Caregiver burden in families of children with juvenile idiopathic arthritis in India.

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%). Most caregivers (70.6%) expressed sadness at diagnosis, and 29.9% continued to express sadness. Nearly two-thirds (65.6%) had to borrow money from others. More than half (59.3%) of the caregivers neglected their health, and 9.0% became sick. Male gender of the child, systemic JIA subtype, low socioeconomic status, high disease activity, extra-articular damage, high parent-reported disease activity and poor quality of life were associated with higher global caregiver burden. JIA has a significant emotional, social, economic, and labour impact on caregivers. Economic and psychosocial support needs to be given to family caregivers caring for children with JIA.

Long-standing and poorly controlled disease in juvenile dermatomyositis is associated with calcinosis: a real-world experience from a low-middle income country.

To study the prevalence and predictors of calcinosis in Juvenile Dermatomyositis (JDM). Medical records over 20 years at a tertiary care rheumatology center in Northern India were reviewed to identify patients with JDM and clinical details were recorded. The frequency of calcinosis, predictors, specific treatment, and its outcomes were studied. Data are expressed as median and interquartile range. In eighty-six patients (median age 10) of JDM, the frequency of calcinosis was 18.2% (8.5% at presentation). Younger age at presentation, longer follow-up, heliotrope rash [Odds Ratio (95% confidence interval), 11.4 (1.4-92.12)], chronic or polycyclic course [4.4 (1.2-15.5)] and cyclophosphamide use [8.2 (1.6-41.9)] were associated with calcinosis. Dysphagia [0.14 (0.02-1.2)] and elevated muscle enzymes [0.14 (0.04-0.5)] were negatively associated with calcinosis. Treatment with pamidronate had a good to moderate response to calcinosis in five of seven children. Calcinosis in JDM is associated with long-standing, poorly controlled disease, and the use of bisphosphonates like pamidronate offer promise in the future for its treatment.

Tuberculosis remains a leading contributor to morbidity due to serious infections in Indian patients of SLE.

INTRODUCTION: Infections are a major cause of morbidity and mortality in systemic lupus erythematosus (SLE). We assessed the incidence and risk factors for major infections in SLE in India. METHODS: A retrospective review of a cohort of 1354 patients of adult SLE (ACR 1997 criteria) seen between 2000 and 2021 at a single center was conducted. Serious infections (need for hospitalisation, prolonged intravenous antibiotics, disability, or death) were recorded. Cox regression was used to determine factors associated with serious infection and the effects of serious infection on survival and damage. RESULTS: Among the 1354 patients (1258 females, mean age of 30.3 years, follow-up of 7127.89 person-years), there were 439 serious infections in 339 patients (61.6 per 1000 person-years follow-up). Bacterial infections (N = 226) were the most common infection followed by mycobacterial infections (n = 81), viral (n = 35), and then invasive fungal infections (N = 13). Mycobacterium tuberculosis was the single most common microbiologically confirmed organism with incidence of 1136.4/100,000 person-years with 72.8% of them being extrapulmonary. Infection free survival at 1 year and 5 years was 82.9% and 73.8%. There were 119 deaths with infection attributable mortality in 65 (54.6%). On multivariable Cox regression analysis, higher baseline activity (HR 1.02, 1.01-1.05), gastrointestinal involvement (HR 2.75, 1.65-4.69), current steroid dose (HR 1.65, 1.55-1.76), and average cumulative steroid dose per year (HR 1.007, 1.005-1.009) were associated with serious infection and higher albumin (HR 0.65, 0.56-0.76) was protective. Serious infections led to greater damage accrual (median SLICC damage index of 1 vs. 0) and mortality (HR was 18.2, 32.7 and 81.6 for the first, second, and third infections). CONCLUSION: Serious infections remain a major cause of mortality and damage accrual in SLE and higher disease activity, gastrointestinal involvement, hypoalbuminemia, current steroid dose, and cumulative steroid dose are the risk factors for it.

