Cardiovascular Lesions of Kawasaki Disease: From Genetic Study to Clinical Management

Kawasaki disease (KD) is an acute febrile systemic vasculitis that was first described by Kawasaki et al.(1) in 1967 in Japanese(2) and in 1974 in English. Currently, it is the leading cause of acquired heart disease in children in developed countries; however, its etiology remains unknown.(3-5) KD mainly affects children less than 5 years of age, especially those in Asian countries. In Japan, Korea, and Taiwan, the incidence ranges from 69 to 218 cases per 100,000 children less than 5 years of age.(6-9) The incidence of KD in Taiwan has increased from 66 to 69 cases per 100,000 children aged less than 5 years.(9-12) Its incidence worldwide is increasing, especially in Japan, where, in 2010, Nakamura et al. reported the country’s highest rate of 239.6 cases per 100,000 children aged 0–4 years.(13) An epidemiologic survey of KD in Taiwan spanning 2003–2006 found that 1.5% of all cases was recurrent (having a second episode of KD and receiving intravenous immunoglobulin [IVIG] treatment).(9) In Taiwan, KD occurs most frequently in the summer (April to June) and least frequently in the winter; for unknown reasons, its seasonal occurrence varies in other countries. The most serious complication of KD is the development of coronary artery lesions (CAL), including myocardial infarction, coronary artery fistula formation,(14) coronary artery dilatation, and coronary artery aneurysm.(15)