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Since the original description in 1967 by Tomisaku Kawasaki of 50 children with a unique constellation of clinical signs and symptoms that did not fit any known disease, children worldwide of various racial and ethnic backgrounds have been described with Kawasaki disease (KD).1,2 While we have learned much about KD over the last 40 years, there is still much about this disease, including its etiology, pathogenesis, and long-term prognosis that eludes us.

Kawasaki disease, which is an acute, self-limited vasculitis, can affect children of any age. It is the most common cause of acquired heart disease in children of the developed world. KD is identified by a constellation of clinical signs and the exclusion, as appropriate, of similar appearing conditions. The case definition of KD, as outlined in the recent guidelines by the American Heart Association, is described in Table 67-1.3

TABLE 67-1Diagnostic Clinical Criteria for Kawasaki Disease


The epidemiologic patterns of KD have been documented in multiple nationwide surveys in Japan.4–6 The most recent Japanese survey reported 15,979 cases in 2014, the highest number of new cases ever reported in Japan.6 Thus, the annual incidence of KD, approximately 308 per 100,000 children younger than 5 years, is increasing steadily. The recurrence rate is 3–4% in children of Japanese descent, and recurrence was associated with greater risk of a coronary artery aneurysm (CAA).7 A much lower rate was noted for a mixed ethnic population in Canada.8

The epidemiology of KD has been less well characterized in the continental United States, where the annual incidence ranges from 9 to 45.2 cases per 100,000 children younger than 5 years.9–12 The variation in estimates reflects the different regions and ethnic groups that have been studied. The rate of KD hospitalizations was highest in Asian/Pacific Islander children, followed by black children (39 and 19.7 cases ...

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