Evaluation of patients with systemic onset juvenile idiopathic arthritis

Abstract

INTRODUCTION: Systemic juvenile idiopathic arthritis (sJIA) is a rare, systemic inflammatory disease classified as a subtype of JIA. There are few studies on sJIA in our country. The aim of this study was to investigate the clinical and laboratory features of patients with sJIA.METHODS: Three hundred three cases with JIA followed up in our clinic were screened, 18 of them (5.9%) diagnosed with sJIA wereincluded to our study. The data was obtained from the electronic file records retrospectively.RESULTS: The median age of the patients was 11.7 years (5.3-20 years), the female/male ratio was 4/14, and the median diagnosis age was 4.9 years (1.5-15.2 years). The median diagnosis delay was 4 weeks (2 weeks-10.8 years). The most common complaints were fever and arthritis which were seen in all cases (n = 18, 100%). One patient had monoarthritis while the other cases had more than one joint involvement. The most common form was the monocyclic form (n = 7, 38.8%). The most common complications were short stature and macrophage activation syndrome which developed in 2 cases. Almost all patients were treated by corticosteroids (n = 17, 94.4%), biological therapy was used in 7 patients (38.8%). Biological treatments were tocilizumab in five patients, canakinumab in one and anakinra in 1 one patient. Remission was achieved in all cases except one patient who was out of follow-up.DISCUSSION and CONCLUSION: SJIA can be challenging to diagnose. Fever and rash in sJIA clinic may inadvertently be attributed to infectious disease. Corticosteroids were the main treatment modalities in treatment, whereas in resistant cases biologicals were highly effective and safe.