Familial occurrence of Kawasaki syndrome in North America

To describe families with multiple members affected with Kawasaki syndrome (KS) to increase awareness of the familial occurrence of KS among practitioners who care for these patients.

Retrospective review of medical records at 2 medical centers and data collection from remote KS families who contacted the KS Research Program at the University of California, San Diego.

Eighteen families with multiple affected members were identified. There were 9 families with 2 affected siblings. In San Diego, 3 (0.7%) of 424 KS families had sibling cases. Nine families were identified with KS in 2 generations or in multiple affected members, yielding a total of 24 KS-affected children. No clear pattern of inheritance could be deduced from these pedigrees, and it is likely that multiple polymorphic alleles influence KS susceptibility.

Physicians should counsel affected families and make them aware of the potential increased risk of KS among family members.