• J Clin Rheumatol · Jan 2011

    Case Reports

    Rapid and sustained remission of systemic juvenile idiopathic arthritis-associated macrophage activation syndrome through treatment with anakinra and corticosteroids.

    • Normi Bruck, Meinolf Suttorp, Maria Kabus, Georg Heubner, Manfred Gahr, and Frank Pessler.
    • Division of Rheumatology and Immunology, University Children's Hospital, Technical University Dresden, Fetscherstrasse 74, Dresden, Germany.
    • J Clin Rheumatol. 2011 Jan 1; 17 (1): 23-7.

    AbstractWe describe 2 patients with systemic juvenile idiopathic arthritis and macrophage activation syndrome. Treatment with recombinant interleukin 1 receptor antagonist (anakinra) and a corticosteroid rapidly induced remission, which could be maintained with anakinra monotherapy at a stable dose of 2 mg/kg per day. Pain at the injection site during the initial injections was the only adverse effect attributable to anakinra. Untoward effects of corticosteroid treatment were mild because prolonged therapy with high-dose corticosteroids could be avoided. These results suggest that early institution of interleukin 1 blockade merits further investigation for the treatment of macrophage activation syndrome and, perhaps, related conditions such as hemophagocytic lymphohistiocytosis.

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