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Kawasaki disease is an acute systemic vasculitis of childhood. Children are predominantly affected at less than 5 years of age, and coronary artery involvement is responsible for most of the morbidity and mortality of the disease. Since the institution of intravenous immune globulin in the treatment of the disease, outcome has significantly improved. Although multiple infectious agents and toxins have been implicated in the etiology of the disease, none has been identified. Activation of the immune system is known to occur in the acute stage of the disease and plays an important role in the pathogenesis of the disease.