Abstract
Kawasaki disease is a vasculitis syndrome of unknown cause, first reported by Tomisaku Kawasaki in 1967. It is the leading cause of acquired heart disease in developed countries. Because the causes of Kawasaki disease have not been identified, there are no specific therapies for Kawasaki disease patients. Now, intravenous immunoglobulin (2 g/kg single infusion) plus aspirin (30 mg/kg/day) therapy is considered to be the standard therapy. However, 20% of the patients do not become afebrile despite completion of the intravenous immunoglobulin therapy; these intravenous immunoglobulin non-responders are considered to be high risk for coronary artery lesion. Recently, new models to predict intravenous immunoglobulin non-responders were developed in Japan. Risk stratification of initial therapy for Kawasaki disease patients became not a vision, but a fact. In this review, I explain the history of acute therapy for Kawasaki disease patients, the most recent therapeutic strategy, and future perspectives.
