Abstract

Objective: Systemic juvenile idiopathic arthritis (sJIA) is one of seven subtypes of JIA. The aim of this study was to investigate the characteristics, the course and the outcome of sJIA patients in our clinic and to identify what has changed over the last decade in sJIA. Material and Methods: Files of sJIA patients that were followed between March 2002 and April 2019 were evaluated. Results : During the study period 36 patients were diagnosed with sJIA in our clinic (19 were male). Mean age of patients was 81.7±50.1 months at the onset of disease. Most common presenting features were fever (100%), arthritis (83.3%), rash (66.7%) and hepatosplenomegaly (38.9%). Seven patients (19.4%) were diagnosed as macrophage activation syndrome (MAS) due to sJIA. All patients had received corticosteroids. Additional treatments (disease-modifying drugs, intravenous immunoglobulin and biological agents) were given to 29 patients. In our clinic, anti-IL-1 agents have been used since 2011. In this group 26 (72.2%) patients were diagnosed after 2011. After the usage of biological agents, the duration of steroid exposure significantly decreased (p= 0.001). Conclusion: After the introduction of biological agents in the treatment of sJIA, the duration of steroid exposure decreased significantly. Considering anti interleukin (IL)-1 and anti IL-6 treatments among the priority treatments in sJIA patients is important in terms of both providing rapid disease control and reducing side effects related to drugs.

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