arthritis and other causes of polyarthritis .Here we present a case of young boy who was diagnosed as probable hypercholesterolemia with hypercholesteremic arthritis.

Report of the Case: 19 year old male came with complaint of swelling of joints of hand and feet since 8 years. He had e yellowish hard nodules all over the body since birth which were increasing in size with age. On examination there was boggy swellings in multiple joints of hand and foot and yellow nodules on elbows,knee and on buttocks. Lab investigations revealed serum cholestrol-504mg/dl,serum HDL cholesterol 25mg/dl serum LDL cholesterol 42mg/dl.On Punch biopsy of lesion were found to bexanthomas. Radiograph showed cystic and erosive lesions of metacarpophalangealjoints.

Conclusion: Metabolic disorders are often encountered in clinical practiceof these disorders are associated with musculoskeletal and dermatological manifestations. Familial hypercholesterolemia can give rise to variosmusculo skeletal disorders such as mono,oligo,polyarthritis and migratory arthiritis.Therefore it is important to recognise the association between musculoskeletal manifestations and hyperlipidemia for diagnostic and therapeutic purposes.


  PM0030: Autoimmune pancreatitis in IgG4 syndrome with Type 1 diabetes mellitus: A rare case presentation Top


Abhishek Agrawal, Abhimanyu Nigam, V P Pandey, Sanjay Dubey;

M.G.M. Medical College and M.Y. Hospital, Indore, Madhya Pradesh, India

Case Report: A middle aged nonalcoholic and non-hypertensive female comes with complain of-

  • abdominal pain since 1 week
  • vomiting since 4 days
  • no past history of DM/HTN or any chronic illness
  • No significant family history


On examination-

Pulse- 82/min, BP-126/80 mmHg, SpO2-98% on RA, RR- 18/min.

Per abdomen-Epigastric tenderness present, no guarding and rigidity per abdomen.

Mild pallor and pedal edema present.

Reddish brown rash over both cheeks, nose and forehead with sparing of nasolabial fold.

rest systemic examination was normal.

Laboratory Investigations-

  • Hb- 8.3 gm/dL, TLC-3200/Cumm, platelet- 1lac/Cumm, ESR- 38/mm, RBS- 216 mg/dL, FBS-164mg/dL, PPBS- 258mg/dL HbA1C-5.6
  • S. amylase- 807U/L, S. Lipase- 695 U/L
  • RFT, LFT & Lipid profile- NORMAL
  • T3-1.38ng/mL, T4-7.93microgram/dL, TSH-12.12 microIU/mL, AntiTPO- Positive.
  • ANA- Positive
  • Anti-Ribosomal antibodies- Positive
  • USG abdomen- heterogenous pancreatic echotexture with mild ascites suggestive of Acute pancreatitis
  • Urine R/M- proteinuria- (2+), 24-hour urinary protein- 550mg/24 hour so a renal biopsy planned. Renal biopsy suggests-lupus nephritis class II
  • C-peptide- 0.4ng/mL (0.5-2ng/mL) suggests type 1 DM
  • IgG4 level-3.8g/L (0.03-2.0g/L).


Discussion: Autoimmune pancreatitis (AIP) is a distinct form that can present with nonspecific symptoms like abdominal pain, vomiting and jaundice. type 1 AIP is associated with multiorgan involvement named IgG4 related disease. Association with other autoimmune diseases is common in type 1 Autoimmune Pancreatitis.

On the basis of above findings patient diagnosed as a case of-

Autoimmune Pancreatitis with type 1 DM- IgG4 disease,

SLE with Lupus nephritis and Autoimmune Hypothyroidism

Patient was treated with prednisolone, subcutaneous insulin, cyclophosphamide pulse therapy, levothyroxine supplement.

Conclusion: Whenever patient presents with acute pancreatitis and Type 1 DM, with no underlying chronic pancreatic damage, one should suspect for autoimmune pancreatitis and IgG4 correlation is to be evaluated.autoimmune pancreatitis is not related to only involvement of exocrine functions, endocrine functions of pancreas should also be looked for.


  PM0031: Ankylosing spondylitis a rare cause of peripheral nephropathy Top


Nischal Modak, V P Pandey, Monika Porwal;

MGM Medical College, Navi Mumbai, Maharashtra, India

Objective: Presenting ANKOLYSING spondylitis as a rare presence of peripheral neuropathy.

Introduction:

Ankylosing Spondylitis (AS) is an inflammatory disorder of unknown etiology that primarily affects the axial skeleton; peripheral joints and extra articular structures are also frequently involved. The disease usually begins in the second or third decade; male to female ratio is 3:1. Common sites of joint involvement are sacroiliac joints, spine, ischial tuberosities and heels. The most common extra articular manifestation is anterior uveitis (40%). Peripheral neuropathy is a rare presentation.

Objective: Ankolysing spondylitis a rare cause of Peripheral nephropathy.

Material study- 28 year old Hindu, male nondiabetic non hypertensive ,labor by occupation presented with chief complaints of low back pain since 3 years ,B/L knee and ankle pain since 2 years and losing of slippers since 6 months and difficult to hold cup of tea since 6 months followed by difficulty in walking stairs followed by difficulty in combing hairs since 6 months. On examination Pain over sacroiliac region. Normal higher mental function normal cranial nerve. Motor system - Atrophy of all of all 4 limbs, power 3 / 5 in all 4 limbs with hypotonia in all 4 limbs .Planter refl2x mute and all reflex +1.sensory system glove and stocking sensory loss. With intact bowel and bladder.Schober test positive .On Inx Normocytic normochromic anemia. NCV polyneuropathy with axonal degeneration was raised with raised ESR,HLB27 positive.ANA positive with Normal B12,Toxic screen. Therefore, diagnosis of ankylosing spondylitis was made with peripheral neuropathy.


  PM0032: A child with recurrent and refractory angioedema Top


M Sabarinath, T N Tamilselvam, N Balakrishnan, Karthikeyan, N Sujatha, R Ramesh, S Mythili; Department of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

13/female child presented with history of recurrent episodes of swelling on face and erythematous rashes over palms, soles for the past 3 years. Child also had history of recurrent episodes of fever and multiple joint pain, 3 years past during the initial phase of her illness. There was no history of photosensitivity, oral ulcers, frothy urine and pedal edema. Child underwent evaluation multiple times in nearby hospital for facial swelling and managed with antihistamines, steroids with response to treatment

in 3-5 days. At 11 years of age child had acute abdomen pain and underwent emergency appendicectomy. On examination during present admission, child had angioedema involving periorbital region and bilateral cheeks [Figure 1]. Child also had purpura over palms and erythematous macules in pressure areas of clothing like waist line [Figure 2]. Her complete blood counts showed Hb 10.3gm/dl, TC 10500, PLT 2.5lakhs and with normal renal and liver function test. Her urine routine and PCR were within normal limits. Her ENA profile showed positive dsDNA and normal complements. Her C1 esterase inhibitor levels were normal. She was diagnosed as case of childhood lupus presenting with recurrent angioedema and cutaneous vasculitis. She was managed with antihistamines, steroids, HCQ and MMF. On follow up after four months of treatment, child had two episodes of angioedema with minimal response to antihistamines, steroids and subsiding completely with FFP transfusion [Figure 3]. We present this as a case of childhood lupus with predominant symptoms of angioedema and having poor response to immunosuppression. A literature review of SLE presenting with acquired angioedema was done. There were several case reports pointing to multifactorial etiology for angioedema in SLE with varying response to pulse steroids, antimalarial, azathioprine, danazol and plasmapheresis.
Figure 1: Angioedema treatment

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Figure 2: Lesions in waist line

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Figure 3: Angioedema after Rx with FFP

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  PM0033: Myositis as initial presentation of sarcoidosis: A rarity Top


Mayank Gupta, Neeeraj Jain, Lalit Duggal, Bhavya Chintala; Sir Gangaram Hospital, New Delhi, India

Case Report: A 39-year-old male patient consulted in Rheumatology OPD with chief complaints of localized swelling over left calf for 6 months. Swelling was more on inner side, associated with calf fullness, without raised local temperature and muscle weakness. On investigations, Hemogram, CPK, liver and kidney function tests were normal. Myositis profile was also done which was negative. MRI of left thigh muscles showed hyperintense signals predominantly in medial head of gastrocnemius. Chest X ray showed reticular opacities in Rt. middle and upper zone. Serum ACE level was normal, Mantoux and QuantiFERON gold test were negative. CECT Chest revealed symmetrical nodules in both lungs with multiple enlarged lymph nodes in hilar,paratracheal region.Muscle biopsy was done from calf muscle which showed intrafascicular and perifascicular epithelioid granulomas without caseous necrosis i.e. in favor of sarcoidosis. MRI of left thigh and calf muscle revealed hyperintense signals in multiple muscles predominantly in medial head of gastrocnemius with thin layer of fluid around muscles suggesting Infective? Inflammatory myositis. So, based on clinical history and investigations, diagnosis of Granulomatous Myositis due to Sarcoidosis. Prednisolone 40 mg daily was started with gradual tapering of dose along with Azathioprine 50 mg twice daily. Patient responded well to treatment with reduction in size of swelling and improvement of symptoms.Muscles are involved in up to 5 % of patients with <1 % are presented as initial musculoskeletal involvement. In our case, muscle involvement was the initial and only manifestation of sarcoidosis with lung affection being picked up on routine screening.

Conclusion: We must consider rare and atypical causes of focal myositis. Tissue biopsy is must for making a diagnosis. Rare presentation of rare disease should be kept in mind.


  PM0034: A case series of malignancies: Mimics of Rheumatological disorders Top


Jeet Patel, Lalit Duggal, Neeraj Jain, Mayank Gupta, Bhavya Chintala; Sir Gangaram Hospital, New Delhi, India

Background: Malignancy and Autoimmunity interplay is full of complexities. We here, describe few cases of malignancies presented as mimics of autoimmune disorders. A case of acute myeloid leukemia had leucocytoclastic vasculitis, positive ANA, positive SS-A, and few clinical features of autoimmune disease and it mimicked for UCTD. Second case of signet ring cell type of adenocarcinoma of G.I tract mimicked CTD as had ANA positivity and ILD. Third case was epithelioid sarcoma which mimicked pyomyositis while fourth case was multiple myeloma presented with leucocytoclastic vasculitis, positive ANA, malar rash and urinary sediments and it had mimicry of SLE. Malignancy can present as an autoimmune disease or it can mimic flare of Rheumatological diseases. Cancer can present as vasculitides, CTDs, myositis or arthritides. Sometimes, it is difficult to ascertain whether autoimmune disorders have caused cancers or malignancy has caused autoimmune disturbances. ANA can be positive in upto 27% cancer patients. There is clearly increased risk of cancers amongst patients of autoimmune diseases compared to general population. Malignancies including metastasis can also present with true paraneoplastic autoimmune disorders. They usually present later in the course of the disease but sometimes surprised internist with sudden appearance in early course of disease. High index of suspicion is required to detect malignancy in autoimmune diseases as both have invariably similar presentations. PET scan and tissue diagnosis are amongst the major investigations to treat patients with spectrum of malignancy and autoimmunity


 Chiari malformation More Details, syringomyelia and polyarticular charcot arthropathy: A rare entity">  PM0035: Chiari malformation, syringomyelia and polyarticular charcot arthropathy: A rare entity Top


Jeet Patel, VedChaturvedi, Mayank Gupta, Bhavya Chintala; Sir Gangaram Hospital, New Delhi, India

Charcot arthropathies or neuropathic arthropathies are a progressive form of destructive, generally painless arthropathies. Syringomyelia has 20 to 25% risk of neuropathic arthropathy. It usually involves upper limb joints, e.g. shoulder, elbow etc. it is usually monoarticular albeit we have described here a case of polyarticular Charcot arthropathy due to syringomyelia. It can be also associated with Chiari malformations in 25% cases, particularly type one Chiari malformations. Surgical correction is advisable for Chiari malformations. No definitive treatment is available for Charcot arthropathy developed due to syringomyelia. To our knowledge, this is a first case of Chiari malformation type one with syringomyelia presented with polyarticular erosive Charcot arthropathy.


  PM0036: Subcutaneous cysticercosis of hand mimicking tenosynovitis: A rarity Top


Mayank Gupta, VedChaturvedi, Bhavya Chintala; Sir Gangaram Hospital, New Delhi, India

Subcutaneous cysticercosis in human is an uncommon parasitic infection, mostly presents as asymptomatic subcutaneous nodules with prevalence of 12.9-38% in India. It may occur as an isolated feature or as part of the disseminated cysticercosis. Here with we report a 43 years female presenting with 10 duration subcutaneous swelling. Ultrasound of left wrist showed a 5×3-mm, well-defined, thin-walled, cystic lesion with an eccentric, echogenic focus measuring around 1.5 mm in diameter in the subcutaneous plane The hypoechoic area surrounding this cyst showed significant exudative fluid collection with. The adjacent soft tissues were thickened and irregular, suggestive of edema. There was no e/o internal vascularity on color Doppler imaging. The radiological diagnosis given was a typical subcutaneous cysticercosis. Magnetic resonance imaging (MRI) evaluation of the brain which was obtained to look for disseminated cysticercosis revealed normal studies. and left-hand MRI s/o-well defined cystic lesion in muscle plane near second meta-carpal measuring approx. 5×3-mm. Extensive surrounding myofascial soft tissue swelling, and edema seen.s/o –Cysticercosis.

  • Subcutaneous cysticercosis is a relatively rare form of cysticercosis but should always be considered during the evaluation of subcutaneous swellings or in suspected cases of tenosynovitis. Ultrasound is a valuable, safe, nonionizing, cost-effective, widely available imaging tool for diagnosis of subcutaneous cysticercosis.
  • There is a wide spectrum of ultrasound patterns of subcutaneous cysticercosis. In classic cases with a cyst containing a scolex within and with a surrounding edema, high resolution ultrasound should always be the primary mode of diagnosis, thus avoiding unnecessary fine needle aspiration cytologies.



  PM0037: Known case of male lupus presented with acute abdominal emergency Top


Prashant Bhanjibhai Dudhagara, Alakendu Ghosh; Department of Rheumatology, IPGMER, Kolkata, West Bengal, India

Background: Thrombotic Storm(TS) is as a rare, extreme, and often lethal clinical entity characterized by a series of thrombotic events which spread over a short span of time involving the arterial and venous circulatory beds in diverse and unusual sites. There are very few case reports of Thrombotic storm successfully managed. Kidney is the most common organ involved in thrombotic storm. Involvement of liver with liver infarct and TTP makes this case rare presentation of SLE.

Case Summary: 31 year old male had history of pain in finger tips and scrotal pain with lupus panniculitis. He had history of taking steroid with HCQ and aspirin, which he stopped on his own for 2 years. Now he presented with acute abdominal pain and chest pain. All surgical cause of abdominal pain was ruled out but he was found to have liver infarcts with raised ACLA and he then developed APS nephropathy with resistant HTN and hypertensive hear failure. He also developed TTP (MAHA, thrombocytopenia, ARF,fever) for which 5 cycles of PLEX was done. He was started immunosuppressant as he had high disease activity with no signs of infection. He was put on oral anticoagulation as he had documented liver infarct. There was drastic improvement after therapy. He was fulfilling criteria of thrombotic storm. After discharge, he was given NIH protocol- 6 doses of cyclophosphamide and his anticoagulation is being continued.

Conclusion: TTP with thrombotic storm is acute emergency in a case of SLE with CAPS. Delay in diagnosisand treatment is related to high morbidity and mortality. After successful correction of thrombotic storm, prognosis of patient of SLE with CAPS.


  PM0038: Burkholderia sepsis mimicking flare of ANCA associated vasculitis: A rare presentation Top


Mayank Gupta, Lalit Duggal, Neeeraj Jain, Bhavya Chintala; Sir Gangaram Hospital, New Delhi, India

Case Report: A 54 year old lady with diabetes mellitus and hypertension presented with complaints of slurring of speech and deviation of angle of mouth towards right since 6 days and weakness of left upper and lower limbs since 3 days. She was diagnosed as AAV two years back and started on Tablet Azathioprine and oral glucocorticoids (off treatment since 8 months). Patient had cutaneous panniculitis along with high p ANCA titer not responding to above mentioned treatment; Injection Rituximab 1 gm was given. Three days later after Rituximab, patient developed above mentioned complaints. MRI spine revealed paraspinal abscess. MRI brain showed hyperintense lesions in fronto-temporal lobe suggestive of Vasculitis. CECT Chest showed multiple nodular lesions more in favor of ANCA associated vasculitis. Blood culture, pus culture of brain lesions and paraspinal abscess showed Burkhaltercepacian. Brain biopsy also revealed Burkholderia infection. She was treated with intravenous Meropenam, ventilator and other supportive treatment. Even after all possible efforts, she succumbed.

Discussion: ANCA associated vasculitis is an autoimmune condition of inflammation of small blood vessels in various organs of human body. It is a multisystem disease with protean manifestations.  Burkholderia pseudomallei Scientific Name Search s a gram negative, bipolar, aerobic, motile, rod shaped bacterium. It causes an infectious disease called melioidosis. Risk factors are Diabetes Mellitus, Chronic Renal Failure, Chronic Lung Disease, excessive alcohol consumption. Immunosuppressive patients can develop acute bloodstream infection which usually.

Results: in septic shock. Neurological melioidosis is a very rare condition.CECT Chest s/o multiple, small, subpleural, randomly distributed, angiocentric, multiple cavitary and non-cavitary nodules in favour of ANCA associated vasculitis.

Conclusion: Burkholderia infection can complicate vasculitis course particularly in immunocompromised individuals. Early identification of organisms in tissue is the gold standard for diagnosis. Prompt treatment with Carbapenems and 3rd generation cephalosporins is a key to patient management.


