Does Kawasaki disease have a genetic predilection?

Updated: Jul 29, 2018
  • Author: Tina K Sosa, MD; Chief Editor: Russell W Steele, MD  more...
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A genetic predilection to KD has long been suspected. [23, 24] Siblings of affected children have a 10-20 times higher probability of developing KD than the general population, and children in Japan whose parents had KD seem to have a more severe form of the disease and to be more susceptible to recurrence. [25] This risk of 2 family members having KD is greatest in twins, for whom the rate is approximately 13%. [26]

In 1978, Kato et al discovered that patients with KD are more likely to express HLA-Bw22J2, which is a major histocompatibility complex antigen seen predominantly in Japanese populations. This further implicated a genetic influence to the increased susceptibility to KD in Japanese patients. [27, 28] A genome-wide linkage analysis of affected sibling pairs was performed in Japan, and a multipoint linkage analysis identified evidence of linkage on chromosome 12q24. [29]

Dergun et al, Newburger et al, and Burns et al described families with multiple members affected with Kawasaki disease. [30, 31, 32] In these families, KD occurred in 2 generations or in multiple siblings. No clear pattern of inheritance could be deduced from these pedigrees. Therefore, multiple polymorphic alleles likely influence KD susceptibility.

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