Anand Prahalad Rao, Anugna B, Anil Kumar Tennelli, Debasis Patro, Paromita Nath, S. D. Subba Rao, Jyothi Raghuram
European Journal of Rheumatology - 2026;13(1):1-7
Objective: Systemic-onset juvenile idiopathic arthritis (sJIA) follows a variable and unpredictable course, with long-term outcomes remaining inconsistent worldwide despite therapeutic advancements. This study aimed to evaluate the disease course and clinical outcomes of the sJIA cohort after 5-year follow-up. Methods: The study retrospectively analyzed 100 sJIA patients with at least 5 years of follow-up. Ten patients were excluded due to insufficient data. Data on demographics, clinical features, treatments, outcomes, and complications were collected. Results: The mean age at disease onset was 6.75 +/- 3.64 years, with a median follow-up of 7 years. Common clinical manifestations included fever (100%), arthritis (96%), rash (55%), and hepatosplenomegaly (32%). Polyarticular arthritis was observed in 70.83% of cases. Treatment modalities included Non-steroidal anti-inflammatory drugs (100%), steroids (89%), methotrexate (86%), thalidomide (31%), lenalidomide (21%), and tocilizumab (13%). While 11% of patients responded to NSAIDs alone, 46% achieved remission with glucocorticoids and methotrexate. A total of 46% of the patients exhibited a monocyclic disease course, and 27% of the patients had polycyclic and persistent courses. On multivariate analysis, diagnostic delay (odds ration (OR) = 1.02, 95% CI: 1.01-1.03) and symmetric arthritis (OR = 2.36, 95% CI: 1.65-3.32) were identified as key predictors of persistent disease. Common complications included infections (20%), joint damage (14%), and macrophage activation syndrome (11%). At 5 years, 62% achieved drug-free remission, 16% remained in remission on medication, and 21% had active disease. Mortality was noted in 1 patient due to a suspected cerebrovascular accident or meningitis. Conclusion: Delayed diagnosis and symmetric arthritis at onset increased the risk of persistent disease, highlighting the need for early and aggressive treatment for better outcomes.
