Abstract Introduction Enthesitis related arthritis (ERA) category of juvenile arthritis is a form of juvenile spondylarthritis. In many immune-inflammatory disorders a subclinical phase precedes a clinical disease. First degree relatives either due to genetic predisposition or sharing of environment have a high recurrence risk for these diseases. No data is available in siblings of ERA patients. Methods 38 sibling-proband pairs were evaluated after consent. Clinical examination, X-ray LS-spine, MRI of SI-joints and MSUS for enthesitis were done to detect subclinical disease in siblings. Frequency of lymphocyte and monocyte subsets, IL-17 producing NK cells, NK-T cells, γδ T cells and CD4 lymphocytes, IL-6 and TNF-α producing monocytes was measured using FACS. Faecal calprotectin levels were measured using ELISA. Results 38 siblings of 36 proband (13.8±3.5 years) were included. 20 of 38 siblings were HLA-B27 positive. Among siblings, none had arthritis, 1 each had inflammatory back pain and uveitis. On radiological assessment, 5 showed evidence of sacroiliitis (Grade II-4, Grade IV-1) on X-ray while 5 siblings had bone marrow edema around SI joints (one quadrant-4, four quadrants-1) on MRI. On MSUS enthesitis was seen in 2 siblings (patellar tendon-1, Tendoachillies-1). All but one with abnormalities were HLA-B27 positive. In comparison to siblings, the probands had lower frequency of classical monocytes (p < 0.05), IL-6 producing monocytes (p < 0.01) and higher frequency of Th17 cells (p < 0.05). The faecal calprotectin levels were higher in probands (p < 0.01). Analysing only the HLA-B27 siblings since subclinical disease was seen in only HLA-B27 siblings, the frequency of IL-17 producing CD4 T cells and γδ T cells (p < 0.01) and faecal calprotectin levels (p < 0.01) were higher in probands when compared with B27 positive siblings. Conclusion Subclinical disease is seen in subset of HLA-B27 siblings of patients with ERA. Gut inflammation and a pro-inflammatory state probably results in clinical disease in probands. Funding Source n/a Topic Categories Immune Mechanisms of Human Disease (HUM)

Juvenile Idiopathic Arthritis (JIA) causes caregiver burden on families with children affected with it. Our study aimed to explore this multifaceted burden in the Indian context. In this cross-sectional study, we administered the Hindi translated CAREGIVER questionnaire to adult caregivers in the families of JIA patients ≤ 18 years. The responses to the 28 items were used to calculate the burden scores in various dimensions. The relationship of the global burden scores with demographic and socioeconomic factors were analysed. Non parametric tests were used. Two hundred twenty-one caregivers participated with a median age of 39 years (IQR 32–45). This included 116 fathers, 50 mothers, 32 brothers, 18 uncles, three grandfathers, one sister, and one grandmother. The JIA patients had a median age of 15 (12–17) years, and the male-to-female ratio was 3.2:1. Enthesitis-related arthritis was the predominant subtype (72.4

Renal disease in primary Sjogren’s Syndrome(pSS) occurs as tubulointerstitial nephritis(TIN) or glomerulonephritis(GN). Data from India on pSS are sparse and even less on nephritis. We studied the prevalence and impact of renal disease on patient outcomes. We reviewed 179 (F:M 12.7:1, age 41.7 ± 12.9 years) patients of pSS from records at a single centre from 2000 to 2020. Data on nephritis, clinical and laboratory variables were collected from baseline visit. Outcomes studied were chronic kidney disease(CKD) and death. We identified predictors of nephritis and rising creatinine on follow-up. Fifty-four (30.17%) patients had nephritis. Their mean age was 40.19 ± 13.28 years with 157.3 person-years follow-up. Vasculitis (OR 2.33, 1.02–5.3), fatigue (OR 3.29, 1.63–6.65), ANA positivity (OR 7.79, 1–60.62), anti-Ro52 (OR 2.74, 1.18–6.39), anti-La (OR 2.13, 1.1–4.14), both Ro and La (OR 2.4, 1.23–4.69) and lymphopenia (OR 2.27, 1.16–4.41) predicted nephritis on univariate analysis. On multivariate analysis, only fatigue (OR 2.83, 1.22–6.57) and an interaction between polyarthritis and vasculitis (OR 9.17, 1.15–72.96) was associated with nephritis. Creatinine at one (1.6 ± 1.17 mg/dL vs. 0.8 ± 0.2 mg/dL) and 2 years (1.62 ± 1.19 mg/dL vs. 0.8 ± 0.2 mg/dL) follow-up was higher in the nephritis group. Baseline haematuria, leukocyturia, 24 h urinary protein and thrombocytopenia were independent predictors of rising creatinine. Six patients died and 10 developed CKD. Event-free (death or CKD) survival was 89.1% at 5 years. Patients with nephritis had worse event-free survival. Our cohort had a younger age of onset of Sjogren’s syndrome and a higher prevalence of nephritis than previously reported. Fatigue, polyarthritis and vasculitis at baseline predicted the development of nephritis. Nephritis was associated with a higher probability of death or CKD.

