Abstract:

Objective: Systemic juvenile idiopathic arthritis is deemed as a subtype of the disease complex known as juvenile idiopathic arthritis but differs in the point of its clinical manifestations and pathophysiological features. Besides, macrophage activation syndrome is a potentially fatal complication of systemic juvenile idiopathic arthritis requiring timely management. The study aims to evaluate the presence and recurrence of macrophage activation syndrome according to the initial symptoms and pattern of different phenotypes in systemic juvenile idiopathic arthritis to reveal the comprehensive association of these interrelated disorders. Methods: The study was conducted at the Department of Pediatric Rheumatology in Istanbul University, Istanbul Faculty of Medicine with a retrospective design, covering the date range 2015 to 2021. Patients, aged 0-18 years, being followed up with a diagnosis of systemic juvenile idiopathic arthritis with or without macrophage activation syndrome were enrolled in the study. The details of demographic data and disease-related clinical and laboratory information were investigated both from their records and the hospital database. The patients with missing or insufficient data and without regular follow-up were excluded from the study. Results: Seventy-eight patients followed up with the diagnosis of systemic juvenile idiopathic arthritis were included in the study. The gender distribution in the study was equivalent randomly (F/M: 39/39). The median age at the study was 174 (29-229) months. The development and recurrence of macrophage activation syndrome revealed statistical significance between the genders; p=0.01 and p=0.02 respectively. Macrophage activation syndrome was more common in patients with evanescent rash (p=0.00) and those without arthritis (p=0.01). The development of macrophage activation syndrome was statistically higher in patients with predominant systemic symptoms (p=0.02) and polyphasic course (p=0.01). The presence of serositis (p=0.01) correlated with the recurrent macrophage activation syndrome. Conclusion: The results from our study were consistent in revealing the association between macrophage activation syndrome and the systemic features in systemic juvenile idiopathic arthritis, despite a lack of correlation with arthritis in the initial presentation of the disease. Early clinical indicators and comprehensive studies are required to predict the development of macrophage activation syndrome.

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