Authors: C M Hedrich N Bruck B Fiebig M Gahr
Publish Date: 2011/11/15
Volume: 32, Issue: 11, Pages: 3525-3530
Abstract
Systemic onset juvenile idiopathic arthritis SoJIA is a rare inflammatory disorder It can result in disease and treatmentrelated disability SoJIA is characterized by remitting fevers evanescent rash generalized lymphadenopathy hepatomegaly/splenomegaly and/or serositis Nonresponsiveness to standard therapy with corticosteroids and disease modifying antirheumatic drugs is not uncommon IL1β has been shown to be a main contributor to the pathogenesis of SoJIA Anakinra a recombinant IL1β receptor antagonist was shown to be effective in small cohorts of therapyresistant adult and pediatric Still’s patients In order to assess the efficacy and safety of firstline anakinra treatment in SoJIA we reviewed the charts of all SoJIA patients in our institution from 2005 to 2010 searching for firstline anakinratreated patients We report the clinical and laboratory course of four SoJIA patients The mean followup was 135 range 2–50 months Anakinra was started at doses from 15 to 4 mg/kg for a median duration of 3 range 3–18 months Two patients responded to anakinra monotherapy 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 treatmentrelated adverse reactions other than local injection site inflammation This is the first singlecenter series reporting anakinra as firstline treatment in SoJIA We show rapid efficacy of anakinra in early SoJIA with reduced treatmentrelated side effects A subset of patients remains corticosteroid dependent Further studies are warranted to follow larger cohorts and to assess longterm safetyWe 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
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