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.

The prevalence and clinical characteristics of anti-HMGCR (anti-3-hydroxy-3-methyl-glutaryl-coenzyme A reductase) antibodies in idiopathic inflammatory myopathy: an analysis from the MyoCite registry.

This study aimed to determine the prevalence and clinical characteristics of anti-HMGCR antibodies in idiopathic inflammatory myositis (IIM) at a tertiary care centre in northern India. Data (adult and children) were retrieved from the MyoCite dataset, identifying patients with polymyositis, dermatomyositis, and antibody-negative IIM whilst fulfilling the ACR/EULAR criteria. SLE, sarcoidosis, and systemic sclerosis were included for comparison as disease controls. The baseline clinical profile, laboratory tests, and muscle biopsies were retrieved and analysed. Descriptive statistics and non-parametric statistics were used for comparison. Among 128 IIM (112 adults, 16 children, M:F 1:2.8) of age 37 (24-47) years and 6 (3-17) months disease duration, 4 (3.6%) young adults tested positive for anti-HMGCR antibodies. All children and disease control tested negative for the antibody. Anti-HMGCR + IIM exhibited higher muscle enzymes [AST (367 vs 104 IU/L, p = 0.045), ALT (502 vs 78 IU/L, p = 0.004), and CPK (12,242 vs 699 IU/L, p = 0.001] except lactate dehydrogenase with less frequent systemic features such as fatigue than antibody-negative IIM. One young girl presented with a Limb-girdle muscular dystrophy (LGMD) with chronic pattern. None of the patients exhibited rashes, statin exposure, or cancer, though one had anti-Ro52 and mild disease. Our observations depict a younger population while affirming previous literature, including NM-like presentation, and chronic LGMD-like pattern of weakness in one case. Although a small number of children were included, ours is one of the few paediatric studies that evaluated HMGCR antibodies thus far. Further investigations in a larger Indian cohort are warranted to substantiate our findings.

Poor maternal and foetal outcomes in women with systemic sclerosis: an interview-based study at a tertiary centre.

Poor obstetric outcomes are described in rheumatic diseases (RDs) such as systemic sclerosis (SSc). We assessed the effect of the disease in Indian women and compared with those in developed countries and other RDs. Women with SSc (ACR/EULAR 2013 criteria) registered at a tertiary care centre (2010-2016) were interviewed by teleconsultation. Pregnancies occurring after disease onset were compared with those occurring prior to it. Maternal complications included antepartum hemorrhage, postpartum hemorrhage, spontaneous abortion, preterm rupture of membrane, oligohydramnios, infection, prolonged labour, and foetal complications including low birth weight (LBW), intrauterine death (IUD), preterm delivery, and neonatal infection. Results were expressed as median (Interquartile range). Of 200 SSc, 75 patients aged 31 (22-38) years and disease duration 41 (32-50) months were interviewed. Diffuse cutaneous SSc was the most common (42.56%). 127 conceptions before the onset of SSc were compared with 15 after. Among post-diagnosis, 9 (60%) were live births, 3 (20%) spontaneous abortions 1 (6.7%) induced abortion, 2 (13.3%) IUD. Of the live births, 4 (26.7%) were preterm and 3 (20%) were LBW. Pregnancies after disease onset had a higher rate of maternal (OR - 4.9) and foetal (OR - 9.9) complications compared to pregnancies before SSc. Compared to the Italian cohort, Indian SSc patients had a higher abortion rate (OR - 5.8), frequent lower section ceaserean section (OR - 9.4) and lower live births (OR - 0.05). More frequent caesarean deliveries (OR - 93), preterm deliveries (OR - 20) when compared with lupus and favourable maternal outcomes (OR - 0.15), higher preterm deliveries (OR - 9.6) in comparison with Takayasu arteritis were noted. SSc incurs a higher risk of poor maternal as well as the foetal outcome.

Rheumatology education in India: a survey-based cross sectional study.

This study aimed at understanding the perception and perspectives of rheumatology trainees about specialist training in India. Rheumatology trainees (Doctorate of Medicine, Diplomate of National Board) in Indian universities (2010 onwards) were contacted to complete a validated e-survey consisting of 41 questions to evaluate the current rheumatology training in India. Of 53 respondents (M:F 3.4:1, mean age 37 years ± 12.7), 81.1% trained at government hospitals, and 15.1% trained at private hospitals. During training period, 37.5% respondents were exposed to 6-7 h of didactics/week. They treated nearly 175 patients (175 ± 35.4) per week and reported a reasonable level of independence in management of patients with common rheumatic diseases (RDs) during their training (7.5 ± 0.7 SD). However, nearly one-third of the trainees were not exposed to basic immunology and laboratory techniques. Similarly, placement in the radiology department was not a part of the curriculum for nearly half of the trainees, 80% were not confident to manage paediatric RDs and soft tissue rheumatism. Almost 60% did not feel comfortable in addressing ancillary care including patient counselling as they had not received formal training. Among the participants, 59% were not satisfied by the current system of assessment, 86.8% suggested for multiple time point-based assessment systems and 45.3% preferred objective and subjective assessment in final examinations. Rheumatology training in India offers notable exposure to patients and independence in managing cases. However, there is an unmet need for improvement in training in the field of laboratory, radiology and ancillary care, and to overhaul assessment system by including objective evaluation.