  PM0039: Kikuchi disease with subsequent systemic lupus erythematosus: An uncommon case Top


Rashmi Bansal, Mohit Goyal1; Department of Medicine, S.M.S. Medical College, Jaipur1Department of Rheumatology, CARE Pain and Arthritis Centre, Udaipur, Rajasthan, India

Case Report: A 20-year-old female with hypothyroidism and type 1 diabetes presented with three-month history of high-grade fever and erythematosus, macular rash on upper limbs with axillary lymphadenopathy. Ultrasound revealed multiple enlarged lymph nodes with largest measuring 32x16 mm. Bloods revealed elevated ESR and CRP with negative ANA by immunofluorescence and no soluble nuclear antigens. Histopathology of biopsied lymph node revealed histiocytic necrotizing lymphadenitis suggestive of Kikuchi disease (KD). Patient improved with steroids and NSAIDs. 8 months later she presented with three-month history of hair fall, oral ulcers, joint pains, fever and erythematous rash on trunk and limbs. She had hepatosplenomegaly. ANA was positive at 1:1000 dilution with 4+ intensity and homogenous pattern on immunofluorescence. With diagnosis of systemic lupus erythematosus (SLE) she was put on tapering oral prednisolone with addition of azathioprine and hydroxychloroquine. After 5 months, patient continues to do well.

Discussion: KD is a rare, benign, immune mediated disorder characterized by cervical lymphadenopathy, fever, arthralgias and myalgias with predilection towards female gender and young age group. The disease infrequently coexists with or precedes SLE. Differential diagnoses of KD are lymphomas, lymphadenopathies associated with connective tissue disorders and bacterial or viral infections. These can be differentiated from KD on lymph node biopsy which shows paracortical foci, necrosis and histiocytic cellular infiltrate. Preservation of nodal architecture, polyclonal infiltrates and negative immunohistochemistry exclude lympho-proliferative malignancies and viral infections. The absence of neutrophils, presence of hematoxylin bodies and plasma cells with vasculitis are helpful in distinguishing SLE from KD.

Conclusion: Whilst it's important that the self-limiting KD is recognized, possibility of other diseases including SLE should be considered. Lack of markers to predict which patients progress to SLE means that patients diagnosed with KD require close follow up.
Figure 1

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  PM0040: Systemic lupus erythematosus associated with hereditary C1-inhibitor deficiency Top


Anuj Shukla, Priyanka Gaur; Niruj Rheumatology Clinic, Ahmedabad, Gujarat, India

Case Report: A 19-year old girl presented with the diagnosis of juvenile Systemic Lupus Erythematosus(SLE) with onset at age 11years. Her clinical features are mainly cutaneous, mild Raynaud's phenomenon, polyarthralgia and fever. Her 21-year old brother was also diagnosed with juvenile SLE at age 16years. He also had mainly cutaneous features but lately in last 6months had developed lupus nephritis.

Their 60-year old father is a known case of hereditary angioedema from age 6years. He has episodes of recurrent angioedema and acute abdomen pain. Son and daughter have no history of angioedema and father has no history of symptoms suggestive of SLE. Mother has neither of the two diseases.

Son and daughter were positive for Anti-Sm, RNP and Anti-Ro-60. Father's sera was negative for ANA and Anti-dsDNA(ELISA) was negative for all three. Serum complement C4 was low for all while son had also low C3-values. C1-esterase-inhibitor values were low for all three [Figure 1]. Next-Generation-Sequencing(NGS) showed a same frameshift mutation in the SERPING1 gene for both father and son.

Discussion: Here, we report a family with hereditary type-1 C1-inhibitor deficiency (decreased levels of C1-inhibitor) with an autosomal dominant inheritance. 23-cases of SLE associated with hereditary C1-inhibitor deficiency had been reported.[1] Defect in the inhibitor protein might lead to hyper-activation of the complement pathway, which may explain the clinical features of SLE. Both father and the son have same genetic mutation but different phenotype. This variation can be explained by some SLE-predisposing genetic polymorphisms in the early complement genes of the children inherited from the mother. Such polymorphisms is likely to be absent in father and thus protecting him from complement hyper-activity and SLE.

Conclusion: Thus, in addition to hereditary angioedema, hereditary C1-inhibitor deficiency can be associated with SLE in certain susceptible individuals.
Figure 1

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Reference

  1. Koide M, Shirahama S, Tokura Y, Takigawa M, Hayakawa M, Furukawa F. Lupus erythematosus associated with C1 inhibitor deficiency. J Dermatol 2002;29:503-7.



  PM0041: A case of ankylosing spondylitis with turbulent course Top


Arindam Nandy Roy, Yarram Ashok Kumar, Syeda Sana Fatima; Department of Rheumatology, Yashoda Hospital, Secunderabad, Telangana, India

Background: Hydroxychloroquine (HCQ) has shown benefits in treating rheumatic diseases such as SLE, RA and scleroderma. The risk of developing irreversible maculopathy and consequent vision loss is a possible serious complication with HCQ use. Modern day screening Methods: can detect retinopathy early in such patients.

Objective: Primary aim was to assess the prevalence of HCQ maculopathy in Indian patients with rheumatic diseases by modern day screening Methods. Secondary aim was to look for the risk factors of HCQ maculopathy.

Methods: This cross-sectional study was carried out in the Dept of Rheumatology, Yashoda Hospital, Secunderabad between July 2017 to March 2019. 984 subjects having different rheumatic diseases, who had used HCQ for 1 year and beyond and evaluated with at least Humphrey Visual Fields were included. Retinopathies other than HCQ were excluded. Informed consent was taken.

All data regarding Age, Gender, BMI, Diagnosis, Comorbidities, Daily dosage and Duration of HCQ use and Screening Methods: (Humphrey Visual Fields, Spectral Domain Ocular Coherence Tomography, Fundus Auto Fluorescence, Multifocal Electroretinogram, Fundus Fluorescein Angiography) were noted.

Chi Square test was used for statistical analysis and p<0. 05 was considered significant.

Results: Maculopathy was found in 13. 5% patients on HCQ beyond 1 year.

No statistical association was seen between HCQ maculopathy and Gender (p= 0. 189), BMI (p=0. 289), Diagnosis(p=0. 865), Comorbidities and daily dosage of HCQ (p=0. 171).

However, a significant correlation was found between HCQ maculopathy and Age {8. 4%<30 years VS 20. 3%>60 years(p=0. 033)}, Weight {8. 7%<50kg VS 15. 1%>60kg(p=0. 045)}, HCQ duration {11. 7%<5 years VS 21. 1%>5 years (p=0. 002)} and Cumulative dose {283. 79 g VS 231. 33 g (p=0. 006)}.

The detection rates of maculopathy by different Methods: were HVF (13. 5%), SDOCT (13. 3%), mfERG (12. 8%), FAF (12. 1%), FFA (11. 8%), HVF+SDOCT (13. 3%), HVF+mfERG (12. 8%) and HVF+FAF (12. 1%).

Conclusion: Hydroxychloroquine maculopathy is not infrequently seen in rheumatic disease patients in India.


  PM0042: Case of refractory diffuse alveolar hemorrhage in systemic lupus erythematosus: Role of intrapulmonary human recombinant activated factor VII therapy Top


Prakash D Paymode, V Sarath Chandra Mouli, Shilpa Suvarna, Srisaila Datta; Department of Rheumatology and Immunology, Krishna Institute of Medical Sciences Hospital, Secunderabad, Telangana, India

38 year old female, presented with cough with hemoptysis, dyspnea for 2 weeks and a fever episode 2 days before with past history ofpolyarthritis and skin rash. On examination, patient had tachypnea, tachycardia, pallor, bilateral basal crepitations with normal Blood pressure and SpO2 95%. Evaluation revealed severe anemia, ANA-IF 4+, very high Anti ds-DNA, low complements, proteinuria (1600 mg/day). HRCT chest showed bilateral diffuse ground glass opacities with interstitial opacities [Figure 1]. Her infective workup was negative. As per ACR/SLICC criteria she was diagnosed as SLE with lupus nephritis and diffuse alveolar hemorrhage (DAH). She was treated with Methylprednisolone pulse (1000 mg for 5 days), cyclophosphamide (500 mg),IV immunoglobulin(2g/kg), plamapheresis (4 cycles), Rituximab later. Despite this, DAH was progressive in the form of frank hemoptysis (200 ml/day), falling saturation and Hb. After reviewing literature, we administered intrabronchial humanrecombinant activated factor VII(rFVIIa) initially 3 mg followed by 4 mg after. Next day, hemoptysis reduced and stopped after 2nd dose. Her Hb and oxygenation improved significantly. She was continued on prednisolone (1mg/kg), cyclophosphamide fortnightly. Her DAH resolved completely. Unfortunately, on follow up after a month she succumbed to death due to sepsis.

Discussion: DAH in known complication of SLE with high mortality. In literature, in few case reports, local intrapulmonary therapy with one or more doses of recombinant Factor VIIa was found to have a good to excellent hemostatic effect by forming rFVIIa-tissue factor complexand improved oxygenationin patients with DAHwithout systemiccomplications. 1, 2. We present this case of refractory DAH responded to this newer therapy, adding to literature.

Conclusion: Intrapulmonary rFVIIa therapy is very effective should be tried in refractory DAH.
Figure 1

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  PM0043: Systemic chronic capillary leak syndrome associated with serum Anti-SSA/Ro antibodies Top


Anuj Shukla, Priyanka Gaur; Niruj Rheumatology Clinic, Ahmedabad, Gujarat, India

Case Report: 31year old lady presented with generalized edema from 20days. On examination, she had puffiness of face and pitting pedaledema. Investigation showed low serum albumin 3gm/dl, high globulin 3. 5gm/dl without proteinuria. There was no evidence of thyroid, hepatic or heart dysfunction or allergic manifestations. Her ANA was 4+nuclear speckled 1:100 with anti-Ro60(92AU/ml) and Anti-Ro52(94AU/ml) positive. Her ESR (25mm at 1hour) and serum C-reactive protein (0. 75mg/dl) were slightly raised while complements C3(0. 74gm/l) and C4(8mg/dl) were persistently low. She had no other features of Sjogren's syndrome.

The symptoms waxed-waned but persisted for 3months. Based on these features, she was diagnosed as a case of systemic chronic capillary leak syndrome (CLS). Her protein electrophoresis showed low albumin with polyclonal gammopathy but no paraproteinemia. So Anti-SSA/Ro was suspected to be the culprit. Anti-SSA/Ro persistent positivity was confirmed by ELISA 116U/ml(>15positive).

She refused regular treatment with immunomodulators and continued home remedies. She revisited after 10months and was 16weeks pregnant with complaint of increased edema from last 1month. She is now treated with hydroxychloroquine 5mg/kg and counselled regarding the risks.

Discussion: Systemic-chronic-CLS is characterized by capillary endothelial dysfunction causing extravascular leakage of fluid and small proteins example albumin resulting into hypoalbuminemia, hemoconcentration and generalized edema. In about 80% of cases, monoclonal gammopathy is detected. Acute leakage can be fatal with hypotension. Other features of pleural, pericardial effusion and ascites can be seen in severe cases. In a case series of CLS associated with autoimmune disease, 3/5 had Anti-SSA/Ro positive.[1]

In such cases, these antibodies are believed to cause capillary endothelial dysfunction mediated by autoimmunity or inflammation.

Conclusion: Thus, Systemic-chronic-CLS can be a rare manifestation of autoimmunity associated with anti-SSA/Ro antibodies positivity.

References

  1. Guffroy A, Dervieux B, Gravier S, Martinez C, Deibener-Kaminsky J, Hachulla E, et al. Systemic capillary leak syndrome and autoimmune diseases: A case series. Semin Arthritis Rheum 2017;46:509-12.



  PM0044: SLE presenting as chronic meningitis in a paediatric patient: A case report Top


Archana Singh, T N Tamilselvam, G Nikhila, N Sujatha, S Karthikeyan; Institute of Rheumatology, MMC, Chennai, Tamil Nadu, India

Pachymeningitis is a rare clinical entity associated with infections, malignancy or rheumatological diseases. Idiopathic hyertrophic pachymeningitis has also been reported. It is characterised by thickening of intracranial dura matter, depicted by a MRI scan. SLE can present with a wide spectrum of clinical presentations and neurological involvement.

We report a case of a pediatric patient who was treated for chronic meningitis and subsequently diagnosed as SLE.

A 15 years old female patient presented with history of headache, low grade fever of 6 months duration. There was no history of blurring of vision, weakness of any limb, altered sensorium or seizures. There was no history of rash, photosensitivity, oral ulcers or polyarthritis/polyarthralgia. She was evaluated at a local hospital for meningitis and started on ATT and acyclovir empirically. But she showed no improvementand was referred to medicine department of our hospital. On examination, patient had mild pallor. Her otherwise general physical examination was unremarkable with no evidence of a CTD. Central nervous system examination was normal with no neurological deficits. Blood investigations showed ESR-45, Hb- 9. 8g/dl, TLC- 4100 cells/μl and PC- 2. 4lacs/ μl. Her blood biochemistries were normal. CSF analysis showed no cells, normal glucose and protein. CSF bacterial and fungal cultures, CBNAAT for tuberculosis, VDRL, India ink staining, VZV IgG/IgMwere negative and adenosine deaminase (ADA) levels were normal. MRI showed bilateral tentorialenhancement and pachymeningitis. Her renal, cardiac and pulmonary evaluation was normal. Based on MRI report, rheumatologist's opinion was sought. We advised for autoimmune work up and patient was started on high dose steroids. Her CRP was >6mg/dl, ANA was 4+, C3/C4 were low and anti dsDNA was 545 IU/ml. She showed significant improvement in her symptoms and was discharged.


  PM0045: Digital gangrene as the presenting clinical feature in connective tissue disorders: A case series from Rajasthan, India Top


Neeraja Vijayan, Sonu Pandit, Maya Gopalakrishnan, Gopal Krishna Bohra, Mahender Kumar Garg; All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

Introduction: Digital gangrene is a known complication of connective tissue disorders (CTD), with a varied prevalence of 30% in systemic sclerosis to 5% in rheumatoid arthritis. Here, we describe 6 patients with digital gangrene secondary to CTDs and their outcomes.

Case Discussion: Of 6 patients who presented with digital gangrene, all were females, 5 had gangrene of lower limbs, and one had both upper and lower limb involvement. The underlying CTDs were: Mixed connective tissue disorder (MCTD), SLE, Antiphospholipid syndrome (APS), Rheumatoid arthritis- CREST overlap syndrome while other two patients had undifferentiated vasculitis syndrome. All patients received on pulse methyl prednisolone followed by prednisolone which was tapered to lowest possible dose over few months. All patients except one subsequently improved with no gangrenous extension, without any requirement for surgical intervention. The patient with pulmonary-renal syndrome had fulminant course, was started on mycophenolate-mofetil and further planned for rituximab, but succumbed to illness.

Discussion: Isolated digital gangrene maybe the first presentation of various CTDs. In our experience, 5 of 6 patients presenting with digital gangrene recovered with steroid use alone, not requiring other immune suppressants. Currently all five patients are doing well on follow up.

Conclusion: Prompt initiation of immunosuppression with steroids is essential in CTD associated limb gangrene. This can arrest progression and avoid unnecessary surgical interventions or limb loss.
Table 1: Important clinical variables in patient diagnosed with systemic lupus erythematosus related pseud obstruction

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  PM0046: A perplexing case of primary APLA with bilateral adrenal hemorrhage Top


Rajesh Kumar, Ratul Seal; AIIMS, Bhubaneswar, Odisha, India

Antiphospholipid syndrome (APS) or Hughes syndrome is an autoimmune thrombophilic condition that have circulating antibodies against plasma proteins that binds to phospholipids and are clinically characterized by recurrent arterial and venous thrombotic events and pregnancy morbidities. Atraumatic adrenal hemorrhage (AD) leading to adrenal insufficiency is a rare, but lifethreatening presentation of APS in whichadrenal vein thrombosis secondary to immune complex accumulation causes disruption of adrenal gland outflowultimately leading to hemorrhage infarction of adrenal gland (AD). AD is a rare presentation found in 0. 4% of APS cases and conversely APS is diagnosed in less than 0. 5% of AD. Initial diagnosis and follow up in such cases is very challenging.

A 33 years old female presented with fever for 15 days, abdominal pain, low blood pressure with no history ofabnormal bleeding or any pregnancy loss. Serum cortisol levels were low and antinuclear antibodies were absent. Abdominal CT and MRI imaging revealed bilateral adrenal hemorrhage. Meticulous workup into etiology showed increased lupus anticoagulant antibodies (IgM) and b2GP1 (IgM)on two occasions 12 weeks apart which clinched the diagnosis of 'Primary APLA' after ruling out secondary causes, in accordance of the 'Sydney Criteria' and it presented with bilateral AD hemorrhage and adrenal insufficiency. Treatment was started with steroid replacement along with warfarin and other supportive measures with close monitoring. Subsequently the patient became asymptomatic, hypotension state improved and repeated imaging in last two years of follow up, showed resolution of the adrenal hematomas.

Conclusion: A high index of suspicion is required for evaluation of adrenal insufficiency and 'APLA' should be kept in mind in cases presenting with unexplained adrenal hemorrhage. Although this is a rare presentation of APLA thenumber of recent incidences are on the rise probably due to increasing use of better radio diagnostic modalities.


  PM0047: Unilateral sacroilitis in patients with sytemic connective tissue disease Top


Vignesh Mantharam, Saranya Chinnadurai, Balaji Chillukuri, Shanmugesh Selvaraj, Shankar Ramachandran, Rajeswari Sankaralingam; Department of Rheumatology, Sri Ramachandra Institute of Higher Education and Research, SRIHER, Chennai, Tamil Nadu, India

Background: Patients with autoimmune diseases are at risk of infection and malignancy. Here we present two cases of unilateral sacroilitis (Tuberculous sacroilitis in Systemic Lupus erythematosus and metastatic adenocarcinoma in Systemic sclerosis).

Case 1: A 32 year old lady, a known SLE for 4 years with history of lupus nephritis in remission and severe pulmonary hypertension came with fever and mechanical low back pain and right buttock pain (8/10) with early morning stiffness not relieving with analgesics. MRI pelvis showed right sacroilitis with surrounding muscle edema. CT-guided bone biopsy from the sacrum showed ill-defined necrotic epitheloid granulomas over the bony fragments suggestive tuberculosis.

