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Kawasaki Disease (Mucocutaneous...
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PEDIATRICS Vol. 112 No. 6 December 2003, pp. e495-e497

EXPERIENCE AND REASON

Are Children With Kawasaki Disease and Prolonged Fever at Risk for Macrophage Activation Syndrome?

Aleixo Muise, MD PhD*, Susan E. Tallett, MB BS Med FRCPC*,{ddagger} and Earl D. Silverman, MD FRCPC*,§,||

* Department of Paediatrics
{ddagger} Division of Pediatric Medicine
§ Division of Rheumatology
|| Department of Immunology, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada M5G 1X8

Kawasaki disease (KD) patients are known to be at increased risk for coronary artery lesions. We present evidence of another possible complication associated with KD: macrophage activation syndrome (MAS). In this case, a patient with KD and prolonged fever developed MAS. This case is of particular interest because of the late age of onset and recurrent nature of KD as well as the complication of MAS. We also present a review of the literature that supports the inclusion of MAS as an infrequent complication of KD.

Key Words: Kawasaki disease • macrophage activation syndrome • hemophagocytosis • intravenous immunoglobulin

Abbreviations: KD, Kawasaki disease • IVIG, intravenous immunoglobulin • ASA, acetylsalicylic acid • MAS, macrophage activation syndrome • LDH, lactate dehydrogenase

Received for publication Jun 6, 2003; Accepted Aug 12, 2003.


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