Patient Perspectives on the Effect of the SARS-CoV-2 Pandemic on Patients With Systemic Sclerosis: An International Patient Survey.

BACKGROUND: The coronavirus disease (COVID-19) pandemic and its subsequent effects on health care systems have significantly impacted the management of chronic rheumatic diseases, including systemic sclerosis (SSc). METHODS: In this context, a 25-item anonymized e-survey was posted on the Twitter and Facebook e-groups and pages of various scleroderma organizations and patient communities to assess the problems faced by patients with SSc during the pandemic, with a focus on effects on the disease, drug procurance, continuity of medical care, and prevalent fears among patients. RESULTS: Of the 291 participants (median age of 55 [43.5-63] years, 93.8% females), limited systemic sclerosis was the most common diagnosis (42.3%). Many patients experienced problems attributable to the COVID-19 pandemic (119, 40.9%), of which 46 (38.7%) required an increase in medicines, and 12 (10.1%) of these needed hospitalizations for disease-related complications. More than one-third (36.4%) were on glucocorticoids or had underlying cardiovascular risks (39%) that would predispose them to severe COVID-19.A significant proportion (38.1%) faced hurdles in procuring medicines or experienced disruption in physiotherapy sessions (24.7%). One-quarter (24.1%) felt it was difficult to contact their specialist, whereas another 7.2% were unable to do so. Contracting COVID-19 was the most prevalent fear (71.5%), followed by infection in the family (61.9%), and a flare of the disease (45.4%). Most respondents preferred teleconsultations (55.7%) over hospital visits in the pandemic period. CONCLUSION: The results of the patient survey suggest that the COVID-19 pandemic has affected many patients with SSc and may translate to poorer outcomes in this population in the postpandemic period.

Insights into the knowledge, attitude and practices for the treatment of idiopathic inflammatory myopathy from a cross-sectional cohort survey of physicians.

The idiopathic inflammatory myopathies (IIM) are heterogeneous and lead to high morbidity and mortality. Knowledge deficits among healthcare professionals could be detrimental to clinical care. Identifying areas of deficient Knowledge, Attitude and Practice (KAP) of IIM can improve physician education and patient outcomes. To assess the proportion of physicians treating IIM with poor KAP and identify the key areas of deficit. An anonymised and validated e-survey (57 questions) was circulated among physicians treating IIM (purposive sampling). Responses were evaluated using the Likert scale for good (> 70% correct response), poor (> 20% chose > 2 answers) and the rest as intermediate consensus. Descriptive statistics were used. Intergroup comparisons were done using non-parametric tests. Of 80 (9.1% of 883) respondents, 90% were rheumatologists and 37.5% academicians. The knowledge base of treating physicians was good in specific domains such as triggers (80-90%), clinical presentation (MDA5, statin myositis, steroid myopathy, anti-synthetase syndrome) (82-92%), IIM mimics (41-89%), investigations (23-92%) and risk of osteoporosis in IIM (79%). There is also an intermediate knowledge base/consensus for outcome measures (30-56%) and response criteria (30-53%). There was poor knowledge and consensus on trials (27-34%), EULAR/ACR criteria (31%) and exercise-based interventions (17-62%). While 90% agree on the need for muscle biopsy to diagnose polymyositis, only one-third advocated it for juvenile and adult DM. Physicians have a good understanding of the triggers, clinical presentation and mimics of IIM. Poor to intermediate knowledge and consensus exists regarding muscle biopsy, outcome measures, response criteria and exercise-based interventions, which could be addressed through future focussed educational initiatives.

Pulmonary mucormycosis in systemic lupus erythematosus: successful management of a case along with review of literature.

Pulmonary mucormycosis is rare in systemic lupus erythematosus. A 20-year-old lady with lupus nephritis and neuropsychiatric lupus was treated with injection methylprednisolone and cyclophosphamide. After few days, she developed fever, breathlessness, and hoarseness of voice. After neck and chest imaging, possibility of mucormycosis was considered which was later confirmed on microbiological test. Patient was treated with conventional amphotericin B. Literature review was done, and 8 patients with disseminated or pulmonary mucormycosis were identified with SLE. In patients with high index of suspicion, early imaging can help in diagnosis and early and aggressive management even with conventional amphotericin B can result in favorable outcome. Key Points • Pulmonary mucormycosis in systemic lupus erythematosus is rare. • Radiological investigation can guide towards diagnosis. • Early and aggressive treatment can lead to good outcome.

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.