Case 2: A 34 year old lady, a known Systemic sclerosis with ILD for 5 years, presented with chronic cough for 2 months. On evaluation, CT chest showed left upper consolidation. Bronchoscopy was normal and BAL cytology was negative for infection and malignancy. In view of non- resolving pneumonia, patient was started on empirical tuberculosis therapy. 10 days later, she developed low back pain and left buttock pain (5/10). MRI showed left sacroilitis with STIR intensities at the attachment of adductors and obturator externus. CT guided biopsy from the left sacral bone revealed moderately differentiated adenocarcinoma. PET CT showed increased metabolic uptake from the left upper lobe and also in left 3rd rib, sacral ala and posterior element of L4, mediastinal lymph nodes and left sided pleural effusion suggestive of stage IV adeno-carcinoma of lung.

Discussion: Presence of unilateral buttock pain, unilateral sacroilitis with inflammation beyond the joint line in patients on chronic immunomodulatory therapy made usdo CT-guided biopsy which revealed the diagnosis.

Conclusion: One should not ignore a patient with severe low back pain and unilateral buttock pain. All patients with unilateral sacroilitis should be evaluated for microbiological evidence of infection or histopathological evidence of malignancy.


  PM0048: Tuberculous arthritis mimicking flare of rheumatoid arthritis Top


Vaibhavi G Velangi, Ishita S Shah, Yogesh Preet Singh; Fellow in Rheumatology

Introduction: Joint infection complicating rheumatoid arthritis (RA) although infrequent is well known. Patients with RA have 4 fold-increased risk of Tuberculosis(TB). Pulmonary TB is the most common from accounting for more than 50% of cases. Musculoskeletal TB (MSK TB) involvement occurs in only 1-3%. The most common presentation of MSK TB is chronic monoarthritis and can be mistaken for flare of RA.

Index case: A 40-year-old lady with disease onset in 2013, presented to us in May 2018 with active RA. She was not on any medications. She was started on methotrexate(MTX) 15 mg/week. As left elbow was affecting her day-to-day activities it was injected with triamcinolone acetonide 40mg.

She was subsequently lost to follow-up. In the interim, she visited doctors locally for further treatment. Sulphasalazine 2g/day and deflazacort 6mg/day were added; MTX was continued at 15mg / week. Intra-articular glucocorticoid was repeated twice over a period of 3 months. The last injection was given 3 months prior to repeat visit to our centre.

She presented to us again in January 2019 with progressive increase in left elbow pain, swelling, erythema and local raise in temperature. She did not have any constitutional symptoms. Joint ultrasound findings as per [Figure 1]. Synovial fluid AFB smear and mycobacterial culture were positive [Table 1]. Anti tubercular treatment was started. At follow-up after 2 months elbow pain and swelling had reduced considerably.
Figure 1: Ultrasound of the left elbow. Ultrasound of the left elbow synovial thickening (up to 10mm) with minimal joint effusion, erosion of radial head and olecradnon bursitis

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Table 1

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Conclusion: Tuberculous arthritis can mimic a flare of RA. High degree of suspicion is required for diagnosis. Presence of disproportionate local inflammation should raise possibility of underlying infectious arthritis. Constitutional symptoms may be absent. In suspected cases synovial fluid analysis for micro-organisms should bedone at the earliest for good outcomes.


  PM0049: Acute gout mimicking flare of osteoarthritis Top


Vaibhavi G Velangi, Ishita S Shah, Yogesh Preet Singh; Fellow in Rheumatology

Background: Osteoarthritis(OA)is associated with aging. The incidence of symptomatic OA is 12-16% in adults > 60yrs of age. Crystals are not infrequently observed within the articular tissues of degenerated joints. Flare of OA can be similar to attack of mono-articular gout presenting with pain, effusion and limitation of joint mobility. Acute gout attack and flare of OA can mimic each other.

Methods: This is a retrospective case series of OA patients in whom an acute attack of gout mimicked flare of OA. Patients satisfying the 2016 ACR Clinical Criteria for OA knee were included. Duration of data collection was from 2016 to 2019. The demographic and clinical data is described in Table 1. The median age was 60 years (50-75). Out of 9 patients 7 were male. The median duration of OA was 36 months (6-72) and median duration of each attack was 21days(4-60). Knee X-rays could be retrieved in 7 patients. Kellgren and Lawrence (KL)grading for knee OA is as per [Table 1]. All patients presented with acute knee arthritis. Recurrent attacks were seen in 7 out of 9 patients. Synovial fluid analysis confirmed the presence of sodium urate crystals. All patients received colchicine prophylaxis. Urate lowering therapy was started in 3 patients and are doing well on subsequent follow-ups. In rest of the cases follow-up is awaited.
Figure 1: Light microscopy of synovial flyuid needle shaped monosodium urate crystals

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{Table 44}

Conclusion: Gout can mimic a flare of osteoarthritis. It can present as isolated knee arthritis. Knee involvement can be the presenting feature of gout. Most patients have recurrent attacks. The duration of each attack ismore than the expected duration of a typical gout attack. Co-existent gout attack needs to be considered in every patient with flare of OA knee. Synovial fluid analysis for crystals helps in differentiating acute gout from flare of OA.


  PM0050: Tubercular pyomyositis in a case of polymyositis Top


Ishita Shah, Vaibhavi Velangi, Yogesh Preet Singh, Balasubramanyam Shankar; Manipal Hospital, Bengaluru, Karnataka, India

Background: Tuberculosis (TB) is a “re-emerging disease”, with increasing incidence in 21st century, particularly in immunocompromised patients. Musculoskeletal TB accounts for 10%-25% cases of extra pulmonary tuberculosis. Tuberculous myositis is rare and may mimic malignant or other inflammatory diseases.

Case Report: A 56-year-old woman presented with severe pain, localized swelling around left thigh for 3 months. There was no history of fever, loss of appetite or weight loss. Examination showed asymmetrical, tender, red indurated area of 8 cm x 7cm on posterior aspect of thigh. Power at shoulder girdle was 4/5 and pelvic girdle 3/5. Two months ago she had visited another hospital with proximal muscle weakness. Diagnosis of polymyositis was made based on elevated CPK level, electromyogram and muscle biopsy findings. At presentation to us she was on Prednisolone 20mg per day. Investigations were as per the [Table 1]. Ultrasonography and MRI findings were as per [Figure 1] and [Figure 2]. Imaging showed disproportionate left posterior thigh involvement. Biopsy of the affected muscle group showed Acid fast bacilli on smear and culture grew mycobacterium tuberculosis. CT scan of the chest, abdomen and pelvis were normal.
Table 1: Demographic and clinical details

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Figure 1: Ultrasonography of left thigh showed bulky muscles in posterior compartment with overlying subcutaneous oedema and minimal intramuscular free fluid

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Figure 2: (Sagittal section) MRI of both thighs showed the anterior compartment muscles, posterior compartment muscles, bilateral gluteus maximus, obturator internus and externus, sartorius and gracilis muscles bulky with T1, T2 hyperintensity. Involvement of left side was more than rights side and involvement of posterior compartment was more than anterior compartment suggested polymyositis with possible super added infection in the left thigh region

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Active myositis in the setting of active ongoing TB infection was treated with intravenous Immunoglobulins. She was started on anti-tubercular treatment (ATT). Mycophenolate mofetil was added after completing intensive phase of ATT. Improvement in muscle power and reduction in the size of induration was noted at follow up.

Conclusion: In immunocompromised state typical clinical presentation of TB is lacking; constitutional symptoms like fever, weight loss can be absent. In inflammatory myopathy, infective etiology should be considered for any atypical muscle swelling with unusual tenderness or myositis not responding to standard treatment. Biopsy of the muscle or aspiration with culture confirms the diagnosis.


  PM0051: Polyarticular septic arthritis in rheumatological diseases: A case series Top


Nupoor Acharya, Shankar Naidu, Aman Sharma, Shefali K Khanna, Sanjay Jain, Varun Dhir; Department of Internal Medicine, Division of Rheumatology, PGIMER, Chandigarh, India

Background: Polyarticular septic arthritis in adults is an uncommon finding with high morbidity and mortality. Early intervention with medical and surgical treatment is required to improve outcome. Patients with rheumatological conditions are more prone to development of septic arthritis.

Objective: To study the clinical features and outcome of polyarticular septic arthritis in patients with rheumatological illnesses.

Methods: Septic arthritis wasdiagnosed on the basisof synovial fluid showing evidence of microorganismsin theform of culture positivity and/or organism on grams stain with thesynovial fluidshowing frankpus. Polyarticular septic arthritis was defined asinvolvement of two or more joints.

Results: Here we report a series of four patients with different rheumatological conditions presenting with septic arthritis involving multiple joints. One patient had systemic sclerosis, one psoriatic arthritis, one rheumatoid arthritis and had an overlap connective tissue disease. The joints most commonly involved were the ankle and the elbow joints, (involved in 3 patients). The causative organism was Staphylococcus aureus Scientific Name Search  in two patients, pneumococcus in one, and gram-negative bacilli (not cultured) in one patient. All of the patients had received immunosuppressive therapy prior to the onset of septic arthritis. All the patients received intravenous antibiotics and surgical drainage was performed in three patients. Two patients died due to sepsis and two improved after treatment with residual joint damage.

Conclusion: PASA carries a poor prognosis and mortality was high among the group despite aggressive medical and surgical interventions.
Figure 1

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  PM0052: Hide-and-See: Macrophage activation syndrome in adult lupus, a frequent but under-recognized complication. Our experience from a tertiary care hospital in Chennai Top


Ramu Ramaswamy, Rajeswari Sankaralingam, Saranya Chinnadurai, Shanmugesh Selvaraj, Balaji Chilukuri, Vignesh Mantharam, Aishwarya Ramachandran; Department of Rheumatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Background: Macrophage Activation Syndrome (MAS) is a severe life- threatening complication of severe Rheumatological Diseases especially SLE and sJIA. It is thought to be caused by excessive activation and proliferation of T-lymphocytes and Macrophages, leading to widespread hemophagocytosis and cytokine overproduction.

Objective: To study and describe the clinical, laboratory features, complications, precipitating factors, treatment and outcome of Macrophage Activation Syndrome in cases of Adult Lupus.

Methods: A prospective observational study was conducted in a tertiary care centre between January 2018 and June 2019. Our cases were classified as MAS based on the revised HLH 2010 criteria.

Results: Among a total 110 patients with Systemic Lupus Erythematosus (SLE) admitted as inpatients in our hospital, 7 cases (6. 3%) of MAS were identified. Mean age = 28. 57 years, M:F = 0:7, mean duration of current illness = 23. 7 days, mean duration between onset of SLE and onset of MAS ranges from 3 months to 13 years. All the patients clinically had Fever and Lymphadenopathy. 2 cases had Rash at presentation. Among the laboratory features all the patients had Hematological involvement (Anemia n=7, Leucopenia n=5, Thrombocytopenia n=4), Serum Transaminitis, Hyperferritinemia, Hypofibrinogenemia, Hypertriglyceridemia. USG Abdomen revealed mild to moderate splenomegaly in 3 cases. Hypocomplementemia (n=3), High Anti dsDNA titers (n=3). Infectious screening turned out to be positive in 4 out of 7 cases. Viral serologies revealed positive IgM antibodies to CMV in 2 cases, positive IgM/IgG antibodies to EBV infection in 1 case, and Dengue IgM/NS1Ag positivity in 1 case. All patients received Corticosteroids. Cyclophosphamide was the most commonly used immunosuppressant. Rituximab was given in 2 cases. Recorded nil mortality.

Conclusion: SLE flare and Infection were common triggers of MAS in SLE. The presentation of unexplained fever, cytopenia, or liver dysfunction, with high levels of ferritin and LDH, in patients with SLE should raise the suspicion of MAS.


  PM0053: Posterior reversible encephalopathy syndrome in rheumatoid arthritis: A rare clinical dilemma Top


Hargurdas Singh, Prateek Mangal, Dinesh Yadav, Abhinav Chowdury, Raghwendra Singh, Kamal Yadav, Tanweer Ahmad, Ashis Kumar Saha, Rezual Karim; Mata Gujri Memorial Medical College, Kishanganj, Bihar, India

Case Presentation: 35 year old female presented to us with bilateral large and small joint pain with distal interphalangeal joint sparing, morning stiffness for more than 1 hour for 1year and sudden decreased vision in both eyes from 15 days. Patient was on oral methotrexate 7. 5mg/week and DMARDS for 1 month. Vision was finger counting one metre for both eyes and ocular examination could not explain it. So MRI brain was done. CEMRI showed hyperintense areas T2 and FLAIR sequence in right cerebellum, both posterior temporal & parietooccipital region with contrast enhancement, hyperintensity on ADC map without remarkable diffusion restriction suggestive of Posterior Reversible Encephalopathy Syndrome. Ra factor 32 IU/ml, Anti-CCP 83. 21RU/ml, CRP 24mg/l, ESR 48, ANA 7. 84 AU/ml, Anti-dsDNA 0. 32 OD ratio, Urinary ACR 5. 3 ug/mg. Acc to 2010 ACR/EULAR criteria she scored 10/10. Patient was diagnosed as Rheumatoid arthritis with PRES. Patient was given pulse therapy(methylprednisolone). Then put on oral prednisolone and sulphasalazine. Her vision improved significantly in 10 days, was right eye 6/24 and left eye 6/18. Repeat MRI done after 1 month showed improvement and reduction in edema.

Case Discussion: PRES is a clinic-radiological entity of diverse etiology like hypertensive encephalopathy, eclampsia, renal failure, immunosuppresive drugs, autoimmune diseases characterised by headache, visual disturbance, seizures and radiological finding of vasogenic edema especially in areas of posterior circulation. Although exact etiology is not known, it is postulated that rapid rise in blood pressure overcomes cerebral autoregulatory mechanism causing dilatation of cerebral arterioles, opening of endothelial tight junctions and cerebral edema. Treatment consist of removing drug/causative factor. Prognosis is usually benign but may lead to permanent neurological sequale.

Conclusion: PRES is rare condition. Its clinical dilemma that PRES in my patient is due to autoimmune process or oral methotrexate as just 6-7 reported cases have long drug history of methotrexate and mainly in RA. PRES due to RA has never been reported earlier.


  PM0054: Clinical manifestations and outcome of Behcet's disease: An Indian perspective Top


Siddharth Jain, Arghya Chattopadhyay, Shankar Naidu, M Valliappan1, Vishal Sharma2, Varun Dhir, Ramandeep Singh3, Rajesh Vijayvergiya4, Manphool Singhal5, S K Sinha2, Sanjay Jain, Aman Sharma; Department of Internal Medicine, Division of Clinical Immunology and Rheumatology, Departments of1Pulmonary Medicine,2Gastroenterology,3Ophthalmology,4Cardiology, and5Radiodiagnosis, PGIMER, Chandigarh, India

Background: Behçet's disease (BD) is a variable-vessel vasculitis commonly presenting with recurrent oro-genital ulceration, skin lesions and visual disturbances. Ethnic and geographical variations exist in the clinical phenotype of BD. There is paucity of Indian data.

Objectives: To establish a clinico-laboratory profile of Indian BD patients and study their outcome.

Methods: Patients of BD (2006 ICBD criteria) presenting to Rheumatology services at PGIMER, Chandigarh were recruited prospectively from July 2017. Demographic, clinical, laboratory and radiology data and treatment outcomes were analysed.

Results: 47 patients with mean(SD) age 30. 2 (10. 8) years were recruited. 39 (83%) were males. The median duration of disease at presentation was 5. 7 years (2 weeks to 15 years). 44 (93. 6%) had oral ulcers, 38 (80%) had genital ulcers, 62. 5% had skin lesions, 72%had ocular involvement while 16 (34%) had vascular disease. Amongst non-criteria manifestations, 8 (17%) had gastrointestinal involvement while 4 (8. 5%) had neuro-behcets. Amongst vascular BD, 11 had arterial aneurysms, 8 had deep venous thrombosis (including cortical venous thrombosis) whilst 3 had both. Fever and joint pains were seen in 80% and 58% respectively. The median ESR and CRP were 32 (4-90) mm and 76 (5-198) mg/L. HLA-B51 and pathergy positivity was noted in 7/13 (54%) and 2/7 (28. 6%) patients respectively. Most patients with ocular disease were managed with azathioprine +/- cyclosporine. Meselamine or sulphasalazine was used in 3 patients with GI involvement. 9 patients with arterial aneurysms required use of cyclophosphamide while one required infliximab. Adalimumab was used in 3 patients, 2 for refractory GI disease and 1 for recurrent PG-like extensive cutaneous ulcers. Three patients (6. 4%) died, 2 because of massive haemoptysis due to pulmonary artery aneurysm, while 1 had a sudden cardiac death.
Figure 1: MRI brain at the time of admission, MRI brain after 1 month

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Conclusion: Male preponderance, arterial aneurysms, ocular disease and gastrointestinal involvement was much more common in our Indian BD patient cohort compared with other countries.


  PM0055: Methotrexate misadventures: A case series from a tertiary care hospital Top


Balaji Chilukuri, Sowmya Parvathareddy, Saranya Chinnadurai, Vignesh Mantharam, Shanmugesh Selvaraj, Ramu Ramaswamy, K Punnagi, Rajeswari Sankaralingam; Departments of Rheumatology and Pharmacology, Sri Ramachandra Medical College and Research Institute, Chennai, Tamil Nadu, India

Case Series: Methotrexate (MTX) is a commonly prescribed safe immunosuppressant in Rheumatology. We report 6 consecutive cases of MTX toxicity. Five cases received MTX for Rheumatoid arthritis and one case received MTX for Granulomatosis with polyangitis. These cases have been divided into 2 groups- acute and chronic. Four cases in acute group developed toxicity due to erroneously taking MTX daily (cumulative mean dose 50 mg) during initiation of treatment. Two cases in chronic group developed toxicity on stable doses of weekly MTX (weekly mean dose 20mg). Risk factors identified for chronic toxicity were old age, renal dysfunction and concomitant use of leflunomide. Clinical manifestations were oral mucositis (n=6), gastrointestinal intolerance (n=5), skin ulcer (n=1). Cytopenias observed in chronic group were more severe than the acute group. Pneumonitis was not observed in our case series. Mean folinic acid dose administered was 230 mg and 470 mg in acute and chronic groups respectively. Oral mucositis was treated with topical squish of syrup prednisolone, antacid and promethazine. Mean hospital stay was 4. 5 days and 10. 5 days in acute and chronic groups respectively. Both patients in chronic group had persistent cytopenias and prolonged illness which responded to recombinant Human Granulocyte-Colony Stimulating Factor (G-CSF) and platelet transfusion.

