Clinical profile and outcome of patients with ANCA-associated vasculitis with special reference to vasculitis damage...

Antineutrophil cytoplasmic autoantibody-associated vasculitis (AAV) is the most commonly encountered primary small-vessel vasculitis, with an annual incidence of 20 per million. The data on AAV from the Southeast Asian ethnicity is scarce. The present longitudinal observational study was undertaken to determine the clinical profile and outcomes in fifty-eight consecutive patients of AAV attending the Department of Rheumatology in a tertiary care centre in India. The majority (39, 67.2%) were females with a mean (± SD) age at baseline of 39.2 (± 15.2) years. Granulomatosis with polyangiitis (GPA) was the most common phenotype, accounting for 40 cases (69.0%). The patients were followed up for a mean (± SD) duration of 35.4 (± 31.4) months. The most commonly affected organ domains were the respiratory (46, 79.3%), musculoskeletal (41, 70.7%), and the renal domains (38, 65.5%). In our cohort, a significantly higher number of patients exhibited ophthalmological involvement (31, 53.4%). There were a total of 22 relapse events among 11 patients, representing a relapse rate of 19%. The case-fatality rate was 10.6%. The majority received treatment with intravenous methylprednisolone (46; 79.3%) and intravenous cyclophosphamide (42; 72.4%) during the induction phase, while rituximab (28; 48.3%) was the preferred first-line maintenance agent. BVAS of 22.5 was related to VDI ≥ 5 (p = 0.000). The mean (± SD) Vasculitis Damage Index (VDI) accrued was 2.41 (± 1.97). The most common treatment-related damage included osteoporosis (40%), diabetes (40%), and cataract (13%), whereas the common disease-related damage included diastolic hypertension (58%) and impaired lung function (27%). Higher Birmingham Vasculitis Activity Score (BVAS) at presentation (OR: 1.25, 95% CI 1.031-1.522) and relapse of scleritis (OR: 35.27, 95% CI 1.152 -1000.000) were found to be independent predictors of high VDI ≥ 5. This is the first study from Eastern India that has looked into the clinical profile of AAV and its damage index.