Kawasaki disease may be a hyperimmune reaction of genetically susceptible children to variants of normal environmental flora

Abstract

Kawasaki disease (KD) is an acute febrile systemic vasculitis of unknown etiology that occurs predominantly in children <5 years of age. For the etiopathogenesis of KD, there is no agreement even as to whether KD is an infectious disease or an immune-mediated disease. The epidemiologic characteristics of KD, including the strict predilection of age in all ethnic groups and the gradually increased incidence after the KD emergence, suggest that KD is affected by the immune maturation in early childhood that may be determined by genetic factors, and KD is also affected by the changed environmental circumstances such as improved public hygiene. We postulated that the pathogenesis of KD is a hyperimmune reaction in genetically susceptible children to the variants of normal flora, which are induced by the environmental factors. Using this hypothesis, we might partly explain the clinical and epidemiological characteristics of KD. We expect that this hypothesis may help to determine the causative agents for KD in the near future.

Figure 1

Annual incidence of KD cases per 100,000 children age < 5 years in Japan, 1961–2000.

Figure 2

Annual incidence of KD cases per 100,000 children age < 5 years in Korea, 1972–2005.