Discussion: MTX widely used by Rheumatologists due to proven efficacy and affordability has the potential to cause toxicity. Both acute and chronic toxicity present with similar manifestations though chronic toxicity is more severe and needs prolonged treatment. Patient compliance, awareness about its correct administration, potential toxicity, warning symptoms of MTX toxicity, regular monitoring of renal function can prevent incidence of toxicity.

Conclusion: We observed that Chronic MTX toxicity is associated with more severe mucositis and myelosupression which needs prolonged and aggressive treatment including higher doses of folinic acid and G-CSF than acute MTX toxicity.
Figure 1

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  PM0056: A case series of Bullous Lupus: Higher incidence of NPSLE and juvenile patients Top


P Akshay, G Meghna, R Pratyusha, D Phani Kumar, R Liza; Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Objective: To study the clinical profile of patients with bullous systemic lupus erythematosus (BSLE) in a tertiary care centre.

Methods: All SLE patients presenting with generalized vesiculobullous rash from 2006 to 2019 were identified from lupus registry. Those with localized, drug induced or infectious bullous lesions were excluded. The data was analyzed with Microsoft excel.

Results: Fifteen patients were identified. Mean age was 19. 4±6. 2 years. Of them 13(86. 6%) were females and 5(33. 3%) were juvenile. Juvenile lupus accounted for a significant proportion. Thirteen patients (86%) had bullous rash as the first manifestation of lupus while 2 had mean disease duration of 1. 5 years. Lesions were predominantly located on face, trunk and upper limbs. Lower limb involvement was less frequent with fewer lesions. Mucosal involvement was seen in one. Lesions healed with dyspigmentation in 5 and scarring in 1 patient and two had secondary infection.

Extra-cutaneous manifestations were seen in 14(93. 3%) patients with neuropsychiatric lupus being most common(56%). Of NPSLE manifestations, psychosis was most frequent (55. 5%) followed by seizures (33. 3%). Psychosis and bullous rash were concomitant in six patients, psychosis preceded in 2 and developed later in one but all within 6 months of rash.

Second most common was nephritis(33%) occurring concomitantly in 3 and after a median 2. 5 years in 2. Median SLEDAI of the cohort was 10(IQR 4. 5-14).

Skin biopsy was done in 6 patients all showing subepidermal blistering and neutrophilic dermal infiltrates. Eight patients receivedcyclophosphamide, 6 IV methylprednisolone, and 1 received rituximab.

Conclusion: Bullous lupus occurs in younger lupus patients. It is associated with neuropsychiatric lupus and lupus nephritis.


  PM0057: Case of Behcets syndrome with necrotic ulceration of lip, scrotal ulcer and leucocytosis Top


Varghese Koshy, Vandana, George Koshy, Vandana Gangadharan; Command Hospital (Central Command), Lucknow, Uttar Pradesh, India

Patient is a 23 year oldmale who initially became symptomaticwith sore throat, oral ulcers and dysphagia. He also developed an ulcer over the lower lip near the right angle of mouth which gradually became necrotic with purulent discharge.

He was initially managed with IV antibiotics for one week at a peripheral hospital and subsequently transferred to Command Hospital (WesternCommand). At this centre he also developed a scrotal ulcerative lesion.

Evaluation at the tertiary centre revealed a necrotic lip ulcer along with scrotal ulcer and Leucocytosis(more than 30, 000/mm3). Biopsy from the scrotal lesion revealed pustular vasculitis.

A clinical diagnosis of Behcets disease was made.

Patient showed an excellent response to Injection Methylprednisolone pulse 1gm OD for 3 days followed by tapering oral corticosteroids and Tab Azathioprine.

There was complete resolution of his lip and scrotalulcers.

Discussion: Leuko-cytoclastic vasculitis, fibrinoid necrosis of postcapillary venules, or perivascular neutrophilic accumulations are some of the reported patterns in the early stages of the cutaneous lesions.[1] Use of high dose corticosteroid, inspite of leucocytosis, and second line immunosuppression with Azathioprine, MMF, Methotrexate or Colchicine is also required to achieve and maintain resolution of the lesions.

Conclusion: Since Behcets Disease is a clinical diagnosis, a high index of suspicion is a prerequisite to diagnosing the condition and reporting of cases with its varied and myriad manifestations.

References

  1. Chun SI, Su WP, Lee S, Rogers RS 3rd. Erythema nodosum-like lesions in Behçet's syndrome: A histopathologic study of 30 cases. J Cutan Pathol 1989;16:259-65.
Figure 1

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  PM0058: Gastrointestinal sarcoid: A rare initial manifestation of sarcoidosis Top


Pooja J Belani, Vishad Viswanath; Institute for Rheumatology and Immunology Sciences, Thiruvananthapuram, Kerala, India

A 30-year-old gentleman presented with 3 years history of intermittent watery loose stools, 2-3 episodes/month, 4 times a day, that was difficult to flush with inflammatory low backache, EMS of 30-60 mins and difficulty in turning in bed since 4 months. There was no weight loss, fever, recurrent oral ulcers, red painful eyes, chronic cough or breathlessness. He had past history of scalp psoriasis 6 years back improved with coal tar treatment. General and systemic examination, including MSK was unremarkable. Possibility of Spondyloarthritis was strongly considered. Hemogram and renal parameters were normal except elevated AST/ALT and S. ALP, for which USG abdomen and MRI abdomen was done that was suggestive of preaortic and paraaortic lymphnodes, with hepatosplenomegaly. Liver, gastric antrum and lymphnode biopsy were suggestive of well-formed granuloma with negative cultures for TB, negative IHC markers for lymphoma. Differentials thought were Crohns disease and sarcoidosis. S. ACE levels and calcium were normal. Provisional diagnosis of sarcoidosis was made on the basis of hepatic granuloma which is rare in Crohn's disease. He was started on tapering Prednisolone 40mg/day.
Figure 1

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Discussion: Symptomatic gastrointestinal(GI) tract involvement(excluding hepatobiliary system) is rare manifestation of sarcoidosis with prevalence of 0. 1-0. 9%. Only 44 cases of asymptomatic gastric involvement are recorded till 2010. Stomach is the most common site and small intestine is least common. Absolute pointers favouring sarcoidosis over Crohn's disease include hypercalcemia, lung involvement, elevated ACE levels, cardiac involvement and generalized lymphadenopathy. Palpable splenomegaly and hepatic granulomas are rare in Crohn's as compared to sarcoidosis. Lymphoma can rarely present as granulomatous disease. Infections like Tuberculosis, Histoplasmosis, Syphillis, and chronic EBV need to be ruled out. Steroids should be started in symptomatic patients with the addition of steroid-sparing agent in case of nonresponse or steroid dependence.

Conclusion: Sarcoidosis is great mimicker and should be considered in the differential diagnosis of any systemic granulomatous disease.


  PM0059: Myasthenia Gravis masquerading as myositis in autoimmune diseases Top


Kasturi Hazarika Manesh Manoj, Prashant Bafna, Rasmi Ranjan Sahoo, Anupam Wakhlu; Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India

Herein, we discuss two cases of Myasthenia gravis(MG), one associated with primary Sjogren's syndrome (SS) and the other with Takayasu arteritis (TA).

Case 1: A 60yr-old female admitted with history of difficulty in walking, polyarthralgia and dry mouth for the last 2 years. She had mild proximal and distal weakness. Initial investigations revealed severe hypokalemia, with normal anion gap metabolic acidosis & alkaline urine with nephritic range proteinuria and no active sediments. Renal tubular acidosis was suspected. A positive ANA & ENA along with clinical features confirmed a diagnosis of SS. Later, she developed quadriparesis including distal muscle & bulbar weakness, despite improving hypokalemia. Myasthenia gravis was suspected and confirmed with a positive neostigmine test and positive myasthenia autoantibody profile.
Table 1: Laboratory tests

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Case 2: A 35yr-old female admitted with h/o easy fatiguability since 2 years, absence of pulses of right upper limb since 8 months and generalized weakness for 3 months & symptoms suggestive of palatopharyngeal weakness for 15days. On admission, she had severe neck and proximal muscle weakness and drooping of eyelids which on further questioning had a diurnal variation. A positive neostigmine challenge and repetitive nerve stimulation test confirmed MG. The right upper limb blood pressure was elevated. A CT aortogram revealed diffuse narrowing of the infrarenal aorta including bilateral external iliac arteries and narrowing of the left subclavian artery and proximal part of left axillary artery s/o TA.

Both patients were initially managed with IVIG, low-dose steroids gradually increased to 1mg/kg prednisolone to avoid acute worsening of MG and pyridostigmine. For case 1, potassium supplementation along with correction of acidosis was done and second-line immunosuppression with mycophenolate mofetil initiated. For case 2, anti-hypertensives were added as required and started on azathioprine as steroid-sparing agent.

Conclusion: MG can complicate the presentation of a number of autoimmune conditions.


  PM0060: Myelitis in adults and children with lupus: Experience from a single tertiary care center over 25 years Top


Pankti Mehta, Latika Gupta, Hafis Muhammed, Durga P Misra, Able Lawrence, Vikas Agarwal, Amita Aggarwal, Ramnath Misra; Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Introduction: Myelitis can rarely occur in the setting of lupus. Understanding the prevalence, demographic profile, clinical and serologic profile and treatment outcomes can be helpful for early identification and better management.

Methods: Medical records over 30 years from 1989-2018 from a large tertiary care center in Northern India were reviewed to identify patients with various forms of myelitis. Their demographics, clinical profile, course of illness, autoantibody profile and outcomes were compared with patients of lupus without myelitis. For each case, 2 matching comparators were drawn by the hospital registration number. Categorical variables were compared using chi-square and continuous variables using students-t test. p<0. 05 was taken as statistically significant. All statistics were done using SPSS (v23, IBM 2010).

Results: Of the 10 (0. 56%) of 1768 lupus cases who had myelitis, 3 (30%) were classified as Neuromyelitis Optica- NMO, 5 (50%) had NMO-Spectrum Disorder- NMOSD, 1 (10%) as Transverse Myelitisand 1 (10%) had a Clinically isolated syndrome -CIS. In 7 (70%), Myelitis was the first manifestation of Lupus. 6 of the 10 had relapsing disease (17 events) with a median time to relapse 2. 05 years (range 0. 08–15 years). ANA was negative to start with in 2 cases. 1 (25%) of the 4 tested positive for Anti-Aquaporin 4 antibody. Nephritis (2 vs. 15, p-0. 007) and hematologic (0 vs. 8, p=0. 029) manifestations were seen less often in Lupus with myelitis than those without it. Of the 7 who followed up, all received maintenance immunosuppression with Cyclophosphamide, Azathioprine or Rituximab.

Conclusion: Myelitis can be the first manifestation of lupus. CIS although not a part of ACR 1999 criteria for NPSLE is often seen in these patients. Nephritis and hematologic manifestations are less common in Lupus with myelitis. The disease should be aggressively treated with maintenance immunosuppression to prevent relapses.
Figure 1: Timeline of events of all 10 patients. Steroids give for every relapse (Not mentioned separately). Steroid sparing agent mentioned in bold whenever give. Outcome mentioned on a liker scale at the extreme right corner in red. J- January, F-February, A-April, Ju-July, O-October, S-September, N-November, A-Arthritis, LETM- longitudinally extensive transverse myelitis, ON- optic neuritis, CYC-Cyclophosphamide, RTXRituximab, AZA-Azathioprine, ACS-Acute confusional state, LN-Lupus nephritis, NIH-National institute of health protocol, ELNT-Euro-lupus nephritis trial protocol

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  PM0061: Case report of focal myositis in a patient of systemic sclerosis Top


G S Nikhila, T N Tamilselvam, Archana Singh, Karthikeyan, Sujatha; Department of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

25 yr old female was admitted with pain and swelling of left thigh for 3 weeks. She was a known case of Limited cutaneous systemic sclerosis with early ILD and was on MMF 1. 5 gm/day. On examination, there was a tender swelling of 4x3 cm in anterior aspect of middle 1/3rd of left thigh. Muscle power was normal and there was no systemic symptoms. Investigations showed elevated ESR and muscle enzymes with other biochemistry and CBC being normal. MRI left thigh was suggestive of myositis involving left Rectus femoris muscle. EMG showed myopathic pattern in left Rectus femoris, NCS was normal.

Patient was treated with oral steroids at 0. 5mg/kg/day and MMF continued. She responded to treatment with resolution of pain, swelling and normalizing muscle enzymes.

Discussion: Focal myositis is a rare benign inflammatory pseudotumour of skeletal muscle, that usually present in the extremities. Systemic symptoms and muscle weakness are rare. Diagnosis is by presence of elevated muscle enzymes, MRI imaging and muscle biopsy. Being an inflammatory condition, this responds well with steroids and immunosuppression. Focal myositis is describedin CTDs like Systemic sclerosis as one type of myositis.

Conclusion: Focal myositis should be considered when evaluating a patient with soft tissue mass in muscle plane in CTDs and is a DD for muscle sarcomas.
Figure 1

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  PM0062: Early onset rheumatoid vasculitis Top


Abhishek Pandey, Deepak Gautam, Lalit Prashant Meena, Ankit Mishra; Department of Medicine, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India

Rheumatoid vasculitis annual incidence among patients with rheumatoid arthritis (RA) is less than four per million. The condition usually occurs in patients with longstanding, severe rheumatoid arthritis. We are going to present a case series of ten patients presented with early onset rheumatoid vasculitis. We compared clinical characteristic, laboratory data and course in this case series.
Figure 1

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  PM0063: Atypical manifestation as an initial presenter of systemic lupus erythematosus Top


Meghanad Meher, Rajesh Kumar, Ratul Seal; AIIMS, Bhubaneswar, Odisha, India

Here we described 4 atypical clinical manifestation of systemic lupus erythematosus(SLE) whose prevalenceis in the range of 0. 5-2%, but as an initial presenter of SLE is very rare though there are sparsh literature.

SLE with Digital Gangrene: A 20 year old female presented to us as 2 month history of gangrene over right great toe which was preceded by pain in the toe, pain in knee, fatigue & loss of hair. physical examination revealed gangrene of right great toe. On further investigation patient was diagnosed as a case of SLEwith digital gangrene. Patient was treated with pulse methylprednisolone, oral steroid, pulse cyclophosphamide, mycophenolate mofetil, &hydroxychloroquine.

SLE with Spontaneous Hydropneumothorax: 20-year old male presented to emergency department with a history of fever for 2 months, shortness of breath & right sided chest pain for 2 days. On systemic examination and routine investigation patient was diagnosed as a case of right sided hydropneumothorax was established. On further evaluation of fever patient was diagnosed as a case of SLE. Patient was treated with intercostal chest tube drainage, steroids & hydroxychloroquine.

SLE with Macrophage Activation Syndrome: 15-year old female presented with fever, loss of hair, multiple joint pain, rash, bleeding from nose & gums. On general examination facial puffinace, malar rash, palatal ulcer& bleeding from gums was there. Onfurther investigation we diagnosed as a case of SLE with macrophage activation syndrome. patient was treated with pulse methylprednisolone, oral steroid, hydroxychloroquine & cyclosporine.

SLE with Thrombotic Thrombocytopenic Purpura: 62 year male presented to us with altered sensorium, persistent hiccups for 10 days. Examination finding reveled purpura and ecchymosis all over the body, fever, tender knees. On further investigation patient was diagnosed as a case of SLE-TTP. Patient was treated with pulse intravenous methylprednisolone, oral steroid & hydroxychloroquine.


  PM0064: Anti-SRP myopathy: Case series from a single tertiary center with review of literature Top


Rajiv Ranjan Kumar, Saket Jha, Aadhaar Dhooria, GSRSNK Naidu, Susheel Kumar, Shefali Khanna Sharma, Aman Sharma, Sanjay Jain, Varun Dhir; PGIMER, Chandigarh, India

Background: Signal recognition peptide (SRP) is a cytoplasmic RNA protein that translocates newly synthesized protein across the endoplasmic reticulum (ER). Myositis associated with SRP is severe and refractory, may affect all ages and is usually associated with polymyositis.

Objective: We describe the clinical features, treatment, and outcomes of anti-SRP myopathy patients from a single tertiary care center.

Methods: A retrospective, single-center, study. Patients with myositis with SRP antibody positive by immunoblot and fulfilling ENMCC criteria-2017.

Results: 6 patients (all female) were recruited; mean age 49±14. 5 years. Five patients had symptoms duration of £ 12 months. All patients presented with proximal muscle weakness; two had severe muscle weakness, two each had neck weakness, dysphagia, bulbar weakness, and muscle atrophy; and one had mild distal muscle weakness. Four patients fitted in the dermatomyositis spectrum while two were consistent with polymyositis. One patient had myocarditis, none had interstitial lung disease. Muscle biopsy was done in 3 patients; one showed necrotizing inflammation while the rest showed nonspecific myositis. The mean CPK level was 1034 ± 675 IU/L. All patients received corticosteroids with added immunosuppression (methotrexate in 5, azathioprine in 1). One patient was refractory to methotrexate, steroids, rituximab, intravenous immunoglobulins, plasma exchange, and tacrolimus and expired. On follow-up (11. 8± 7 months), five patients were doing well with 2 relapses. Ovarian cancer was seen in one patient.

Conclusion: Anti- SRP myopathy is a severe form of inflammatory myositis. Our patients had a good response (barring one) with corticosteroids and conventional immunosuppression.


  PM0065: Tuberculosis a mimicker, masquerader and response modifier to autoimmune diseases Top


Kattel Vivek, Agrawal Yamuna1, Tilwee Virend, Gupta Neetu2, Malviya Sourabh; Medanta Superspecialty Hospital,2MGM College, Indore, Madhya Pradesh, India,1BP Koirala Institute of Health Sciences, Dharan, Nepal

Introduction: Globally almost a quarter of population has latent tuberculosis and 6-8% has autoimmune diseases. Overlap between these two conditions may present with various clinical pathology.

Cases: A 42-year home maker with polyarthritis, raised ESR/CRP, HLAB27 positive and grade I bilateral sacroilitis (SI) on X-ray pelvis on high dose steroid and methotrexate was planned for TNF blockers against recurrent then refractory left sided uveitis. One month rifampicin and isoniazid for her Monteux and IGRA positive latent TB relived her uveitis and she was prospectively diagnosed as tubercular uveitis and competed ATT course. Active tuberculosis can mimic HLAB27 syndrome (uveitis, peripheral arthritis) due to high prevalence of latent tuberculosis and asymptomatic HLAB27 positive in community.

