&NA;Kawasaki disease is an acute vasculitis of young children that is complicated by the development of myocarditis and coronary artery abnormalities. Recent studies indicate that the prevalence of cardiovascular abnormalities can be significantly reduced by treating patients during the first 10 days of illness with high‐dose intravenous gammaglobulin, particularly at a dose of 2 g/kg. Thus, early recognition and prompt treatment of this illness is critical for a successful outcome. This process would undoubtedly be facilitated if the etiologic agent or toxin that causes Kawasaki disease were known. In this regard, studies of the past year strongly suggest that a superantigen plays an important role in stimulating the massive immune activation associated with this illness. These observations may provide an important new direction for investigations into the etiology and pathogenesis of Kawasaki disease.Current Opinion in Rheumatology 1993,5:41‐50