Anakinra: A safe and effective first-line treatment in systemic onset juvenile idiopathic arthritis (SoJIA)
Systemic onset juvenile idiopathic arthritis (SoJIA) is a rare inflammatory disorder. It can result in disease and treatment-related disability. SoJIA is characterized by remitting fevers, evanescent rash, generalized lymphadenopathy, hepatomegaly/splenomegaly, and/or serositis. Non-responsiveness to standard therapy with corticosteroids and disease modifying antirheumatic drugs is not uncommon. IL-1β has been shown to be a main contributor to the pathogenesis of SoJIA. Anakinra, a recombinant IL-1β receptor antagonist, was shown to be effective in small cohorts of therapy-resistant adult and pediatric Still’s patients. In order to assess the efficacy and safety of first-line anakinra treatment in SoJIA, we reviewed the charts of all SoJIA patients in our institution from 2005 to 2010, searching for first-line anakinra-treated patients. We report the clinical and laboratory course of four SoJIA patients. The mean follow-up was 13.5 (range: 2–50) months. Anakinra was started at doses from 1.5 to 4 mg/kg for a median duration of 3 (range: 3–18) months. Two patients responded to anakinra mono-therapy; two cases required corticosteroids. Normalized body temperatures and the absence of evanescent rashes were achieved after a median of 4 (range: 2–10) days. We did not see treatment-related adverse reactions other than local injection site inflammation. This is the first single-center series, reporting anakinra as first-line treatment in SoJIA. We show rapid efficacy of anakinra in early SoJIA with reduced treatment-related side effects. A subset of patients remains corticosteroid dependent. Further studies are warranted to follow larger cohorts and to assess long-term safety.
KeywordsSystemic JIA Still’s disease Inflammation Anakinra Interleukin-1
We thank our SoJIA patients and families. We thank Prof. Dr. rer. nat. Sybille Bergmann (Department of Clinical Chemistry and Endocrinology, University Hospital “Carl Gustav Carus”, Dresden) for her support in serum cytokine analyses. We further thank the staff of University Children’s Hospital Dresden for taking care of our patients and Christine Hendrix for her contributions.
Conflict of interest
The authors (C.M. Hedrich, N. Bruck, B. Fiebig, and M. Gahr) state no conflict of interest.
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