A 36-year businessman diagnosed case of HLAB27 positive Spondyloarthritis was previously treated for latent tuberculosis on adalimumab under remission presented with right knee arthritis with ESR 141mm and CRP109U/mL.Synovial tapping revealed inflammatory arthritis and patient was changed to eternacept with addition NSAIDS and methotrexate. He worsen with calf myositis not responding to broad spectrum antibiotic. A trial of ATT drug was initiated and he started to respond by the end of a week. He continued intensive phase for two months followed by eight months continuous phase of ATT regime. Post ATT his spondyloarthritis is under remission with sulfasalazine. In our case, latent tuberculosis treatment did not prevent the reactivation of tuberculosis.

Discussion: Clinical judgment on manifestation that mimics tubercular or autoimmune diseases is tough. In high latent tuberculosis geography trial of anti-tubercular for at least month followed by assessment of autoimmune disease could answers such dilemma unless we have a test to differentiate between latent and active tuberculosis.

Conclusion: A clear affordable guideline regarding investigation and treatment of latent tuberculosis does not exist for rheumatologic patients at developing world.

Keywords: Autoimmune diseases, biological DMARDS, tuberculosis


  PM0066: Immunological non responder's real or virtual phenomenon Top


Kattel Vivek, Agrawal Yamuna1; Medanta Superspecialty Hospital, Indore, Madhya Pradesh, India,1BP Koirala Institute of Health Sciences, Dharan, Nepal

Introduction: A subset of HIV people on antiretroviral therapy (ART) achieves virological suppression but poor recovery of CD4 cell termed as immunological non-responders.

Case: A 24 years intravenous drug abuser male with HCV for last three years presented as HIV positive (CD4 - 186/ml) on July 2008. Despite ZDV/3TC/EFV for six months he did not achieve immunological recovery but his viral load was below 400copies/ml. On September 2009 he presented with fever and constitutional symptoms for two weeks. On examination he was pale, icteric and had hepatosplenomegaly. Investigation revealed pancytopenia, transaminitis, hepatosplenomegaly, sterile blood culture, normal chest X ray, sputum for acid fast bacilli and PCR for mycobacterium tuberculi negative, negative rK-39, malaria negative. He had CD4 of 156/ml, HIV viral load 72copies/ml, HCV RNA 15600copies/ml. Bone marrow aspiration revealed 3+ Leishmania Donovani (LD) bodies. ARV regimen was changed to TDF/3TC/EFV and tablet Miltefosine 50 mg twice a day for 28 days was initiated. He improved clinically and parasitologically. On April 2010 his second infection of Visceral Leishmaniasis (VL) was treated with Injection Amphoteracin B. On March 2011 and August-2012 he had third and fourth episode of VL infection and was treated with Amphoteracin B plus Miltefosine and liposomal Amphoteracin B respectively. However, the fourth episode was continued with secondary prophylaxis for six months with immunological recovery (CD4 756/ml). On April 2015 his HCV was treated with 12 weeks Sofosbuvir and Daclatasvir with Rapid Viral and Sustained Viral Response.

Discussion: It has been recommended to start HCV treatment in HIV coinfection if CD4 cells are more than 200/ml. Immunological non-responders could be a challenge to initiate HCV treatment especially in limited resources setting.

Conclusion: Immunological non responders might be virtual phenomena.


  PM0067: Isolated thrombocytopenia: A rare presentation of primary Sjogren syndrome Top


Sumit Puloria, V P Pandey, Sanjay Dubey; M. G. M. Medical College and M. Y. H. Hospital, Indore, Madhya Pradesh, India

Case Report: A 30 year female came with complaints of Bleeding per vagina since 3 month & with nonpruritic purpuric rashes over both lower limb more than upper limb since 10 days. No history of fever, joint pain or any drug intake. Vitals- stable & Systemic examination was normal.

Haemoglobin -11. 2 gm%

TLC -5100/Cumm

Platelets - 9000/Cumm

P. S. - RBC microcytic hypochromic with mild anisocytosis seen, few tear drop &pencil cells. Platelets count - inadequate.

ESR24/mm

Bone Marrow aspiration- WNL

Immature platelet fraction- 52% (increased)

Ecg;Urine R/M;Usgabdomen ; Chest x ray - All are WNL

DENGUE /HBsAg/ AntiHCV/ HIV/ Widal -Negative

R. A. factor / Anti CCP/ CRP/ ANTI LKM 1/ ANTI ASMA – Negative

Thyroid profile - WNL

ANA by IFA -SPECKLED PATTERN: 2+

ANA profile by Immunoblot -Specific band for SSA/Ro60KD, SSA/Ro52KD, SSB antibodies

SCHIRMER'S TEST: POSITIVE

  • Rt. & Lt. Eye-3mm &0. 5 mm respectively
  • MINOR SALIVARY GLAND BIOPSY: evidence of focal lymphocytic sailadenitis. FOCUS SCORE – 0. 73/4mm.


Pt. was managed with pulse therapy ofI. V. Methylprednisolone for 5 days & then with oral steroids, clinically her bleeding tendencies were stopped & plateletscount recovered.

Disscussion: Primary Sjogren's syndrome is an autoimmune disease wherethere is lymphocytic infiltration of salivary and lacrimal glands. The most common clinical feature of Sjogren's is dryness of the mouth and eyes. Rarely patients can present with autoimmune cytopenias. Here we report a case of a young female presenting withisolated thrombocytopenia. Incidence is 5-15%.

Conclusion: We should be vigilant enough to look for Sjogren's syndrome in patient presenting with isolated thrombocytopenia as they can have dreaded complication if not managed on time.
Figure 1

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  PM0068: HLH in SLE: A rare presentation Top


Sumit Puloria, V P Pandey, Sanjay Dubey, Yogendra Jamra, Karuna Mujalda; M. G. M. Medical College and M. Y. H. Hospital, Indore, Madhya Pradesh, India

A 20-yr-old female presented with fever, arthralgia, and 1 -episode GTCS.

  • On examination, pulse- 80/min, respiratory rate was 16/min andBP- 110/70 mm of Hg, Temp. - 100. 1 F. General Examination reveals oral ulcers, pallor & icterus
  • Systemic examination reveals generalized maculopapular rashes, sparing nasolabial fold, oral ulcers, splenomegaly
  • Haemoglobin- 5. 7 gm%, TLC- 2000/Cumm, Platelets- 51, 000/Cumm, MCV- 85/fL, ESR- 45 mm/hr, Serum creatinine- 0. 99mg/dl, Total bilirubin- 8. 9 mg/dl, Direct Bilirubin- 6. 8 mg/dl, SGOT- 877 U/L, SGPT- 104U/L, ALP-269IU/L, Triglycerides- 528mg/dL, INR- 1. 24,
  • HIV/ANTIHCV/HBsAg- Negative
  • ANA by IFA revealed +4, Homogenous pattern
  • ANA profile by Immunoblot- ANTI ds DNA - positive
  • Serum Ferritin > 1650 ng/ml
  • ANTILKM-1 /ANTI SMA – Negative
  • Serum ceruloplasmin- 27. 2 mg/dL
  • Bone marrow aspiration- Haemophagocyte were seen.


Pt. was managed with pulse therapy ofI. V. Methylprednisolone, antiepileptics, but pt. succumb within 14 hrs.

Discussion: HLH is seen rarely in patients of SLE

  • Whenwe suspect to have acute flare up of SLE, a possibility of secondary HLH should be kept in mind
  • In our patient, she had acute flare-up of SLE, triglycerides level were raised & serum ferritin level was sent, that also came to be highly raised, her BMA was planned that revealed HAEMOPHAGOCYTES.


Conclusion: ØSecondary HLH with SLE is rare, with an estimated prevalence of 0. 9–4. 6% and when it occurs, differentiating it from lupus flare requires a high degree of suspicion and awareness of this association.

ØEarly diagnosis of HLH in the Background of SLE facilitates the timely selection of an appropriate treatment modality to prevent fatal complications.

Reference

  1. Fukaya S, Yasuda S, Hashimoto T, Oku K, Kataoka H, Horita T, et al. Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: Analysis of 30 cases. Rheumatology (Oxford) 2008;47:1686-91.
Figure 1

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  PM0069: Unmask GBS and discover “the vasculitis” Top


Adamya Gupta, Archana Verma, V P Pandey; MGM Medical College and MY Hospital, Indore, Madhya Pradesh, India

Introduction: Vasculitic neuropathy classically presents as mononeuritis multiplex, in certain patients neuropathy can be symmetric & may predate diagnosis of collagen vascular disease. such GBS if has undiagnosed association with vasculitis generally lead to poor prognosis. So if we diagnose vasculitis, treat vasculitis we treat GBS.

Materials and Methods: Case series of 23 patients came to myh in last 6 months, cases were diagnosed on basis of history, examination, routine blood biochemistry, nerve conduction studies, csf examination, & if suggestive ana was done.

Observations: we found GBS patients 82% males, 17% females young<= 35 years 73%, >35years 26% demyelinating variant 73%, axonal variant 26%

vasculitis was observed in 47% overall of which 26% were demeylinating cases & 21% were axonal variants.

66% of elderly patients (>=35 years) had vasculitis, while only 41% young patients (<35 years) had vasculitis.

40% patients with axonal variety along with vasculitis were anti- ro ab positive.

Arthralgia as a presenting complaint as well as association was seen in 8% patients overall. but every case of arthralgia with GBS had 100% specificity to be ana positive in our study.

Conclusion:

Although allof these patients appeared clinically to have GBSi. e. rapidly evolving symmetrical motor neuropathy, there is no specific laboratory test to definatively diagnose GBS. Thus there may be errors in diagnosis.

Hence several disease may mimic GBS

Thusvasculitic neuropathy should be thought as treatable cause of

  • otherwise poor prognostic GBS
  • worsening GBS
  • GBS who have electrophysiologic or lab findings atypical of GBS like


    • axonal variant GBS
    • aidpa/w arthralgia
    • severe weakness (<3/5 power grade).


Agressive workup like s. ana, msucle biopsy, nerve biopsy should be done to find/ rule out cause of treatable neuropathy.


  PM0070: Tubercular osteomyelitis of bilateral wrist joint Top


Sumit Puloria, V P Pandey, Sanjay Dubey, Ashok Thakur;

M. G. M. Medical College and M. Y. H. Hospital, Indore, Madhya Pradesh, India

A 15 year old boy came with presenting complaints of swelling and pus discharge in the left wrist: 3 year & swelling in the right wrist: 1 month

On General Examination vitals – stable, pallor was present, multiple lymph nodes enlargement seen in axilla and inguinal region.

On Local Examination: multiple firm tender skin-colored to hyperpigmented swelling over wrist, elbow & axillae along with scarring and draining sinuses.

Systemic Examination- WNL

  • Haemoglobin- 7. 6 gm%, TLC- 11, 700/Cumm, Platelets- 3. 5 lac/Cumm, MCV- 65/fL, ESR- 55 mm/hr, serum Creatinine- 0. 58mg/dl, Total bilirubin- 8. 9 mg/dl, Direct Bilirubin- 6. 8 mg/dl, ALP-203IU/L, INR- 1. 24, Serum proteins- 7. 1 mg/dL, albumin- 3. 0mg/dL
  • HIV/ANTIHCV/HBsAg/VDRL/ RA factor - Negative
  • CRP – Positive (96microgm/mL)
  • Culture & Sensitivity (Blood/Urine/Pus)- sterile
  • USG Neck & Axilla – few subcentrimetric lymph nodes seen in anterior triangle of neck, multiple enlarged Lymphnodes are seen in bilateral axillae largest measuring 2. 4. *1. 3 cm
  • USG of Left Wrist: Approximately 2. 8 x 1. 9 x 1. 8 cm heterogenous hypoechoic soft tissue mass seen on dorsal aspect of Lt. wrist joint with destruction of underlying bone seen & adjacent bone cortex appear irregular
  • MRIB/L WRIST: diffuse marrow edema noted in most of carpal bones. capitate & 4th metacarpal severely affected
  • PUS CBNAAT: MTB DETECTED; Rifampicin Sensitive
  • Pt. was managed with A. T. T. & analgesic, and is in follow up.


Discussion: Pt. came with discharging sinuses and multiple enlarged lymph nodes, early recognition of this disease and treatment can prevent joint deformity & functional disability.

Conclusion: Very rare form, constitutes about 1. 7–2% of all TB cases. A general physician was less aware of its presentation. . . Prompt diagnosis and treatment are of utmost importance to avoid the development of skeletal deformities and functional disabilities.


  PM0071: Unusual case report on muscular dystrophy Top


Lakshman Meena, Danveer Bhadu; AIIMS, New Delhi, India

A 37 year female known case of hypothyroidism on thyroxine 50 microgram tablet daily from last 6 year and hypertension on tabletamlodipine 5 mg daily from last two year came to our OPD with chief complain of proximal muscle weakness of upper and lower limb last oneand half year. Power in both upper limb and lower limb was 4/5. She also give history of unable to sit from lying down position (truncal muscle weakness). There is no history of distal muscle weakness, no significant family history, no history suggestive of CTD. On evaluation TSH level was 6mU/L. CPK and LDH level was 853 U/L & 1635 U/L, EMG was normal. During evaluation she was found rounded hypoechoic exophytic lesion (4 x 4 cm) on lower pole of right kidney. PET scan show metabolically active soft tissue mass at inferior pole of right kidney, hypermetabolism of bilateral leg muscles, paraspinal muscles and right side abdominal wall muscles with diffuse atrophy –suggestive of myositis. Histopathology of kidney mass suggestive of renal cell carcinoma for which nephrectomy was done by urology department. However, EMG was normal but due to raised CPK, LDH and PET scan suggestive of myositis &renal cell carcinoma we made a diagnosis of Paraneoplastic myositis and we started Steroid 1mg/kg in tapering dose along with Methotrexate but after 2 month there is no significant improvement. So, we plan and give Rituximab 1gm at 0, 2 weeks. Even after two and half month patient have no significant improvement in muscle weakness and CPK was 721 U/L. So we do muscle biopsy which was suggestive of muscular dystrophy probable calpainopathy.

Conclusion: We should do muscle biopsy when 1. EMG and muscle enzyme finding mismatch 2. Muscle enzyme level no significantly high 3. No response with steroid and DMARDS including biologics.


  PM0072: A case report of autoimmune cytopenias: A rare presentation of primary Sjogren's syndrome Top


V N Nagaprabu, P Velammal1, Gayathir Anand; Sakthi Rheumatology Centre Pvt., Ltd.,1PSGIMS and R, Coimbatore, Tamil Nadu, India

Primary Sjogren's syndrome is an autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands. This inflammation is thought to be caused by B-lymphocytes. The most common clinical feature of Sjogren's is dryness of the mouth and eyes, but rare complications can occur such as autoimmune cytopenia. Here we report a case of immune-mediated cytopenia's that were diagnosed to be due to Sjogren's syndrome.

Case Report: A 30-year female admitted with complaints of bleeding gums, petechial and purpuric rashes over limbs of 10 days duration.

Past history-Known hypothyroidism on thyroxine replacement therapy. History of platelet-rich plasma transfusion 2 years ago.

On examination BP-110/70 mm hg, PR-86/min, Pallor- was present

Icterus, Lymphadenopathy, and Hepatosplenomegaly were absent. ANA by IIF revealed +2. ANA profile showedAnti RO/SS-A positivity. Anti-ds DNA and anti-Smith antibodies were Negative.

The patient was treated with steroids, platelet transfusions, and IVIG.

Investigations-

Haemoglobin-6. 4 g/dl

TLC-8200 cells/cubic mm

Platelet count-4000/Cu mm

Peripheral smear-Normocytic, normochromic RBCs and normal platelets

Reticulocyte count-0. 5%

Coombs test-Negative

HIV, HBsAg and Anti HCV-Non reactive

RFT, LFT-Normal

Immature platelet fraction-55. 2%

ANA-Positive

Anti RO/SS A-Positive

Anti-LA/SS B, Anti ds DNA, Anti Smith antibody-Negative

Schirmer's test-Positive.

Discussion: Leukopenia is seen in 14-42 %, thrombocytopenia in 5-15%, and anemia in 11% of patients with Primary Sjogren's syndrome (pSS)and can manifest individually or in combination.

Treatment generally includes immunosuppressive therapy, corticosteroids and /or IVIG. Plasmapheresis for hemolytic anemia and thrombotic thrombocytopenic purpura complicating pSS is reported. Rituximab has been used as an off-label drug in lymphoma associated with Sjogren's.

Conclusion: Autoimmune cytopenia's may develop before the diagnosis of pSS, and sometimes can be life-threatening. Thus, pSS should be considered in the differential diagnosis of all patients who have otherwise inexplicable cytopenia's.


  PM0073: A rare case report of macrophage activation syndrome: A life threatening condition Top


Ganganpalli Dattaprasad, V P Pandey, Sanjay Dubey; Department of Medicine, MGM Medical College, Indore, Madhya Pradesh, India

Macrophage activation syndrome(MAS) is caused by excessive activation and uncontrolled proliferation of T-cells and well-differentiated macrophages which leads to widespread hemo-phagocytosis and cytokine overproduction, a highly stimulated but ineffective inflammatory-immune response, which can be fatal.

Case Report: A 18-year female patient with no significant past and family history presented with easy fatigability, exertional dyspnea of 1-month duration. Low grade fever, cough and nasal bleeds for 5 days.

On examination, pulse rate-100 beats/minute, BP-100/70 mm hg, respiratory rate-18 cycles/minute. On examination pallor was seen with raised JVP. Chest auscultation revealed loud S1 with pan systolic murmur at apex, bibasilar crepitations. On per-abdomen examination hepatosplenomegaly was present. On investigations pancytopenia related to vitamin-b12 deficiency was found and treated with vitamin-b12 supplements, blood and PRP transfusion. Further evaluation showed features of Macrophage activation syndrome with autoimmune thyroiditis and treated with IV methylprednisolone, IVIG, thyroxine therapy and oral immunosuppressants.