Development and validation of an ama instrument for assessing the disease activity on the basis of constitutional features in Amavata (Rheumatoid Arthritis).

BACKGROUND: Rheumatoid Arthritis (RA), having a striking clinical resemblance to amavata in traditional Indian medicine (Ayurveda) presents an opportunity to look at disease from two different healthcare perspectives. This differential information may potentially supplement one system with the knowledge of the other for optimal application. This study is the first of its kind, where Ayurvedic concepts of amavata have been adopted to enhance the knowledge about RA where optimal care is still beyond the common reach. OBJECTIVE: The study was conducted to develop and validate a novel ama score based upon constitutional features of ama as depicted in ayurvedic literature as a disease activity indicator in RA. MATERIAL AND METHODS: The study was conducted in two parts comprising development and textual validation of the ama assessment instrument (AAI) followed by its clinical testing. AAI comprising ten items, was developed where each item was provided with a range of scores to offer the assessment close to the patient's observations. The score obtained through AAI was clinically and statistically tested on 79 RA/amavata patients randomly selected for validity and reliability. The score obtained through AAI was tested for its correlation with the DAS-28 score and ESR. RESULTS: Ama Assessment Instrument could find a slight correlation with acute phase reactant ESR (r-value between ESR and AMA at baseline is 0.287, and at 1st, 2nd, and 3rd follow-up is 0.276, 0.276 and 0.160 respectively) and DAS-28 (The r value between DAS and AMA at baseline is 0.231, and at 1st, 2nd and 3rd follow up is 0.218, 0.201 and 0.247 respectively). It however emerged as an independent disease status marker since it could mark the changes in the study population on a time scale more precisely as compared to DAS -28 or ESR. When the ama values at different follow-ups were compared, a significant difference was observed consistent with disease activity marker catching constitutional and GI related domain of the patients. When reducing values of ama score were compared to overall improvements as reported by the patients, a similar trend was observed showing that a change in ama score is reflective of a change in disease status and the impact of the disease on the patient. CONCLUSION: This study provided a quantitative measure for the abstract concept of ama which could be used to mark the disease activity in amavata or RA. The change in ama based scores can be used to assess disease status and the intervention related benefits. The observations prompt for the possible inclusion of AAI in RA composite score to make it more dynamic in terms of disease activity identification in RA.

Renal involvement in Sjogren's syndrome: predictors and impact on patient outcomes.

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.

Macrophage migration inhibitory factor (MIF) and IgA anti CD74 antibodies in Indian patients with enthesitis-related arthritis category of Juvenile idiopathic arthritis.

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%) of ERA patients and was not detected in any patients of AS or healthy controls. Patients with ERA have high MIF levels that show modest correlation with disease activity. Higher synovial fluid MIF levels suggest that it may play a role in synovitis seen in ERA. IgA antiCD74 antibodies are rarely seen in ERA.

Differentiating flare and infection in febrile lupus patients: Derivation and validation of a calculator for resource constrained settings.

