Key result
The pathogenesis of Kawasaki disease likely involves an initial infection by microorganisms producing superantigens and heat-shock proteins that trigger an autoimmune response in genetically susceptible individuals.
Why the study?
The causes and pathogenesis of Kawasaki disease remain a mystery despite various theories regarding infectious agents and host genetic susceptibility.
This review synthesizes evidence to propose a comprehensive etiological model for Kawasaki disease involving microbial superantigens, heat-shock proteins, and genetic susceptibility to explain the development of coronary artery lesions.
Hypothesis-generating model for Kawasaki disease; leaves open prospective validation before any clinical application.
Kawasaki disease (KD) is a multisystem vasculitis that primarily affects the coronary arteries of young children. The causes of KD remain a mystery. It is suspected that some sort of infectious agent is involved because KD has epidemicity and seasonality. That said, the incidence of the disease is high among Japanese people, so it can be speculated that the hosts may have some sort of genetic characteristic that leaves them susceptible to KD. Various theories regarding the etiology have been asserted, such as the infectious vasculitis theory, autoantigen theory, superantigen theory, and RNA virus theory; however, none of them have been able to overcome this epidemicity. Taking into consideration the knowledge gained from previous reports, the best scenario explaining the pathogenesis is "individuals with certain genetic backgrounds are affected by microorganisms which trigger KD." In this article, the pathogenesis of KD is discussed with a focus on the microorganisms mentioned above, along with the previous and current hypotheses as well as my own opinion.
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Satoru Nagata (2019) conducted a review in Kawasaki disease. Infectious agents (superantigens and heat-shock proteins) was evaluated. The pathogenesis of Kawasaki disease likely involves an initial infection by microorganisms producing superantigens and heat-shock proteins that trigger an autoimmune response in genetically susceptible individuals.
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