Patient deteriorated and developedright upper limb DVT which later complicated as pulmonary thromboembolism.

Bone marrow biopsy-Normoblastic marrow with marked histiocytes and hemophagocytes.

Discussion: MAS is usually seen with systemic juvenile idiopathic arthritis(JIA), still's disease, SLE, Kawasaki disease and characterized by fever, hepatosplenomegaly, generalized lymphadenopathy, CNS dysfunction, and hemorrhagic symptoms. Laboratory abnormalities include pancytopenia, increased levels of ferritin, liver enzymes, LDH, triglycerides, D-dimers, and soluble interleukin-2 (IL-2) receptor and decreased fibrinogen levels. Histopathologic feature of MAS is the accumulation of well-differentiated macrophages exhibiting hemophagocytic activity in bone marrow biopsy specimens or aspirates.

Conclusion: Early recognition and aggressive therapy are critical in MAS. All pancytopenia patients should be evaluated for MAS.
Figure 1: Normoblasticmaturation of bone marrow with many histiocytes (blue arrow), erythrophagocytosis (Yellow arrows), blood vessels proliferatlion (red arrows) and megakaryoacyte (black arrow)

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Table 1: Lab values of the two cases

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  PM0074: Vasculitis in tuberculosis: A drug or a bug? Top


R Pratheesh Chandran, M Sudhagar; Pondicherry Institute of Medical Sciences, Puducherry, India

Case Report: A 64-year-old male diagnosed to have active pulmonary TB (Left lower lobe cavitating pneumonia)-sputum positive started on Anti tuberculous therapy. He was started on modified anti tubercular therapy due to alcohol induced hepatitis. His hepatitis resolved in two months. (He was on Isoniazid, Pyrazinamide, Ethambutol and Levofloxacin). Meanwhile patient developed skin rashes-erythema multiforme). Following he was started on steroids and all ATT were stopped (Dechallenged). He was then started with Ethambutol, Amikacin, Levofloxacin. As his hepatitis resolved he was started on Rifampicin and also Pyrazinamide except for Isoniazid (Rechallanged). The skin lesion showed a tremendous improvement after withdrawal of Isoniazid. Hence Isoniazid was not rechallenged. Although a rare entity, Isoniazid among ATT should be considered as a potential cause of Drug induced Vasculitis.

Discussion: Tuberculosis as a disease and also anti tubercular drugs can cause vasculitis. Anti-tubercular drugs induced vasculitis though a known entity Isoniazid induced vasculitis remains rare. This is a case of ANCA positive vasculitis, where drug withdrawal and early initiation of steroids remains the treatment of choice.

Conclusion: With this case report, we aim to create awareness and vigilance about rare, but potentially serious drug reaction. If any ATT induced vasculitis is suspected, Isoniazid as a cause of it should also be considered.
Figure 1

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  PM0075: Atypical presentations of takayasu arteritis in young females: Case reports Top


G Mounika Reddy, Bhowmik Meghnathi, Raghurama Reddy; Prathima Institute of Medical Sciences, Karimnagar, Telangana, India

Case Report 1: A 23-Year-oldprimi presented to ER with loss of fetal movements for 2 days. On examination left upper limb pulses were absent, right upper limb B. P 220/110 mm. Hg, left upper limb B. P 110/70 mm. hg, carotid and abdominal bruit heard on auscultation. She was evaluated and investigated further and found to have Intrauterine death of fetus secondary to Takayasu arteritis.

Discussion: TAKAYASU ARTERITIS is commonly associated with first trimester abortions and IUGR. We report a rare occurrence of Intrauterine death with TA.

Case Report 2: A 24-Year-old female known case of TAKAYASU ARTERITIS and on regular follow-up has presented to OPD with redness, watering of right eye for which she was referred to ophthalmology OPD. she was diagnosed with phlyctenular conjunctivitis and was started on antibiotic and steroid eye drops for which patient has not responded. She was further investigated and Mantoux test was positive. She was started on ATT andresponded well.

Discussion: TAKAYASU ARTERITIS have been shown to have association with Tuberculosis with HSP-60kDa as the probable pathogenesis. Phlyctenular conjunctivitis though a common presentation has been rarely presented secondary to tuberculosis. We report a case of phlyctenular conjunctivitis with mantoux positive as a rare presentation of TB, co-existing with TA.

Conclusion: We hereby report 2 cases of Takayasu in young women with varied presentation. Though IUGR and first trimester abortions are noted in Takayasu pregnancy, IUD occurence is rare (<5%). Phlyctenular conjunctivitis though associated with TB, seen as a rare co-association with TA. As there is limited evidence of rare associations of TA, there should be high clinical suspicion and further research has to be done in this field.
Figure 1: Case 2: Phlyctenular coniunctivitis

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  PM0076: Hypokalemic periodic paralysis: A rare presentation of primary Sjogren's syndrome Top


Ganganpalli Dattaprasad, V P Pandey, Sanjay Dubey; Department of Medicine, MGM Medical College, Indore, Madhya Pradesh, India

Primary Sjogren's syndrome is characterized by dryness of mouth and eyes. Distal RTA is a rare presentation that can cause hypokalemic-periodic paralysis.

Case Report: A 33-year female with no significant past and family history admitted with progressive quadriplegia for four days.

On examination, pulse rate was 78 beats/ min, Blood pressure 110/70 mm of hg, Respiratory rate 16 cycles/ minute. Motor examination revealed flaccid quadriplegia with power 2/5 in all four limbs with sluggish reflexes and flexor plantars. ECG showed prominent U waves. ABG was suggestive of normal anion gap metabolic acidosis with pH 7. 085. LFT, RFT, and TFT were normal. NCS ruled out AIDP.

Retrospective history revealed dryness of eyes and mouth with foreign body sensation in eyes since past few years. Treated with intravenous potassium chloride and the patient improved.

Discussion: Distal RTA characterized by an inability of the distal nephron to acidify the urine leading to hyperchloremic, hypokalemic, metabolic acidosis with a normal serum anion gap and urine pH greater than 5. 5.

The common cause of distal RTA is diminished H+-ATPase activity with resultant hypokalemia leading to urine pH persistently >5. 5. The most common histological renal lesion in RTA with Sjogren's syndrome is interstitial nephritis characterized by lymphocytic and plasma cell infiltrates surrounding renal tubules and by hypergammaglobulinemia leading to distal renal tubular dysfunction and loss of H+-ATPase pumps in the intercalated cells. Steroids are useful in refractory cases.

Conclusion: All acute flaccid paralysis patients should be looked for autoimmune causes. < 5% patients with Sjogren syndrome present with hypokalemic periodic paralysis and distal RTA.
Figure 1: ECG showing features of hypokalemia

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Table 1: Investigation details

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  PM0077: Rarer presentation of a rare disease in pediatric population Top


S Rajesh , G Asok Kumar, Anu K Vasu; Kerala Institute of Medical Sciences, Thiruvanathapuram, Kerala, India

A 13-year-old boy was admitted with history of fever for 2 weeks with erythema over lips and tongue with no lymphadenopathy. Blood investigations were normal except for high ESR 65mm/hour and CRP 31mg/L. Echocardiogram was normal, chest X ray showed haziness over the lower zone of the left upper lobe and hence treated with antibiotics for 7 days with resolution of fever. On follow up at 6 weeks, patient was asymptomatic but had persistent high ESR and CRP. Ultrasound showed mild hepatosplenomegaly and echocardiogram revealed thickened posterior pericardium with minimal pericardial effusion. HRCT chest showed multiple mediastinal lymphadenopathy, consolidation of left upper lobe and an anterior mediastinal mass probably thymic mass. Repeat echocardiograph done at 10th week showed coronary artery aneurysm, pericardial effusion, thickening around proximal aorta suggestive of aortitis and mild impairment of LV function. 18F-FDG PET/CT study revealed metabolically active mediastinal adenopathy, thymic and pericardial infiltrates and a metabolically active right iliac bone lesion. Mediastinoscopic lymph node biopsy was suggestive of benign reactive lymph node with plasmacytosis. On immunohistochemistry an increase in IgG4 positive plasma cells were identified. Ig G4 positive plasma cell count per high power field was 30-35. The ratio of IgG4 positive to CD 138 positive plasma cells was 35-40%. Serum Ig G4 level was elevated, 292mg/dl. Treatment was initiated with steroids tapered gradually with normalization of inflammatory parameters and repeat cardiac MRI showed normal sized coronaries.

Discussion: This case presenting with fever and then during immediate follow up evaluation revealing a rarer cardiac involvement in the form of coronary artery aneurysm with confirmatory histopathological features of IgG 4 disease. IgG4-related cardiac manifestations include involvement of the myocardium, 3rd degree heart block due to SA node involvement, cardiomegaly, constrictive pericarditis, pseudotumours around coronary arteries, valvular regurgitant lesions involving the aortic and mitral valves.


  PM0078: Thionamide induced myositis- report of a case and systematic review of literature Top


Koshy Nithin Thomas, Sujata Ganguly, Durga Prasanna Misra, Ramnath Misra, Latika Gupta; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Discussion: A 31-year-old lady presented with rashes and muscle weakness 2 weeks after taking Carbimazole for Grave's disease. She had Gottron's papules and a Heliotrope rash with proximal myopathy. Investigations revealed elevated muscle enzyme levels, positive ANA and an abnormal EMG. Unlike most earlier case reports of Thionamide induced myositis where cessation of the drug has resulted in resolution of symptoms, her muscle weakness continued to worsen. She was given I131 therapy for her Grave's disease, and her muscle weakness and rashes gradually improved with immunosuppressive therapy over six months.

Conclusion: Though rashes are a well described adverse effect of Thionamides, the development of a rash in the setting of muscular symptoms is not common in literature (n=13). Rarely, there have been case reports of Anti-thyroid drug induced lupus[1] as well as ANCA vasculitis.[2]. An “antithyroid arthritis syndrome”, which comprises of fever, rash, pruritus, arthralgia and myalgia has been reported. However, muscle weakness in not a feature of this syndrome.[3] The development of frank dermatomyositis induced by Thionamides has not been previously reported in adults.

References

  1. Wang LC, Tsai WY, Yang YH, Chiang BL. Methimazole-induced lupus erythematosus: A case report. J Microbiol Immunol Infect 2003;36:278-81.
  2. Gunton JE, Stiel J, Caterson RJ, McElduff A. Clinical case seminar: Anti-thyroid drugs and antineutrophil cytoplasmic antibody positive vasculitis. A case report and review of the literature. J Clin Endocrinol Metab 1999;84:13-6.
  3. Shabtai R, Shapiro MS, Orenstein D, Taragan R, Shenkman L. The antithyroid arthritis syndrome reviewed. Arthritis Rheum 1984;27:227-9.



  PM0079: Rare case of coexisting polyarteritis nodosa and antiphospholipid syndrome: A diagnostic and therapeutic challenge Top


W A E Udeshika, R M Wickramarachchi, M De Silva1,2Senior Registrar in Rheumatology,3Consultant Rheumatologist

Case: A 50-year-old man with hypertension and type 2 diabetes mellitus with macro vascular complications, 6 months ago presented with high grade fever, constitutional symptoms, bilateral upper and lower limb rash with reduced conscious level for 3-weeks duration.

On examination he had a vasculitic rash in upper and lower limbs, dry gangrene in left second and third toes with impalpable peripheral pulsation. Further he had distal, motor sensory peripheral neuropathy and reduced consciousness with GCS 12/15 with no signs of meningism or focal neurological signs.

Hematological investigations revealed neutrophil leukocytosis, anemia, mild thrombocytopenia, and elevated ESR and CRP. Septic screening, HIV, ANA, ANCA, serum cryoglobulins were negative. MRI brain, MRA, MRV revealed acute infarctions in left parietal lobe with small vessel disease. Skin biopsy revealed medium vessel vasculitis with fibrinoid necrosis and CT mesenteric angiogram revealed vasculitic changes in mesenteric vessels. He was treated with IV methyl prednisolone and had a dramatic response. Ward stay was complicated with extensive thrombosis in pelvic veins. His antibodies for Lupus anticoagulant became positive, 12 weeks apart and started on lifelong warfarin therapy for Antiphospholipid syndrome. Despite aggressive immunosuppression with cyclophosphamide, he remained bed bound with high morbidity.

Discussion: This patient with evidence of medium vessel vasculitis as evident by fibrinoid necrosis in the skin biopsy with mesenteric and neurological manifestations warrants treatment with aggressive immunosuppression. Coexisting stroke and extensive thrombosis in the pelvic veins raised a therapeutic challenge of starting anticoagulation given after weighing benefits over risks.

Conclusion: Antiphospholipid syndrome(APLS) secondary to systemic vasculitis is extremely rare, reported roughly 20 cases in literature. This patient who had biopsy proven Polyarteritis nodosa had unusual presentation with mild thrombocytopenia, extensive pelvic and abdominal vein thrombosis and positive lupus anticoagulant assay, which can only be explained by coexisting APLS.


  PM0080: Retinal vasculopathy in children with systemic lupus erythematosus: Report of 2 cases Top


Sandesh Guleria, Ankur Jindal, Dharmagat Bhattarai, Anirudha Aggarwal, Bhartendu Sharma, Ramandeep Singh, Deepti Suri, Surjit Singh; Postgraduate Institute of Medical Education and Research, Chandigarh, India

Case Description: Patient 1: A 9-year-old boy presented with fever and vomiting for 15 days. Examination was unremarkable except for pallor. Laboratory investigations revealed anemia and thrombocytopenia.

During fundus examination, he was found to have cotton wool spots in both eyes. He was further worked up for lupus. ANA (4+ speckled pattern) and anti-dsDNA were positive. Fundus FFA showed capillary 'drop-out' and leakage of dye. These findings were suggestive of lupus retinitis. His refraction testing revealed low vision in both eyes (perception of light at 3 feet).

He was given 5 daily pulse of injection methylprednisolone followed by oral prednisolone. Six, monthly injection cyclophosphamide (500 mg/m2/month) were also administered followed by azathioprine. He was also initiated on hydroxychloroquine, warfarin and aspirin. He had gradual improvement in vision and at 1½ years of follow up, his vision has improved to a visual acuity of 6/36 in both eyes. Fundus examination showed significant reduction in cotton wool spots and repeat FFA has revealed no leakage.

Patient 2: An 8-year-old boy presented with fever and rash for 1 month. Examination revealed malar rash and oral mucosal ulcers. Laboratory investigations showed lymphopenia, positive ANA and anti-dsDNA. His fundus evaluation showed cotton wool spots. FFA was suggestive of capillary 'drop-out' and leakage of dye. Visual acuity was normal.

He was given 5 daily pulse doses of injection methylprednisolone followed by oral prednisolone. Six monthly pulse doses of injection cyclophosphamide were also administered. He was later put on mycophenolate mofetil. At 1. 5 years of follow up, he has quiescent disease with normal fundus examination.

Discussion and Conclusion: Retinal vasculitis is a serious ocular manifestation of lupus. All children with lupus should have detailed ophthalmological evaluation for this potential complication. Children with vaso-occlusive retinopathy need aggressive immunosuppressive therapy and the prognosis remains guarded.


  PM0081: Pancreatitis in SLE Top


N Rishabh, C Balakrishnan, Y Sandeep, S Sameer; PD Hinduja Hospital and Research Center, Mumbai, Maharashtra, India

SLE is a disease with protean manifestations. Pancreatitis though rare does occur and has been thought as a complication of drug treatment rather than disease. However recent reports including onset of lupus with pancreatitis suggest that this may be manifestation of an autoimmune process.

We have come across 5 patients with lupus pancreatitis.

All patients were females in second and third decade of life and presented with abdominal pain and vomiting.

Labs showed low C3 and raised amylase and lipase and positive ANA and dsDNA and imaging showed bulky diffuse pancreatitis. All patients were treated with steroids and use of steroid sparer.

Discussion: Lupus Pancreatitis is a rare entity with incidence of 0. 4-1. 1/1000 cases of SLE.

Various mechanism includes vasculitis microthrombi and intimal thickening.

Carries high mortality rate of 27% higher than non SLE associated pancreatitis.

Although uncommon with lupus suspect pancreatitis if a patient presents with vomiting abdominal pain and the need to rule out other causes. Pancreatitis may be an initial manifestation hence early recognition is vital since it is associated with high mortality rates.


  PM0082: Gouty arthritis in a premenopausal woman: An unusual case Top


Suraj Chaudhari, Lubna Khurshid, Sanjiv Kapoor, Anand N Malaviya; Department of Rheumatology, ISIC Superspecialty Hospital, Vasant Kunj, New Delhi, India

Case Report: A 54 year old woman presented for the first time with 8 days of acute inflammatory arthritis of the knees, ankles, right elbow, and low-grade fever for 2 days. She had intermittent, asymmetrical arthritis involving large and small joints for the past 20 years; treated as seronegative RA with glucocorticoids and NSAIDs with transient relief and repeated flares in different joints. Her vital signs were normal, Cushingoid face, swollen and tender knees, ankles and the right elbow with subcutaneous nodules and gross deformities in several hand joints with wasting of quadriceps. Hand radiograph showed damage and deformities]. The examination of the other systems was unremarkable.

Investigations: High ESR 117 mm, Hb-8. 9 gm/dL, WBC 7490/cmm, platelets 1, 52, 000 /cumm. Serum Creatinine - 1. 02 mg/dL, ALT/AST: 19/34 units/ml, serum uric acid 12. 4 mg/dL, Urine R/M showed monosodium urate crystals, RF and ACPA -negative. The right knee joint synovial fluid showed negatively birefringent MSU crystals seen in polarised light microscopy. Test hypoxanthine-guanine phosphoribosyltransferase (HGPRT) and 5-phosphoribosyl-1-pyrophosphate (PRPP) enzyme could not be done.

Discussion: Gouty arthritis is mainly a disease of males > 40 years of age. Therefore, it considered most unusual in women in reproductive age except if they: (i) Have deficiency HGPRT or increased levels of PRPP enzyme; (ii) Are on long-term cyclosporine treatment (iii) Have chronic kidney disease. Diagnosis is based upon clinical characteristics and demonstration of MSU crystals in synovial fluid. Targeted treatment with SUA to be kept < 5 mg/dL over long-term is effective.

