Kawasaki disease (KD) is one of the most common vasculitides of childhood. The incidence of KD is greatest in children who live in East Asia (eg, Japan, Korea, Taiwan) or are of Asian ancestry living in other parts of the world. Other risk factors include male gender, age between six months and five years, and family history of KD.
The etiology of KD remains unknown. Inflammatory cell infiltration into KD vascular tissue leads to vascular damage, but the stimulus for this inflammatory infiltration has not been identified.
The similarities between KD and other pediatric infectious conditions suggest that KD is caused by a transmissible agent. However, no studies have convincingly identified a specific virus, bacteria or bacterial toxin, or other pathogen associated with KD. The etiology may be a previously unidentified infectious agent. An alternative theory to a specific inciting agent is that KD represents a final common pathway of immune-mediated vascular inflammation following a variety of inciting infections.
Genetic factors appear to contribute to the pathogenesis of this disorder, as suggested by the increased frequency of the disease in Asian and Asian-American populations and among family members of an index case. A number of gene polymorphisms are associated with an increased susceptibility to KD, and some of these variants are also associated with coronary artery lesions and aneurysm formation.