Background: Patients with Systemic Lupus Erythematous (SLE) are at an increased risk of infection and it is often difficult to differentiate between infection and disease activity in a febrile patient with SLE. Methods: Patients with SLE (SLICC criteria) presenting with fever between December 2018 and August 2021 were included. Neutrophil to lymphocyte ratio (NLR), NEUT-x, -y, -z indices, Erythrocyte sedimentation rate (ESR), C-reactive protein(CRP), C3, C4, anti-dsDNA antibodies, and procalcitonin(PCT) were tested in addition to investigations as per the treating physician's discretion. Based on the clinical assessment and laboratory data, the febrile episode was classified into infection, disease flare, or both. Statistical analysis was done using GraphPad prism v8.4.2. A novel composite score was devised and validated with a calculator incorporated is a spreadsheet. The performance of a previously proposed model of duration of fever, CRP, and dsDNA (Beca et al) was evaluated and other models using PCT and NEUT-Z were explored. Results: Among 168 febrile episodes in 166 patients with SLE (25 (19-32) years), 46 were due to infection, 77 due to flare, 43 due to both, and two due to other causes. High SLEDAI 2K (0.001), anti-dsDNA (p = 0.004), and low complements(C3, p = 0.001 and C4, p = 0.001) were characteristic of disease flare, whereas high total leukocyte count (TLC) (p = 0.008), NLR (p = 0.008), NEUT-x (p = 0.001), -y (p = 0.03), -z (p = 0.002), CRP (p = 0.001), and PCT (p = 0.03) were observed with infection. A model using age, TLC, and CRP was devised using 80% of the cohort with an AUC of 0.88 (0.78-0.97) which was validated in the remaining 20% to have an AUC of 0.83(0.60-1.0). The model devised by Beca et al yielded an AUC of 0.74. Use of PCT did not improve the discrimination between flare and infection. A Model of C4 and NEUT-z analyzed in a subset performed well and needs further exploration. Conclusion: A composite score of low cost and routinely available parameters like age, TLC, and CRP gives a good discrimination between infection and flare in a febrile patient with SLE.

Catatonia in systemic lupus erythematosus: case based review.

Catatonia is a rare psychomotor syndrome characterized by stupor, posturing and echophenomena. It can be associated with schizophrenia, infections, drugs and autoimmune causes like anti N-methyl D-aspartate (NMDA) receptor encephalitis and systemic lupus erythematosus (SLE). Here we report two cases of systemic lupus erythematosus with catatonia, who improved with immunosuppressive treatment and review the cases described in the literature. The first case presented with fever, pancytopenia, toxic epidermal necrolysis (TEN)-like rash and later developed catatonia and macrophage activation syndrome (MAS). The second case presented with acute cutaneous lupus erythematosus (ACLE), fever, alopecia, polyarthralgias, nephritis, cytopenias along with catatonia. Successful management of this syndrome requires prompt recognition and treatment with immunosuppression as well as benzodiazepines with or without electroconvulsive therapy (ECT).

Spectrum of Myelitis in Systemic Lupus Erythematosus: Experience from a Single Tertiary Care Centre over 25 Years.

BACKGROUND: Inflammatory myelitis rarely occurs in Systemic Lupus Erythematosus (SLE). METHODS: Medical records from a tertiary care centre in India (1989-2018) were reviewed to identify patients with myelitis in SLE and their clinical characteristics and outcomes were compared with two matching comparators drawn from adjacent hospital registration numbers in the SLE database. RESULTS: Ten patients had myelitis from a cohort of 1768 patients with SLE. Myelitis was the first manifestation of lupus in 7 (70%). Cervicothoracic cord was most frequent site of involvement. ANA was negative at onset in 2 cases. One of 4 was positive for Anti-Aquaporin 4 antibody. Four had relapsing disease (16 events) with a median time to relapse of 0.65 years (0.3- 7 years). All cases received steroid sparing agents over the follow-up duration (78.5 patient years). Lupus nephritis (20% vs. 75%, p=0.004) and haematologic manifestations (0 vs. 25%, p=0.02) were less common. Higher frequency of anti-Ro antibodies was noted in the group with myelitis (p=0.05). CONCLUSION: Myelitis can be a presenting feature of SLE with lupus nephritis and hematologic involvement being rare. Relapses are common that mandate long-term immunosuppression.

High Prevalence of Active Tuberculosis in Adults and Children with Idiopathic Inflammatory Myositis as Compared with Systemic Lupus Erythematosus in a Tuberculosis Endemic Country: Retrospective Data Review from a Tertiary Care Centre in India.

