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Do Corticosteroids Improve Outcome in Kawasaki Disease?

Intravenous methylprednisolone is not indicated for the primary treatment of acute KD in children.

Kawasaki disease (KD) is a mucocutaneous vasculitis apparently triggered by an unknown infectious agent in infants and young children with a genetic predisposition. It is the leading cause of acquired heart disease in children. In Japan, KD affects 1 in every 185 children. In the multiethnic U.S., that figure is about 25 children per 100,000 (5000 new cases annually). Diagnosis rests on a constellation of clinical signs that often emerge over time, including rash, fever, conjunctivitis, erythema of the lips and oral mucosa, and cervical lymphadenopathy. There is no single diagnostic test. Untreated, KD leads to coronary artery aneurysms in up to 25% of children. Standard therapy is intravenous immunoglobulin (IVIG) and high-dose aspirin given within the first 10 days of illness. Even with early treatment, approximately 5% of children develop coronary artery aneurysms, 1% develop giant aneurysms, and some develop recurrent KD.

Until now, studies of the value of adding systemic corticosteroids to the standard treatment have been inconclusive. Investigators in the Pediatric Heart Network performed a multicenter, randomized, placebo-controlled, double-blind trial to see if IV methylprednisolone added to conventional IVIG and aspirin therapy would reduce coronary artery abnormalities in children with acute KD. Children seen between days 4 and 10 of fever who met other strict diagnostic criteria were enrolled. Those with previous IVIG therapy, recent systemic corticosteroid treatment, or previous KD were excluded. Over 2 years, 199 of the 589 children diagnosed with KD were enrolled. All patients received IVIG and high-dose aspirin; in addition, half received 30 mg/kg of IV methylprednisolone and half received placebo.

Compared with the placebo group, the corticosteroid group had shorter initial hospitalization and lower erythrocyte sedimentation rates at 1 week, but both groups had similar coronary dimensions at weeks 1 and 5. In addition, the total number of days of hospitalization, days of fever, incidences of IVIG retreatment, and number of adverse events did not differ between groups. The authors conclude that intravenous methylprednisolone therapy is not indicated in the primary treatment of acute KD in children.

Comment: The number of KD patients collected over a mere 2 years is staggering, especially given the broad exclusion criteria. As stated in the accompanying editorial, "the failure of corticosteroids to benefit these patients underscores the difference between KD and other chronic vasculitides." More prospective, randomized, controlled studies of other therapies are needed to improve outcomes in patients with KD, especially those who do not respond to IVIG therapy. Studies by a multicenter international genotyping consortium are currently examining the genetic component of KD.

— Mary Wu Chang, MD

Published in Journal Watch Dermatology February 14, 2007

Citation(s):

Newburger JW et al. Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease. N Engl J Med 2007 Feb 15; 356:663-75.

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• Medline abstract (Free)

Burns JC. The riddle of Kawasaki disease. N Engl J Med 2007 Feb 15; 356:659-61.

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• Medline abstract (Free)