Genetics

A genetic culprit has not been identified in Kawasaki disease, although the evidence suggests a contributing genetic component is highly likely.  Kawasaki disease does not appear to follow Mendelian inheritance but familial aggregation is well recognised¹.

• There is significant geographical variability with the highest case numbers reported in Japan and South Korea (Table 1).
• Relative risk is 10 times higher if an older sibling has been affected.
• Concordance risk in identical twins is approximately 13%.
• There is increased incidence in children who have a positive family history.

Table 1. Incidence of Kawasaki disease in various countries¹

Interestingly, several single-nucleotide polymorphisms in different genes implicated in Kawasaki disease are also associated with other inflammatory conditions (such as rheumatoid arthritis, ulcerative colitis and systemic lupus erythematosus) which may indicate a common pathway in the inflammatory immune response⁵.