AIM: Infections are the leading cause of morbidity and mortality in idiopathic inflammatory myositis (IIM) with India being endemic for Tuberculosis (TB). We compared and contrasted the prevalence, clinical profile and outcomes of active TB in IIM with systemic lupus erythematosus (SLE). METHODS: Medical records were reviewed for adults and children with IIM (Bohan and Peter criteria) and SLE (ACR criteria) at a tertiary care hospital in India from January 2015 to October 2017. Follow-up was recorded until February 2020 for all those who had developed active TB. RESULTS: Of 167 (132 adults and 35 juvenile) IIM and 280 (131 adults and 149 juvenile) SLE, active TB occurred in 24 (14.4%) IIM (22 (16.7% of 132) adults; 2 (5.71% of 35) juvenile) and 18 (6.4%) SLE [(8 (6.1% of 131) adults; 10 (6.7% of 149) juvenile, p-value < 0.01]. Patients with IIM had higher odds of developing TB as compared with SLE [OR 2.24 (CI 1.5-5.5), p=0.007]. The risk of developing active TB was 68-fold and 30.4-fold higher in patients with IIM and SLE, respectively, as compared with the general population. Extrapulmonary forms were more common (14/24). Nearly half developed TB during active IIM, at a glucocorticoid dose of 0.25 (0-1.5) mg/kg/day. Over a follow-up duration of 27 months (8-184), all were cured of TB, though prolonged course of anti-tuberculous treatment was required in 25%, and five IIM relapsed during treatment. CONCLUSION: Patients with IIM have increased risk of active TB, with common extrapulmonary forms, slow response, and relapses during treatment.

Clinical spectrum of active tuberculosis in patients with systemic lupus erythematosus.

INTRODUCTION: There is paucity of data on tuberculosis in Indian patients with systemic lupus erythematosus (SLE). We retrospectively studied clinical features and outcome of tuberculosis in SLE. METHODS: Medical records of patients who developed tuberculosis simultaneous or after the diagnosis of SLE were retrospectively reviewed. All patients fulfilled 1997 ACR and/or SLICC 2012 classification criteria for SLE. A diagnosis of tuberculosis required bacteriological, histopathological or CT/MRI suggestive of tuberculosis and initiation of four drug antituberculous therapy. Baseline parameters were compared with the rest of cohort to identify predictors of tuberculosis. RESULTS: In our cohort of 1335 SLE patients, 48 (3.6%) developed tuberculosis. Incidence of tuberculosis was calculated to be 733 per 100,000 patient years and occurred after a mean disease duration of 3.0 ± 4.1 years. Extrapulmonary tuberculosis (n = 37) was commoner than pulmonary tuberculosis (n =11). Most common radiological pattern in pulmonary tuberculosis was miliary and musculoskeletal TB was most common extrapulmonary TB. A microbiological diagnosis was obtained in 52.1% patients. Male gender was associated with higher risk of tuberculosis [OR 3.30 (1.55-7.05)]. Mortality was 14.5% and all patients who died had either disseminated (n = 5) or central nervous system (CNS) tuberculosis (n = 2). CONCLUSION: Incidence of tuberculosis in SLE is higher than general population and is associated with different phenotype and higher mortality. Male gender was associated with increased risk of tuberculosis in SLE.

Macrophage activation syndrome in systemic lupus erythematosus and systemic-onset juvenile idiopathic arthritis: a retrospective study of similarities and dissimilarities.