Conclusion: Gouty arthritis should be considered as one of the possibilities in an asymmetrical seronegative oligo-polyarthritis with acute intermittent attacks. Presence of subcutaneous nodules may also suggest the possibility of gouty tophi. Such patients must be carefully investigated for gouty arthritis because it is a treatable condition.


  PM0083: Connective tissue diseases with rare associations: Nightmare for rheumatologists Top


Vaijayanti Joshi, SmitaUpadhye, Sameer Melinkari, Sameer Jog, Sujit Jagtap; Deenanath Mangeshkar Hospital and Research Centre, Pune, Maharashtra, India

Background: Managementofconnective tissue disorders(CTD)likesystemic lupus erythematosis (SLE), sclerodermais always achallenge for rheumatologists. Therearevaried multisystemic manifestationssometimes CTDs show rare associations with macrophage activation syndrome(MAS), thrombotic thrombocytopenic purpura(TTP), neuromyotoniaand become a nightmare for management.

Objective: Toshare experience of 3 cases, 2 of SLE, one presented with TTP, 1 withMAS. 1 case of scleroderma presented withneuromyotonia. To discuss management of the conditions.

Methods: CASE 1:39 YR female known SLEonHCQS, leflunomide for her arthritis presented with generalized weakness, anemia and jaundice, initially thought of leflunomide toxicity but after evaluation turned out to have TTP. She underwent plasmapheresis and rituximab infusions and showed a significant recovery.

Case 2: 26yr male known case of SLE presented with high grade fever, cytopenia and splenomegaly. He was extensivelyworked up for infections finallywas diagnosed to have MASsecondary to SLE and treated with high dose steroids and rituximab. He responded dramatically. He remained in remission for 3 years however showed a relapse and managed on same line.

Case 3: 44yr male diagnosed as systemic sclerosis presented with severe backache and myalgia followed by spontaneous persistent muscle twitching of calves, thighs and arms. He developed severe insomnia and restlessness. EMG confirmed it to be neuromyotonia and he had voltage gated potassium channel(VGKC) antibodies. He responded well to plasma exchanges and immunosuppression.

Results and Conclusions: The literature shows few case reports and series sharing CTDs with those rare associations. Although etiologies are elusive certain autoimmune mechanisms may be shared and provide basis for their associations.

Timely diagnosis and aggressive treatment are needed as they can be life threatening.

Keywords: SLE, TTP, MAS, neuromyotonia, scleroderma


  PM0084: Acute liver failure in a patient with SLE and autoimmune hepatitis Top


Anjana G Varier, C E Eapen, Philip Joseph, John Mathew; Christian Medical College, Vellore, Tamil Nadu, India

Case Report: A 30 year old female, a case of SLE with autoimmune haemolytic anemia diagnosed 10 years back presented to us with history of jaundice and vomiting of 2 weeks duration. On evaluation she was found to have acute severe hepatitis. She was diagnosed with autoimmune hepatitis after excluding infection, drug induced liver injury and Budd Chiari syndrome. She was started on steroids but her disease rapidly worsened to fulminant hepatic failure. A decision for urgent liver transplantation was made after multidisciplinary discussion. An orthotopic liver transplantation was done following which she recovered completely. Her disease activity is currently well controlled on the immunosuppression for liver transplantation and she is asymptomatic since then.

Discussion: Co-occurrence of SLE and Auto Immune Hepatitis is rare. It is also rare for autoimmune hepatitis to result in acute liver failure. The important issues in management of Acute Liver Failure by Autoimmune Hepatitis are: (A) establishing an appropriate diagnosis; (B) evaluation of risks and potential benefits of immunosuppressive therapy; and (C) urgent consideration for Liver Transplantation. Emergency Liver Transplantation becomes crucial if responses toimmunosuppressants are not confirmed promptly after introduction. We report a case of auto immune hepatitis who presented to us 5 years back who had rapidly worsened to fulminant hepatic failure. Emergency intervention in the form of liver transplantation within 10 days prevented the patient from succumbing to her disease. The ongoing immunosuppression for transplant has kept her clinically and serologically quiescent for the autoimmune disease.

Conclusion: Prompt diagnosis of autoimmune hepatitis and early evaluation of its response to immunosuppressants with early decision for liver transplantation can improve the mortality and morbidity of these patients.


  PM0085: Osteomalacia: Mimicking the myopathy Top


Sandeep Nagar, Debaditya Roy, Uma Kumar; Department of Rheumatology, AIIMS, Delhi, India

Case Report: A 16 years girl presented with history of pain in both upper limb for 1. 5 years, started proximally then progressed to distal associated with weakness. She was having bilateral chest pain for 6 months, pain in both arms and back pain for 1 month. Pain was persistant on rest and activities, intensity 10/10 (VAS), with nocturnal awakening, butock pain, difficulty in sitting and squatting. this pain used to relieve with analgesics. She had history of seizure disorder at the age of 9 for which she received valproic acid for 6 months.

On examination her gate was antalgic, tenderness over chest wall. muscle power was 5/5 after alleviating pain. She had no history suggestive of any rheumatic or neurological disease.

Her hemogram, liver and kidney functions were normal, with normal electrolytes, thyroid profile, vit D, B12, folic acid. Viral markers, ENA profile ESR, CRP, CPK, LDH, acid alpha glucosidase, NCV, EMG all were normal. serum total Ca was 9. 6 mg/dl, PO4-3. 4 mg/dl, ALP-146 IU/L. Her xraypeviswasdone which showed looser zones and hazy sacroiliac joint margins.

On the basis of clinical features and radiological findings diagosis of osteomalacia was made then she was started on calcium and vit D supplementation (RDA) and analgesics.
Figure 1

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Discussion: Osteomalacia means soft bones. It occurs when there is insufficient mineral or osteoblast dysfunction so that osteoid does not mineralize properly. Decreased vit D is the most common cause of osteomalacia, some inherited conditions that affect mineralization can also be responsible. Usually symptoms and blood tests are enough to make the diagnosis but sometimes x-ray can show up the changes.

Conclusion: Presenting this case of oasteomalacia in which diagnosis was delayed in suspicion of neuromuscular or rheumatologic disorders.


  PM0086: IgG4 related disease with tubulointerstitial nephritis: A case report Top


Daisy Doley, Mandeep Bindra, Jennifer Prabhu, John Mathew; Christian Medical College, Vellore, Tamil Nadu, India

Background: IgG4- related disease (IgG4-RD) is an immune mediated, fibroinflammatory condition with multisystem involvement. It is characterised by storiform fibrosis, obliterative phlebitis and dense lymphoplasmacytic infiltrate of affected organs on biopsy. IgG4-tubulointerstitial nephritis (IgG4-TIN) is the major manifestation of renal involvement in IgG4-RD, leading to acute or chronic kidney dysfunction. The mainstay of treatment is corticosteroids.

Case Presentation: We describe here a 53 year old gentleman diagnosed as a case of chronic kidney disease for 1 year who presented with bilateral parotid involvement and submandibular swelling for which he was referred. Renal biopsy demonstrated plasma cell rich interstitial inflammation with eosinophils in tubulointerstitial compartment. Immunofluorescence for IgG showed tubular basement membrane, interstitial and Bowman capsule granular staining. IgG4 staining shows 35 IgG4(+) cells/40x field. IgG4/IgG(+) plasma cell ratio was 30%. Biopsy from left parotid gland showed dense lymphoplasmacytic infiltrate with patchy fibrosis consistent with a probable diagnosis of IgG4 relate disease.

Discussion: The hallmark of IgG4-RD is dense lymphoplasmacytic infiltration rich in IgG4 secreting plasma cells, resulting in storiform fibrosis and obliterative phlebitis. Similar lesions have also been described in other organs with varying manifestations. TIN is the most common renal manifestation of IgG4 related renal disease. The pathogenesis of IgG4-RDremains poorly understood. Differential diagnoses of IgG4-RD include malignancy, connective tissue diseases, vasculitisand other infiltrative diseases. Corticosteroids are the first line of treatment and response is dramatic. we intend to put the HPE pictures of both biopsies andIgG4 plasma cell staining in the presentation.

Conclusion: Patient was initiated on steroid therapy and showed good clinical response to therapy. He has been kept on follow up. IgG4-TIN must be kept as a differential diagnosis in elderly patients prrsenting with renal dysfunction.

Keywords: IgG4-related disease, Tubulointerstitial nephritis, Parotid involvement, Corticosteroids


  PM0087: Acute lupus pneumonitis: Atypical presentation of prototypical disease Top


Ankur Dalal; The Sarvajanik Medical Trust Hospital, Surat, Gujarat, India

Case Report: A 21 year female received on non-invasive ventilator support with provisional diagnosis of acute viral haemorrhagic fever probably dengue due to short history of high fever, headache, arthralgia, skin rash, thrombocytopenia, elevated transaminases for 1 week and acute respiratory failure (bilateral patchy-inhomogeneous perihilar+lower-zone opacities on chest x-ray-Extensive pneumonitis/ARDS, PO2-60mmHg) for 1 day. Patient had history of blood transfusion (haemoglobin-7. 2gm %) before admission. On investigations; WBC, creatinine, procalcitonin, LDH, CPK, Dengue duo, S. Widal, HIV/HBsAG/HCV, coomb's test, DIC profile, USG abdomen-pelvis, and 2D-echo were normal. Due to breathlessness, anemia, thrombocytopenia (1. 13 Lac/cumm), very high ESR (90 mm/hr), and normal CRP the patient was investigated by keeping in mind differentials like acute lung injury (ALI) secondary to viral or atypical bacterial infection, transfusion related ALI, secondary haemophagocyticlymphohistiocytosis, systemic autoimmune connective tissue diseases (AICTD) and systemic vasculitis. However, further work-up ultimately lead to diagnosis of systemic lupus erythematosus (SLE) presented with acute lupus pneumonitis (LP) with subclinical neurological involvement.

Investigation Results: Urine protein0. 48 gm/24-hr Sputum/Blood culture, Ferritin, ANCANegative HRCT-lungBilateral perihilar patchyconsolidation/minimal pleuraleffusion-Atypicalpneumonitis/ARDS MRI-brainDemyelination/Encephalitis C3/C4Low/Normal ANASpeckled+3 ANA-profileAnti-Sm+3/Anti-RNPsm+3/Anti-dsDNA+3/Anti-Nucleosome+3/Anti-histone+2 Patient was started on methylprednisolone and cyclophosphamide pulse therapy and responded dramatically (her hypoxemia and chest x-ray were normalized within 3 days). Patient was discharge on oral prednisolone 1mg/kg in tapering schedule and hydroxychloroquine. Later azathioprine was added as steroid sparing.

Discussion: Acute LP is a rare manifestation of SLE with high mortality and can be very difficult to distinguish from other differentials (especially infection) on first presentation at disease onset. Early diagnosis and treatment with steroids are essential for better outcome.

Conclusion: High index of suspicion of LP, timely investigations, and early treatment is the key for favorable prognosis in patients presented with this atypical lethal manifestation of prototypical AICTD - SLE.


  PM0088: Amyloidosis in rheumatoid arthritis: A learning experience Top


Kattel Vivek, Riyaz Asad, Malviya Sourabh; Medanta Superspecialty Hospital, Indore, Madhya Pradesh, India

Introduction: A amyloidosis is a disorder characterized by the extracellular tissue deposition of fibrils (serum amyloid A protein). Amyloidosis in inflammatory arthritis is associated with increase morbidity and early mortality.

Case Summary: A 58 years female known case of seropositive Rheumatoid arthritis under three DMARDS presented with sudden rise in creatinine (1. 7 mg/dl to 3. 06mg/dl), hematuria, granular cast and albuminuria (1. 27gm/24hours). On evaluation her blood pressure was 140/90mm/Hg with 7 tender joints and 3 swollen joints. Besides significant blood investigations were raised ESR (100mm/hr) and CRP (10mg/dl), reversal of serum protein (Serum albumin 3. 2g/dl, serum globulin 4. 2g/dl) and anemia (Hb 9. 2gm/dl). In view of rapid rising creatinine renal biopsy was secured favoring diagnosis of crescent nephritis. Biopsy revealed amyloid deposit in glomeruli and vascular region with acute patchy injury over chronic tubule-interstitial changes. She was treated with rituximab induction dose followed by two six monthly doses. She has improvement on creatinine (1. 5mg/dl) and urine (microscopic examination protein trace).

Discussion: Risk of developing amyloidosis in RA is related to duration (mean duration 15. 4years), high disease activity, seropositive, extraarticular manifestations and poorly controlled rheumatoid. RA is common cause of AA Amyloidosis predominantly involving kidneys followed by gastrointestinal system and cardiomyopathy. Renal manifestation depends upon deposit site (vessels, glomerulus and interstitial). Glomerular involvement in form of massive proteinuria and rising creatinine is common clinical presentation among more than 90% cases. Tissue biopsy is diagnostic test. One forth to half of subclinical amyloid fat deposit on abdominal biopsy only develop clinical amyloidosis. Among asymptomatic joints RA high ESR and CRP can be clue of amyloidosis. Biological DMARDs (Rituximab) can be promising to control the primary disease and renal morbidity.

Conclusion: Biological DMARDs have better outcome with secondary amyloidosis in RA.

Keywords: Amyloidosis, rheumatoid arthritis, rituximab


  PM0089: A case of limited cutaneous systemic sclerosis with decompensated chronic liver disease Top


Karthik Harisankar, N G Taruni Devi, N Santa, K Romeo Singh; Regional Institute of Medical Sciences, Imphal, Manipur, India

Case Report: 55 year old female came with complaints of fatigue, occasional pruritus and typical salt and pepper pigmentationin the anterior aspect of forehead and anterior part of leg. She also had abdominal distension with history of constipation and was drowsy at presentation. She had history of hypothyroidism on medication and was recently detected to be diabetic. LFT showed raised bilirubin and alkaline phosphatase. ANA was positive with 1 : 1280 endpoint titre. ENA screen showed positive anti centromere protein B. Skin biopsy was confirmatory of scleroderma. USG showed coarse liver echotexture with ascites. Viral markers were negative. Autoimmune hepatitis panel showed a positive Antimitochondrial antibody and rest of the antibodies were negative. A diagnosis of limited cutaneossysytemic sclerosis with decompensated chronic liver disease with ascites and hepatic encephalopathy was made. Etiology of chronic liver disease was attributed to Primary biliary cirrhosis in view of raisedALP, positive AMA and after exclusion of other causes of chronic liver disease. The possibility of a backdrop of NAFLD was also considered. Patient was managed with immunosuppresants and ursodeoxycholic acid.
Table 1: Laboratary parameters

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Discussion: In systemic sclerosis, liver is rarely affected but occurrence of primary biliary cirrhosis is found to coexist in approximately 2 percent cases. This association is called as Reynolds syndrome. Pateints with Scleroderma and chronic liver disease are prone to Hepatic encephalopathy due to bowel dysmotility and bacterial overgrowth.

Conclusion: Work up for liver disease also should be undertaken in a case of systemic sclerosis and the possibility of coexisting primary biliary cirrhosis has to be considered.


  PM0090: Congenital insensitivity to pain with anhydrosid Top


Anuja Sapre; Kalawati Saran Children Hospital, New Delhi, India

Background: CIPA(congenital insensitivity to pain with anhydrosis) is charaterized by insensitivity to pain, inability to sweat, self mutilatingbehaviour and mental retardation. It is very rare.

Case Presentation: 8 year old with complaints of recurrent boils and pustules and recurrent osteomyelitis that required above knee amputation of right lower limb. The child was DHR positive. Physical examination revealed no withdrawl to pain stimulus. His parents were cousins. There was death of a younger sibling with similar complaints at 3 years of age. The diagnosis of CIPA was confirmed on biopsy.

Conclusion: No cure is available. Preventive approach remains the only possible treatment. Early diagnosis and specific management could result in reduction of frequency and severity of complications.


  PM0091: Polymyxin B induced skin hyperpigmentation in a SLE patien Top


Sarath Jakka, Pradeep Kumar; Narayana Health City, Bengaluru, Karnataka, India

A 40 year old man with history of hypothyroidism presented with the complaints of breathlessness and hemoptysis and it was diagnosed to be due to chronic pulmonary thromboembolic pulmonary hypertension. His blood tests revealed anti phospholipid (APLA) syndrome with positive anticardiolipin antibodies IgM and IgG. Direct Coombs test was also positive. ANA (IF) was negative but complement C4 was low. He then developed abdominal pain with CT scan showing features of bowel vasculitis in jejunum, terminal ileum and cecum. Provisional diagnosis of SLE was done with secondary APLA and bowel vasculitis. He responded well to IV methyl prednisolone and maintained on oral prednisolone and hydroxychloroquine. He then underwent pulmonary thromboembolectomy successfully but the post-operative period was complicated by pneumonia with gram negative MDR Klabsiella septicemia sensitive to polymyxin B and Colistin. Polymyxin B was chosen to be given for 14 days at the dose of 7. 5 lakh units twice a day. Within a week after starting antibiotics, his skin colour darkened dramatically and it persisted for more than 2 months after. We stopped his hydroxychloroquine and maintained on low dose prednisolone and warfarin. His general condition was stable and once his INR is stablised we will be introducing mycophenolate mofetil.

Learning Point: Patients with SLE who already are prone for skin photosensitivity and also taking hydroxychloroquine which also slowly causes skin hyperpigmentation, should avoid polymyxin b injection if a suitable alternative is available.


  PM0092: Granulomatosis with polyangiitis presenting as gastric outlet obstruction: A case report Top


S Bhatt, V Vasdev, Ramakant, A Kumar; AHRR, Delhi, India

A 32-year-old lady presented with 4 months' history of insidious onset epigastric pain, vomiting and abdominal distension, aggravated by meals. She also developed intermittent fever, dry cough and significant weight loss (14 Kg over last 4 months). She denied any history of gastrointestinal bleeding, hemoptysis, breathlessness or ENT manifestations. Evaluation revealed normocytic normochromic anemia (Hb 8. 9 g/dl), normal renal function and urine analysis. Chest imaging showed presence of cavitatory pneumonia in bilateral lungs and endoscopy revealed presence of ulcero-proliferative growth at GE junction. Histopathology of the lesion showed polypoidal hyperplasia with mixed inflammatory infiltrate in lamina propria and mucosa with absence of atypia or malignant cells. Work up for chronic infective and malignant etiology, including malignancy markers and bone marrow study was negative. Further evaluation revealed c-ANCA positive with elevated anti-PR3 titres. She underwent CT guided FNAC from lung lesion which revealed presence of non-caseating necrotizing granulomas. She has been managed with intravenous pulse methyl prednisolone followed by eight Cyclophosphamide pulses as per EUVAS regimen with good response to treatment. She has remained afebrile with improvement in gastrointestinal symptoms, weight gain and significant reduction in the size of gastric mass and outlet obstruction on follow-up endoscopy.

