Systemic onset juvenile idiopathic arthritis: a single center experience

Abstract

Systemic juvenile idiopathic arthritis (sJIA) presents with prolongedfever and systemic features such as arthritis, rash, lymphadenopathy,hepatosplenomegaly and serositis. In this study, we aimed to evaluate theclinical and laboratory findings, and outcomes of sJIA patients from a tertiaryrheumatology center.Between 2010-2017, patients who had been diagnosed with sJIA, participatedin the study. The demographics, clinical and laboratory features, and outcomes,were evaluated retrospectively.Seventy-five sJIA (%56 male) patients were enrolled. The mean age at diagnosiswas 6,45±4,80 years. At the time of diagnosis, the most common findings werefever (%100) followed by arthritis (78,7%), and rash (66,2%). Twenty-fourpercent of the patients present with macrophage activation syndrome (MAS)at the time of diagnosis. Totally, 36% of the patients had at least one MASattack during the course of the disease. 46% of the patients had polyphasiccourse while 54% had one attack (26% monophasic, 28% persistant). All ofthe patients were treated with non-steroid anti-inflammatory drugs (NSAID)and/or corticosteroids at the beginning of the disease. Twenty percent ofthe patients reached remission with corticosteroid or disease-modifyinganti-rheumatic drugs (DMARDs) however the rest of the patients needed atleast one biologic agent. Anakinra was the most common first-line biologictreatment choice (n=45). Fourteen (18,7%) of the patients had polyarticularjoint involvement during the disease course, and 5 of them achieved remissionwith tocilizumab.Systemic JIA is an important disease with high risk of morbidity and mortality.As our center is one of the most important tertiary referral rheumatologycenters in the country, we had a high MAS incidence. Eighty percent of thepatients achieved remission with a biological agent. Anti-IL1 drugs are mostlypreferred for ongoing systemic inflammation. Anti-IL-6 agents are veryefficient in patients with a polyarticular course.