Macrophage activation syndrome (MAS) is a serious complication of rheumatic diseases. Fever and hyperferritinemia are common in active systemic-onset juvenile idiopathic arthritis (sJIA) and cytopenia in active systemic lupus erythematosus (SLE), thus recognizing MAS in them is a challenge. We compared clinical and laboratory parameters, various classification criteria, and outcomes of MAS in SLE and sJIA. Clinical and laboratory data were extracted from case records of patients with clinician diagnosed cases of SLE-MAS (adult and pediatric) and sJIA-MAS, admitted (2004-2018) at a tertiary care hospital. Ravelli, International consensus, HLH-2004, and criteria proposed by Parodi et al. were applied and compared. Among 33 patients (18 females) with MAS, 19 had SLE (7, childhood-onset SLE) and 14 had sJIA. MAS was more likely to be the presenting manifestation of disease in SLE (p < 0.05). There were no differences in the clinical features among them. Patients with SLE-MAS had lower baseline total leucocyte and platelet counts (p < 0.01), whereas patients with sJIA-MAS had significantly higher median CRP (p = 0.002), fall in TLC (p = 0.012), delta ESR/CRP ratio (p = 0.02), and lower fibrinogen level (p = 0.006). Neutrophil-to-lymphocyte ratio, ferritin/CRP ratio, and the number of patients with ferritin/ESR > 80 were similar. Only 6/33(18%) fulfilled the HLH criteria. Criteria meant for sJIA-MAS or SLE-MAS performed well for both diseases and the majority of patients could be diagnosed using them. Two patients died in each group. MAS in SLE and sJIA is more similar than dissimilar in clinical features and outcome. Criteria meant for MAS in sJIA or SLE-MAS performed equally well in both diseases.

Infections Are Leading Cause of In-Hospital Mortality in Indian Patients With Inflammatory Myopathy.

OBJECTIVES: We explored causes of in-hospital mortality in patients with inflammatory myositis at a tertiary care center in Northern India. METHODS: Records of adults and children diagnosed with dermatomyositis (DM), polymyositis, or anti-synthetase syndrome (ASSD) who died between 2000 and 2018 were reviewed, and causes of death were determined. RESULTS: Of the 38 patients, 20 had DM (including 2 clinically amyopathic DM), 4 juvenile DM, 12 polymyositis, whereas 2 had ASSD. Median age at death was 42.0 (interquartile range, 32.8-52.5) years. Median disease duration at death was 18.5 (interquartile range, 2.0-23.5) months. Twenty-four (63.2%) had infection as the primary cause of death. Other causes of death included pharyngeal muscle weakness and aspiration (n = 6), myocarditis (n = 2), respiratory failure (n = 2), cerebral bleed (n = 2), and pulmonary embolism (n = 1). One patient succumbed to rapidly progressive interstitial lung disease, whereas another patient with ASSD died following respiratory distress after rituximab infusion. In post hoc analysis, although thrombocytopenia appeared to be a risk factor for early mortality (odds ratio, 13.3; 95% confidence interval, 1.4-123.8; p = 0.01), this was not supported in the multivariate analysis. CONCLUSIONS: Infections are the most common cause of in-hospital mortality in myositis patients.

Prevalent vertebral fractures incur high risk of future fractures in inflammatory myositis.

OBJECTIVE: To assess accrual of new vertebral fractures (VF) in patients with idiopathic inflammatory myositis (IIM) over a period of time. METHODS: Hundred patients who were previously enrolled for a cross-sectional study on prevalence of asymptomatic VF were telephonically requested to review with repeat spinal radiographs and dual-energy X-ray absorptiometry (DEXA) after 3 years. Radiographs were scored using Genant's semi-quantitative technique. Disease activity and damage were assessed by myositis damage index (MDI) extent of damage and modified MDI for which the osteoporotic fracture item in MDI was removed. VF progressors were compared with non-progressors. RESULTS: Of 31 patients reviewed, 11 had dermatomyositis, 8 polymyositis, and 6 each overlap and anti-synthetase syndrome. Eighteen patients underwent DEXA scan. Seventeen had VF at baseline. At 91.62 patient years of follow-up, total number of VF increased from 27 to 51. Patients who had previous VF had higher risk of developing a new VF when compared with those with no VF (76.5% vs. 14.28%, RR: 5.35). Patients with old VF accrue fractures at a rate of 26.2 per 100 patient years. The number of fractures correlated significantly with age, T scores at the L4 level, and lower third of radius on DEXA, MDI, and modified MDI. Neither conventional nor disease-related variables differed between progressors and non-progressors. CONCLUSION: Patients with IIM with a prior VF incurred a five times risk of subsequent VF irrespective of disease activity and glucocorticoids. Key Points • Patients with inflammatory myositis are at a high risk of asymptomatic vertebral fractures. • Patients with baseline vertebral fractures incur a high risk of future fractures on follow-up. • Number of fractures is negatively correlated with age, BMD values at lower end of radius, L4, and damage.

