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Monitoring serum IL-18 levels is useful for treatment of a patient with systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome

Tomonari Shigemura1, Takashi Yamazaki1, Yosuke Hara1, Jing-Ni Ou2, Anne M Stevens2, Hans D Ochs2, Kenichi Koike1 and Kazunaga Agematsu3*

Author Affiliations

1 Department of Pediatrics, Shinshu University School of Medicine, Matsumoto 390-8621, Japan

2 Seattle Children's Research Institute, 1900 9th Avenue, Seattle, WA 98101, USA

3 Department of Infection and Host Defense, Shinshu University, Graduate School of Medicine, Matsumoto 390-8621, Japan

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Pediatric Rheumatology 2011, 9:15  doi:10.1186/1546-0096-9-15

Published: 13 July 2011


Systemic juvenile idiopathic arthritis (sJIA) is a systemic inflammatory disease characterized by arthritis, spiking fever and a skin rash that is frequently complicated by macrophage activation syndrome (MAS), a life-threatening disorder. We report a 22-month-old girl with sJIA who developed severe MAS but was successfully treated with corticosteroids, cyclosporin A, and non-steroidal anti-inflammatory drugs by monitoring serum IL-18 levels. IL-18 is an extremely useful cytokine for monitoring the activity of sJIA and MAS, and serum IL-18 can be used as an indicator for the effectiveness of treatment and the decision to discontinue therapy.