Pathogenesis and long-term prognosis of Kawasaki disease : New therapeutic strategy using vasogenesis therapy

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Kawasaki disease (KD) is an acute, self limiting systemic vasculitis syndrome of unknown origin that mainly affects small and medium-sized arteries, particularly the coronary artery. From 3-8% of children develop coronary artery lesions (CALs) after intravenous immunoglobulin (IVIG) therapy, and approximately 4% of KD children develop ischemic heart disease with myocardial infarction. On the basis of clinical features and epidemiological data, KD might be caused by very common infectious agents in some susceptible hosts. We investigated the difference of gene expression between patients with KD and control subjects by use the Affymetrix GeneChip system. Some genetic factors might participate in pathogenesis of KD, and some angiogenesis factors might concern in incidence of CAL. The cardiovascular spectrum of 2180 patients with acute KD experienced over the last 30 years. Of all the patients, 14.9% were diagnosed as having CALs. From 1991 we started IVIG treatment, since then the incidence of CALs has declined to 8.7%. Myocardial infarction occurred in 24 patients, 7 of whom died. Long-term follow-up study is necessary in KD patients, and should pay attention to the possible development of premature atherosclerosis.