Juvenile Reactive Arthritis and other Spondyloarthritides of Childhood: A 28-year Experience from India.

OBJECTIVES: Understanding of Juvenile reactive arthritis (jReA) and other spondyloarthritides of childhood (jSpA) is limited to small case series. Since most of them have speculated pathogenic origins in the gut, we compared and contrasted jReA with other jSpA -Enthesitis-related arthritis (ERA) and undifferentiated SpA (jUSpA). METHODS: A record-based medical data review of jReA, and jUSpA was compared with cohort data of ERA collected for other studies. Data are presented as median (interquartile range) and non-parametric tests used for analysis. RESULTS: Of 179 juvenile SpA (61 jReA; 101 ERA; and 17 jUSpA), 61 had jReA [M:F-52:9; 15.5 (12-18) years] with a disease duration of 2.75(1-36) months. Inflammatory backache IBP (32%), dactylitis (21%) and enthesitis (29%) were common. A significant proportion (14 of 17, 82.3% at >6 months follow-up) had a chronic course. 101 ERA [M:F-93:7; age-16(14-20) years] had a longer disease duration (45 vs 2.75 months, p<0.001), as compared with jReA. Enthesitis and IBP was more common in ERA (OR-2.3 and 3.4 respectively). jUSpA (n=17) had a similar clinico-laboratory profile and exhibited significant (7 of 17, 58.3%) chronicity over 9.5(4.8-37) months follow-up. CONCLUSION: jReA and jSpA exhibit similar features apart from varying disease duration, suggesting that jspA may form a continuum with similar clinico-laboratory profiles plausibly due to shared pathogenesis.

Systemic flare and cutaneous ulceration following cytomegalovirus infection in a patient with anti-melanoma differentiation-associated protein 5 (MDA5) associated myositis: Diagnostic challenge during the time of coronavirus disease (COVID-19) pandemic.

Background: Anti-melanoma differentiation-associated protein 5 (MDA5) associated idiopathic inflammatory myopathy (IIM) often manifests with minimal muscle weakness and rapidly progressive interstitial lung disease (RP-ILD) with a poor prognosis. The clinical presentation may be varied in different ethnic groups. The ongoing coronavirus disease (COVID-19) pandemic has made management even more challenging as certain manifestations may be difficult to diagnose remotely. Aim of the work: To throw light on the rare association of CMV infection in established anti-MDA5 myositis with severe consequences. Similar cases were presented and compared. Case report: A 42-year-old lady presented with heliotrope rash, periorbital edema, ulcerated Gottron's papules, proximal muscle weakness and intermittent fever of six-month duration. Anti-MDA5 antibodies were positive. Active disease, including myocarditis and RP-ILD, were challenging to diagnose on teleconsultation. Upon initiating tofacitinib, cytomegalovirus (CMV) polymerized chain reaction (PCR) came positive. Ganciclovir was started with the possibility of viral activation being the potential driving force for interferon pathway activation and dermatomyositis (DM) flare, but the patient succumbed to the illness. Conclusion: Viral triggers are known to induce autoimmune disease in the genetically predisposed. However, CMV infection in established anti-MDA5 myositis is uncommon and further association with myocarditis is a rare occurrence. Ulcerated Gottron's and periorbital oedema may carry a sinister connotation in Indians with anti-MDA5 DM, with worse manifestations such as myocarditis- which albeit rare, can be fatal.

In-hospital mortality and its predictors in a cohort of SLE from Northern India.

