Multisystem Inflammatory Syndrome in Children (MIS-C) with C
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Recently, an increasing number of patients of SARS-CoV-2 with syndrome which overlaps with Kawasaki Disease (KD) has been reported, supporting the idea that infection is one of triggers of KD. This discussion summarized the reports of simultaneous familial KD cases for understanding etiopathogenesis of both KD and Multisystem Inflammatory Syndrome in Children (MIS-C) related to COVID-19.

It discusses the etiology of these syndromes from the point of view of infection and genetic susceptibility. In 1967, Dr. Kawasaki first described Kawasaki disease (KD) as an acute febrile, mucocutaneous lymph node syndrome with self limited vasculitis that primarily affects infants and children, usually under 5 years of age.

The etiopathogenesis of KD still remains unclear, but is discussed from the view of interplay of two aspects, genetic susceptibility and infection. There were many familial or sibling case reports, familial aggregation of the disease and higher prevalence in Northeast Asia, that indicate genetic susceptibility leading to KD. In fact, evidence of several common variants associated with KD susceptibility were identified in KD patients.

On the other hand, several factors have been proposed as triggers for KD, and infection is considered as one of the candidates. KD in the extra-tropical latitudes of the Northern Hemisphere shows January through March seasonality, and community-wide outbreak was occasionally reported. And also, the children under 6 months of age, who have passive transmission of maternal Igs rarely develop KD. These reports strongly indicate the involvement of infection in KD onset and several virus or antigen have been considered as the trigger of KD, although any consensus is not established ever.