Introduction: Rheumatic diseases are associated with poor obstetric outcomes, especially in developing countries. In a multisystem disease like antineutrophil cytoplasmic antibody-associated vasculitis (AAV), pulmonary and renal involvement may contribute to adverse pregnancy outcomes. we explored pregnancy outcomes in women with AAV and compared pregnancies after disease onset to those that occurred before it. Method: Women with AAV (Chapel Hill Criteria, 2012) registered at a tertiary care center (2001–2021) were interviewed by teleconsultation or during outpatient visits. Maternal complications and fetal complications were recorded. Results: Median age at disease onset was 48 (33–60) years, with the most common subtype being granulomatosis with polyangiitis (13, 48%) followed by microscopic polyangiitis (10, 37%). Twelve women were in the reproductive age group, of which six suffered from a premature menopause. Three pregnancies in three women after disease onset were compared with 62 pregnancies in 23 women with conception before the disease. Pregnancies before disease onset resulted in 58 (93.3%) live birth. One (33.33%) live birth was observed in the pregnancies after disease onset, and disease onset during pregnancy resulting in intrauterine death at 20 weeks period of gestation. One patient is currently in her antenatal period with no complications so far. This study suggests the absence of impact on pregnancy outcome before diagnosis of AAV. Conclusion: Pregnancy after a diagnosis of AAV is rare, and successful outcomes may be reported on occasion.

Relapse in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is associated with significant morbidity and mortality. Utility of ANCA for prediction of relapses is still controversial. PubMed/MEDLINE, Scopus, and WebOfScience were searched, screened and confirmed for inclusion [PROSPERO No: CRD42020220308]. Studies measuring serial ANCA by ELISA or indirect immunofluorescence (IF), reporting relapses with sufficient data to calculate sensitivity and specificity were included. Diagnostic odds ratio (OR), sensitivity, specificity and likelihood ratios (LR) were synthesized using a bivariate mixed-effect regression model. Sub-group analysis included a comparison between ELISA and IIF, anti-myeloperoxidase (MPO) and -proteinase 3(PR3), and type of rise in ANCA. For meta-analysis of survival outcomes, hazard ratios were synthesized using a random-effect model. QUADAS-2 was used for assessing quality of studies, I2 statistic for heterogeneity Begg’s test for publication bias. 2946 abstracts and 43 full-texts were reviewed to identify 26 eligible studies that included 2623 patients with AAV and 848 relapses. Overall heterogeneity was high [I2 = 99

Macrophage migration inhibitory factor (MIF) is a pleiotropic cytokine and has been implicated in pathogenesis of ankylosing spondylitis (AS). CD 74 is the receptor for MIF and IgA antiCD74 autoantibodies have been described from different parts of the world in patients with AS. As enthesitis-related arthritis (ERA) is a form of juvenile spondyloarthropathy, we studied the serum and synovial fluid levels of MIF in ERA and looked for the IgA antiCD74 antibodies in patients with ERA in our population. Patients with JIA (ILAR classification) were studied. Serum MIF levels were measured by ELISA in 101 patients of ERA (synovial fluid also where available) and compared to 28 patients of other categories of JIA, 25 patients each of ankylosing spondylitis and rheumatoid arthritis, and 38 healthy controls. In addition, association of MIF with disease activity was assessed. Ig A antiCD74 antibodies were measured in sera of ERA, AS and healthy controls. Median serum MIF levels were higher in ERA [2.50 (1.20–4.85) ng/ml] than in healthy controls [0.28 (0.16–0.48); p < 0.0001] and patients with RA [1.13 (0.44–2.45); p < 0.01] MIF levels in ERA were comparable to other categories of JIA [2.63 (1.70–4.05)] and patients with AS [3.62 (0.52–6.51)]. Synovial fluid MIF levels were higher than serum levels (p < 0.01). Serum MIF level had an association with the JSpADA score (r = 0.29, p < 0.01). Serum MIF levels had no association with presence of inflammatory markers, enthesitis, inflammatory back pain or sacroiliitis. IgA AntiCD74 antibody was positive only in 3/88 (3.41

Inclusion body myositis is a rare sporadic inflammatory-degenerative myopathy of the elderly. Despite being the commonest type of acquired myopathy after the age of 50, misdiagnosis is extremely common. The most frequent hurdle in identifying new cases is the wrong diagnosis of polymyositis or motor neuron disease. Novel insights into pathogenic mechanisms have heralded the quest for newer therapeutics as well as drug repurposing in this otherwise progressive disorder.

Most rheumatic diseases (RDs) have a predilection for women in the reproductive age group. Common drugs used in rheumatology practice have identified risks to the fetus; thus, adequate pregnancy counseling is of utmost importance. Contraception and adequate preparation for the same can enhance the experience of motherhood and decrease intra as well as peripartum complications. The knowledge, as well as practices of contraception in autoimmune diseases, are low and varied in various populations. The challenges faced in different RDs are unique and keen understanding can be fruitful toward better patient care. A multi-disciplinary team effort between the patient, obstetricians, and the rheumatologist is the key to better maternal and fetal outcomes.