title: Rapid and sustained remission of systemic juvenile idiopathic arthritis-associated macrophage activation syndrome through treatment with anakinra and corticosteroids.
abstract: We 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.
authors.affiliation: Division of Rheumatology and Immunology, University Children's Hospital, Technical University Dresden, Fetscherstrasse 74, Dresden, Germany.
authors.forename: Normi
authors.initials: N
authors.lastname: Bruck
authors.forename: Meinolf
authors.initials: M
authors.lastname: Suttorp
authors.forename: Maria
authors.initials: M
authors.lastname: Kabus
authors.forename: Georg
authors.initials: G
authors.lastname: Heubner
authors.forename: Manfred
authors.initials: M
authors.lastname: Gahr
authors.forename: Frank
authors.initials: F
authors.lastname: Pessler
mesh_terms: D018501:Antirheumatic Agents / Q000008:administration & dosage*; D001171:Arthritis, Juvenile / Q000150:complications*; D002648:Child; D003907:Dexamethasone / Q000008:administration & dosage*; D004361:Drug Tolerance; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000008:administration & dosage*; D055501:Macrophage Activation Syndrome / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D011239:Prednisolone / Q000008:administration & dosage*; D012074:Remission Induction / Q000379:methods
chemical_list: D018501:Antirheumatic Agents; D005938:Glucocorticoids; D053590:Interleukin 1 Receptor Antagonist Protein; D003907:Dexamethasone; D011239:Prednisolone