Update on etio and immunopathogenesis of Kawasaki disease.

PURPOSE OF REVIEW: This review first discusses the pathogenesis of Kawasaki disease based on the results of recently performed studies aimed at identifying Kawasaki disease-susceptibility genes and the results of analyses of the immune system. Following that, we discuss the findings generated using a murine Kawasaki disease arteritis model and speculate regarding the mechanism of Kawasaki disease onset based on immune function aberrations seen in that model. RECENT
FINDINGS: Recent advances in gene analysis studies of Kawasaki disease are contributing not only to prediction of disease susceptibility but also to improving our understanding of the pathogenesis of Kawasaki disease and development of new improved therapies. In addition, Th17/Treg imbalance is observed in patients with acute-phase Kawasaki disease. Th17/Treg imbalance may be an important factor causing disturbed immunological function. IL-17 induced by Th17 cells have proinflammatory properties and act on inflammatory cells, thereby inducing expression of cytokines and chemokines and resulting in tissue inflammation.
SUMMARY: Kawasaki disease vasculitis may be triggered by aberrant activation of inflammatory cytokines mediated by IL-17 that is produced by Th17 cells that have been activated by some infectious agent(s).