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Purpose of review: Since the first suggestion of a superantigen hypothesis for Kawasaki disease over a decade ago, debate on the aetiology remains inconclusive. This article reviews recent publications that address the role of superantigens of group A Streptococcus and Staphylococcus aureus in the pathogenesis of Kawasaki disease.

Recent findings: Over the past few years, new superantigens produced by group A Streptococcus and S. aureus have been increasingly identified, bringing the total known number to more than 30. Several studies on T-cell V[beta] repertoires and seroloepidemiology have demonstrated evidence for the involvement of single or multiple superantigens produced by the two pathogens. The associated superantigens differed in those studies, including streptococcal pyrogenic toxins A and C, staphylococcal enterotoxins A-C, and toxic shock syndrome toxin 1. These disparate findings suggest that the inflammation of Kawasaki disease does not result from a single agent but rather a final common inflammatory pathway in genetically susceptible individuals after numerous infectious agents.

Summary: Certain staphylococcal and streptococcal superantigens are suggested to be responsible for the development of Kawasaki disease. A better understanding of the precise role of the causative agents will lead to accurate diagnosis, more targeted therapy and an improvement of coronary outcomes.

(C) 2007 Lippincott Williams & Wilkins, Inc.