Discussion: GPA is a systemic disease characterized by necrotizing granulomatous inflammation and vasculitis that primarily involve upper and lower respiratory tracts, as well as kidneys. GI involvement in GPA is uncommon and outlet obstruction mimicking gastric malignancy is extremely rare with only few cases reported in literature. Although the biopsy did not reveal presence of granuloma, our patient had raised PR3 titres with evidence of necrotizing granulomas in lung FNAC.

Conclusion: Although rare, GI involvement may be a presenting feature in GPA, with occasional occurrence as gastric tumor-like lesions. Diagnosis should be considered in patients presenting with GI symptoms accompanied by evidence of systemic vasculitis.


  PM0093: Kawasaki disease presenting with peripheral gangrene Top


Deonath Mahto, Anu Maheshwari, Rohit Duggar; Department of Pediatrics, Division of Rheumatology and Immunology, Lady Hardinge Medical College and Associated Kalawati Saran Children's Hospital, New Delhi, India

Kawasaki disease is the most common cause of acquired heart disease in childhood. Early diagnosis and treatment may significantly improve the prognosis. Diagnosing Kawasaki disease in younger children is difficult because of obscure and atypical symptoms, moreover they are at the higher risk of coronary artery abnormalities and complications.

We report a 14 month old baby with fever of 10 days duration and peripheral gangrene without any other clinical manifestations of Kawasaki disease. Kawasaki was diagnosed subsequently due to mild dilation(LMCA SD score +1. 89, LAD SD score +2. 28) of coronary artery and lack of tapering of the arteries on Echo cardiography. After 1 month of follow up, reduction of LMCA SD Score to +0. 77 was confirmatory as per 2017 AHA criteria. In addition, on follow up he developed cracking of lips, periungal and perianal desquamation. He also had history of red eyes early in the course of disease which was absent at the time of presentation. He was treated with high dose aspirin, IVIG and pulse therapy with methylprednisolone. He received aspirin and warfarin on follow up.

To conclude, Peripheral gangrene must be regarded as an important sign of Kawasaki disease in younger children and early treatment can prevent severe permanent coronary involvements and sequae.


  PM0094: An unusual presentation of medium vessel vasculitis Top


M Sabarinath, T N Tamilselvam, N Balakrishnan, H Raghavendra, Anusha Sri Matam, R Ramesh, S Mythili; Department of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

27 year male presented with fever, polyarthralgia and painful bullous skin lesions in lower limb for 2 weeks duration. On eliciting past historypatient had multiple ulcers in legsfollowing similar skin lesions andgangrene of finger tips at 8 yrs of age. He was treated as vasculitis with cyclophosphamide 6 doses and maintained on MMF. The current skin lesions appeared after he defaulted treatmentfor 4 months. He has no other comorbid illness including claudication pain or smoking. On examination patient had tenderbullous lesionsand induration involving both lower limb with multiple papery scars in legs. He also had amputation of his left 5th digit, resorption of right 2nd digit and residual hyperpigmentation over abdomen, limbs. Rest of his examination was normal and all his peripheral pulses were palpable with no bruit in neck, abdomen. On evaluation his complete blood counts were normal with elevated ESR and CRP. His renal, liver function were normal and viral markers were negative. His immunological workup was negative for RF, ANCA, ANA and cryoglobulins. His skin biopsy showed supra basal bulla with inflammatory infiltrate of predominantly mononuclear cells around dermal appendages and engorged vessels suggestive of vasculitis. His CT angiography showednear total occlusion of right superficial femoral, anterior tibialsuggestive of peripheral vascular disease with normal upper limb, abdominal vessels. His APS and prothrombotic workup was negative. He was treated with pulse steroids, MMF, aspirin, supportive measures. His bullous lesions subsided with peeling of skin and ulcers healed with residual hyperpigmentation. We present this as a case of childhood PAN with cutaneous lesions, gangrene in fingers with rare association of total occlusion of lower limb vessels.
Figure 1: Skin lesion showing tender nodules with bullous lesion, CT angio showing occulusion of right femoral artery

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  PM0095: Multifaceted and intriguing presentations of male scleroderma patients: A case series from clinicians perspective Top


N Balakrishnan, N Sabarinath, T N Tamilselvam, R Ramesh, S Mythili; Department of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Case 1: 54/M presented with breathelssness for one month. He was diagnsosed as pulmonary thromboembolismbased on echo and CTPA. He was treated with anticoagulation. During evaluation he was found to have sclerodactyly, Raynauds, salt and pepper pigmentation over scalp and thyromegaly. His ANA status revealed nucleolar pattern by IIF. There was no evidence of ILD and GIT manifestation. Hypercoagulable workup was negative. He was treated with low dose steroid and Azathioprine.
Figure 1: Salt and pepper pigmentation

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Figure 2: HRCT chest showing ILD

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Figure 3: Sclerodactyly

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Case 2: 47/M presented with recurrent episodes of AGE, chronic anemia requiring blood transfusion, loss of weight and appetite since 2010. In 2014 he developed inflammatory polyarthritis and frequent AGE episodes. Anemia was persisting. OGDscopy revealed distal esophagitis and pangastritis. He was treated conservatively. In 2016 he was admitted in our centre with limited cutaneous scleroderma and ILD based on clinical features and ANA positivity. The cause for anemia was found to be AIHA based on DCT positivity. ANA testing revealed speckled pattern by IIF. He was treated with steroids and immunosuppression and he is under follow up.

Case 3: 40/M presented with shortness of breath for two months duration in 2017. Echo revealed moderate pulmonary hypertension without RA, RV dilatation. Three months later he was diagnosed aslimited cutaneous scleroderma and ILD with NSIP pattern. He presented to our centre with inflammatory polyarthritis and GERD. He had arthralgia of large and small joints, sclerodactyly, salt and pepper pigmentation, raynauds with pitted scars on clinical examination. ANA status revealed homogenous pattern by IIF. He was incidentally found to be positive for hepatitis B antigen. He was being treated with calcium channel blockers and phosphodiestrase 5 inhibitors.

We present this case series of multifacted presentation of male scelroderma patientswith initial manifestationofPulmonary embolism, Anemia and inflammatory polyarthritis, Pulmonary hypertension.


  PM0096: Pancreatitis-panniculitis-polyarthritis syndrome with plexopathy Top


Rakesh Kumar Jagdish, Ankur Jindal; Department of Hepatology, ILBS, Delhi, India

Pancreatitis-Panniculitis-Polyarthritis (PPP) syndrome is rare extrapancreatic complication of pancreatic disease with unclear physiopathology. There are few case reports in literature of a syndrome consisting of pancreatitis, panniculitis and polyarthritis known as 'PPP syndrome'. We report a patient with 4th “P” plexopathy along with this “P 3” syndrome. There is only scanty information in literature regarding this type of presentation. A case of38 year old male patient without any prior co morbidities, presents with recurrent mild acute pancreatitis (4-5 episodes) over last 1. 5 years. During the first episode of pancreatitis, he developed symmetrical polyarthritis (swelling of bilateral wrist, bilateral knee and bilateral ankle joint), subsequently leading to flexion deformity at knee and ankle. His wrist and right ankle knee arthritis improved over time with treatment. There were subcutaneous nodules over left leg and both arms. He also noted left elbow weakness and decreased sensations in upper left arm, 2 months after the onset of first episode of acute pancreatitis. Examination at recent admission revealed normal vital parameters, hyperextended left elbow with poorly demarcated sensory loss in left arm, left knee and bilateral ankle joint effusion with active synovitis with decreased range of motion, however small joints and spine were normal. There were multiple 0. 5- to 1-cm sized nodular lesions in both forearms and left leg. Abdominal examination was grossly normal except moderate tenderness at epigastrium with no rebound tenderness. Biopsy of skin nodule showed panniculitis. Laboratory examination was negative for igG4, autoimmune disorders. Imaging confirmed the diagnosis of acute recurrent pancreatitis with polyarticular involvement. Systemic (methylprednisolone, 60 mg daily) and local (intrarticular) steroid therapy resulted in a modest improvement of pain but no improvement in joint deformity. For Plexopathy conservative treatment was given in consultation with neurologist with short course of steroids, pregabalin and amitriptyline but without significant improvement.


  PM0097: Rare manifestation of a rare disease. (Anti PL12 presenting as acute ILD with fulminant inflammatory myopathy) Top


Shaloo Bhasin, Prasan Deep Rath, Swetal Pandey, Rahul Bisaralli; Max Superspecialty Hospital Saket, New Delhi, India

Case Report: 69 years female with seropositive inflammatory poly arthritis presented withchief complains of diffuse myalgias for1 month and acute onsetdyspnea for 1 day. Initial investigations revealed leucocytosis, transaminitis with normal renal, procalcitonin levels. CPK levels were very high. (16340) with a normal urineexamination. Xray chest was s/o extensive b/l fluffy shadows s/o ARDS.

In view of severe respiratory failure patient wasintubated and ventilated and started on iv antibiotics. Bronchoscopy was done, however patient's condition deteriorated further withhigh Fio2inotropic support. During hospital stay patient developed acute ischemia of right upper limb and lower limb with gangrene. Anticoagulation was started for the same.

Further investigations revealed a rapidly rising cpk levels (upto 89 000). RF / Anticcp were high(119 /113). ANA by IF and LIA was negative. Myositis LIA revealed PL12 +++. APLA workup was negative. All cultures including BAL were negative. In view of persistent rising CPK and no clinical improvement a possible diagnosis of acute necrotizinginflammatory myopathy was consideredand patient was started on IVIG. Meanwhile a muscle biopsy was performed which was consistent with inflammatory myopathy. Patient was treated with solumedrol pulse 500mg for 3 days. Her clinical condition gradually improved with clearing of the xray infiltrates and settling of CPK levels.

Discussion: Antisynthetase syndrome (ASS)is characterized by inflammatory myositis associated with ILD. Anti JO-1 is the most common antisynthetase autoantibody, other antisynthtaseantibody namely anti-alanyl-t–RNA synthetase(anti PL 12) has been described. Unlike anti-jo 1 ASS, anti PL12 ASS has a constant lung involvement and gastrointestinal complicationswhereas muscular involvement is less frequent with less severity. Other symptoms includingraynauds and mechanic hands are also rare.

Conclusion: Anti-alanyl-t–RNA synthetase(anti PL 12) are particularly rare antisynthetase antibodies. And should always be considered in patients presenting with Acute ILDand fulminant myositis with no other physical features of typical ASS.


  PM0098: Hypokalemic paralysis secondary to Sjogren's interstitial nephritis: A missed opportunity Top


P Aparna; AIIMS, Bhopal, Madhya Pradesh, India

Introduction: Sjogren's syndrome is an autoimmune disease with glandular and extra glandular manifestations. Tubulointerstitial nephritis is the main renal involvement associated with primary Sjogren syndrome. It can manifest as distal renal tubular acidosis (RTA) which can cause hypokalemia and patient can present with recurrent, fluctuating but reversible episodes of myopathic weakness. RTA has been reported in 4. 3 to 9% of pSS patients; it is more common in middle-aged women, and two-thirds of them will develop symptoms. RTA with hypokalemic paralysis as a presenting feature of Primary Sjogren's syndrome is described in few case reports in literature. It can be the initial presentation in seven percent of Sjogren's patients. Here we describe 2 case reports in support of these.

Case Reports: A 19/F presented to the medicine department and a 34/M presented to neurology department with h/o of progressive weakness of bilateral upper and lower extremities. Neurological examination revealed severe flaccid weakness. Both patients had past medical history was positive for repeated hospital admissions following episodes of weakness and fatigue associated with hypokalemia that responded well to supplemental potassium alone.

Laboratory Results: demonstrated severe hypokalemia with hyperchloremic metabolic acidosis and raised urinary PH. USG abdomen was suggestive of bilateral nephrocalcinosis. EMG/ NCV was done and ruled out AIDP. Immunologic work-up showed a strongly positive ANA and positiveantibodies to SSA and SSB. Schirmer's test was abnormal in one patient, normal in other. Lip biopsy was done and the features were consistent with lymphoplasmacytic sialadenitis compatible with the clinical diagnosis of Sjogren's syndrome. Both patients were treated with steroids and potassium and bicarbonate supplementation, steroids were gradually tapered and stopped and syrup potchlor and bicarbonate were continued. On further follow up, there was gradual improvement in serum potassium with normalization of serum pH and bicarbonate levels and both patients became asymptomatic on these treatment line.

Discussion: The mechanism of hypokalemia in Sjogren's syndrome is because of distal RTA brought about by chronic interstitial nephritis which in turn leads to decrease tubular sodium delivery, defective H-K ATPase, secondary hyperaldosteronism, and bicarbonaturia.

If correctly diagnosed, it is easily treated with immunomodulators, with potassium supplementation and acidosis control with bicarbonates Madhya Pradesh

Conclusion: There is a usual delay in the diagnosis of sjogrens in such presentations as occurred with both patients in our study. This reinforces to keep sjogrens syndrome as DD in recurrent hypokalemicmuscle weakness, especially in the presence of nephrocalcinosis and distal RTA.




    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14], [Figure 15], [Figure 16], [Figure 17], [Figure 18], [Figure 19], [Figure 20], [Figure 21], [Figure 22], [Figure 23], [Figure 24], [Figure 25], [Figure 26], [Figure 27], [Figure 28], [Figure 29], [Figure 30], [Figure 31], [Figure 32], [Figure 33], [Figure 34], [Figure 35], [Figure 36], [Figure 37], [Figure 38], [Figure 39], [Figure 40], [Figure 41], [Figure 42], [Figure 43], [Figure 44], [Figure 45], [Figure 46], [Figure 47], [Figure 48], [Figure 49], [Figure 50], [Figure 51], [Figure 52], [Figure 53], [Figure 54], [Figure 55], [Figure 56], [Figure 57], [Figure 58], [Figure 59], [Figure 60], [Figure 61], [Figure 62], [Figure 63], [Figure 64], [Figure 65], [Figure 66], [Figure 67], [Figure 68], [Figure 69], [Figure 70], [Figure 71], [Figure 72], [Figure 73], [Figure 74], [Figure 75], [Figure 76], [Figure 77], [Figure 78], [Figure 79], [Figure 80], [Figure 81]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6], [Table 7], [Table 8], [Table 9], [Table 10], [Table 11], [Table 12], [Table 13], [Table 14], [Table 15], [Table 16], [Table 17], [Table 18], [Table 19], [Table 20], [Table 21], [Table 22], [Table 23], [Table 24], [Table 25], [Table 26], [Table 27], [Table 28], [Table 29], [Table 30], [Table 31], [Table 32], [Table 33], [Table 34], [Table 35], [Table 36], [Table 37], [Table 38], [Table 39], [Table 40], [Table 41], [Table 42], [Table 43], [Table 44], [Table 45], [Table 46], [Table 47], [Table 48], [Table 49]



 

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PC0090: Clinical...
PC0091: Efficacy...
PC0092: Comparis...
PC0093: Psychome...
PC0094: ASDAS is...
PC0095: Assessme...
PC0096: Non-adhe...
PC0097: Prescrib...
PC0098: Clinical...
PC0099: Could ap...
PC0100: Comparat...
PC0101: Indian p...
PC0102: Ankylosi...
PC0103: Prevalen...
PC0104: A random...
PC0105: Regenera...
PC0106: Role of ...
PC0107: Study of...
PC0108: Study of...
PC0109: An obser...
PC0110: Is the p...
PC0111: Monitori...
PC0112: A prospe...
PC0113: Predicti...
PC0114: Study of...
PC0115: Anti-Jo-...
PC0116: Rheumato...
PC0117: Widening...
PC0118: Quality ...
PC0120: A study ...
PC0121: Biologic...
PC0122: Biologic...
PC0123: The need...
PC0124: Performa...
PC0125: Changes ...
PC0126: Relation...
PC0127: Design o...
PC0128: Use of b...
PC0129: Study of...
PC0130: Comparat...
PC0131: Effects ...
PC0132: A networ...
PC0133: A study ...
PC0134: Demograp...
PC0135: Clinical...
PC0136: Use of p...
PC0137: Safety o...
PC138: Clinical ...
PC0139: Short te...
PC0140: Prematur...
PC0141: To evalu...
PC0142: Clinical...
PC0143: Spectrum...
PC0144: Co-exist...
PC0145: Early rh...
PC0146: A compar...
PC0147: Outcome ...
PC0148: Assessme...
PC0149: Clinical...
PC0150: Incidenc...
PC0151: Osteopor...
PC0152: A study ...
PC0153: Bone den...
PC0154: Factors ...
PC0155: Factors ...
PC0159: Clinical...
PC0160: Validati...
PC0161: Using cl...
PC0162: Efficacy...
PC0163: An etiol...
PC0164: Prospect...
PC0165: Experien...
PC0166: Profile ...
PC0167: Predicto...
PC0168: Case ser...
PC0169: Efficacy...
PC0170: Clinical...
PM0001: HIV pres...
PM0002: Adult on...
PM0003: Rituxima...
PM0004: Complete...
PM0005: Case of ...
PM0006: Arthriti...
PM0007: Idiopath...
PM0008: A tetrad...
PM0009: Not so u...
PM00010: Adult o...
PM0011: Antineut...
PM0012: Case of ...
PM0013: H syndro...
PM0014: An unusu...
PM0015: An elder...
PM0016: A patien...
PM0017: Monoarth...
PM0018: Nocardia...
PM0019: A rare c...
PM0020: Multifoc...
PM0021: An unusu...
PM0022: An uncom...
PM0023: Pseudo-o...
PM0024: Atypical...
PM0025: Idiopath...
PM0026: p-ANCA p...
PM0027: PUO and ...
PM0028: Wegeners...
PM0029: Familial...
PM0030: Autoimmu...
PM0031: Ankylosi...