BACKGROUND: Mortality in SLE has a bimodal peak with early deaths mainly related to disease activity and infection. Although mortality has reduced over years, it is still two to three folds compared to the general population. In India due to increased burden of infection and limited access to health care, the causes may be different. METHODS: Retrospective, review of records of all adult SLE patients fulfilling ACR 1997 criteria, who died in hospital between 2000-2019 at a teaching hospital in India was done. In addition, baseline clinical features were extracted for all adult SLE patients seen during this period.Infections were either bacteriologically proven or based on clinicradiological or serologic evidence. Active disease was defined as SLEDAI 2k ≥ 5. Logistic regression was performed to ascertain risk factors for mortality. RESULTS: A total of 1337 (92% females) patient records were reviewed .The mean age at presentation was 29.9 ± 9 years.60-75% of patients had fever, mucocutaneous disease and arthritis, while nephritis, hematologic, serositis and neurologic involvement was seen in 48.6%, 43.2%, 16% and 10.3% respectively as presenting mainfestations. There were 80 in hospital deaths .Infection was the most common cause of death, with 37 due to infection alone and in 24 disease activity also contributed. Only 18 deaths were due to active disease. Among bacterial infections lung was the most common site and gram negative organism were the most common pathogens. There were 10 deaths due to Tuberculosis(TB) and half of them had disseminated disease. Patients with disease activity had a SLEDAI of 14.8 ± 6.4, with neurological, renal and cardiovascular involvement being the major contributors to mortality in 11, 7 and 6 cases respectively. Higher age at onset, male gender, fever, myositis, neurological, cardiovascular, gastrointestinal involvement, vasculitis, elevated serum creatinine at baseline were independent predictors of death. CONCLUSION: Infections are the most common cause of in-hospital mortality in SLE and TB still accounts for 15% of deaths related to infection. Vasculitis, myositis, cardiovascular and gastrointestinal involvement emerged as novel predictors of mortality in our cohort.

Poor obstetric outcomes in Indian women with Takayasu arteritis.

INTRODUCTION: Takayasu's arteritis (TA) affects young women in the childbearing age group. We studied obstetric outcomes in these patients before and after disease onset. METHODS: Women aged more than 18 years with Takayasu's arteritis (ACR 1990 criteria) were included. Demographic data, clinical features, disease activity using Indian Takayasu Arteritis clinical score (ITAS), Disease Extent Index for TA (DEI.TaK) and damage assessment using TA Damage score (TA), history of conception and maternal and fetal outcomes were recorded from hospital records and telephonic interview. Results are in median and IQR. RESULTS: Of the 64 women interviewed, aged 29 (24-38) years and disease duration 5 (4-10) years, 74 and 38 pregnancies had occurred before and after disease diagnosis in 29 and 20 women respectively. In eight, the diagnosis was made during pregnancy. Age at disease onset was 22 (18-30) years. Type 5 disease was the most common (n = 32, 59.3%), and an equal number of patients had Ishikawa's class I and II disease (n = 26, 40.6%). Median ITAS (n = 44) was 13 (7-16), DEI.Tak 12.5 (9-16.75) and TADS 8 (6.5-10). Twenty-five patients wanted to get pregnant, of which 8 (32%) did not do so because of their disease. Fifteen were unmarried of whom 6 did not marry due to disease. Obstetric outcomes were poorer in pregnancies that occurred after the onset of disease as compared with those before it (RR = 1.5, p = 0.01). Pregnancies after the onset of TA carried a very high risk of maternal [RR3.9 (1.8-8.5), P < 0.001] as well as fetal complications [RR = 2.0 (1.2-3.4), p = 0.001]. Hypertension was the most common maternal complication and occurred most often in the last trimester. The baby weight at birth was lower in pregnancies after disease (2.3 vs. 3.0, p = 0.01). Wong's score greater than or equal to 4 predicted lower birth weight (p = 0.04). ITAS, ITAS-A, DEI. Tak and TADS could not predict obstetric outcomes, and ITAS score exhibited moderate correlation with DEI. Tak (r = 0.78) and TADS (r = 0.58). CONCLUSION: Women with TA suffer from extremely high risk of poor maternal and foetal outcomes. Wong's scoring can be useful to predict birth weight.