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.
KD, 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 62–1.
Table 62–1. Diagnostic Clinical Criteria for KD32 |Favorite Table|Download (.pdf)
Table 62–1. Diagnostic Clinical Criteria for KD32
- Fever ≥ 4 days with at least four of the following:
- Bilateral conjunctival injection
- Mucous membrane changes: injected, fissured lips; strawberry tongue; injected pharynx
- Changes of the peripheral extremities: edema or erythema of the hands and feet; periungal desquamation
- Polymorphous rash
- Cervical lymphadenopathy
- Patients with fever for ≥5 days, with <4 clinical features and echocardiographic evidence of coronary artery disease*
The epidemiologic patterns of KD have been documented in multiple nationwide surveys in Japan.3–5 During a 14-year period (1987–2000), the mean incidence of KD in Japan was 6059 cases per year.6 The annual incidence of KD, approximately 150 per 100,000 children, younger than 5 years, is increasing steadily.
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.7,8 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 per 100,000 children, respectively).9 In Hawaii, where there is a large Japanese-American population, the incidence of KD in Japanese-American children is as high as 197.7 per 100,000 children younger than 5 years.10
The increased incidence in Asian/Pacific Islanders in the United States and in Japan suggests a genetic influence on KD susceptibility. In addition, researchers have described KD pedigrees with multiple affected members.11–13 A retrospective study, evaluating 18 families found no clear pattern of inheritance, suggesting that multiple genetic polymorphisms may contribute to KD susceptibility.14 A genome-wide linkage analysis of KD using affected sibling ...