Kawasaki Disease and the Importance of Family History

Kawasaki disease (KD), a coronary artery vasculitis, is rare in teenagers who nonetheless have an increased risk of coronary artery aneurysms.

A 19-year-old woman had a prolonged febrile illness that resolved without treatment. The diagnosis of KD was made at autopsy at 42 years of age after sudden cardiac death associated with calcified giant coronary artery aneurysm. An interview of the decedent's mother revealed a KD-compatible illness in the decedent's son at 5 years of age that also was not diagnosed.

Susceptibility to KD is influenced by host genetics, and first-degree relatives of an index case have a 10-fold increased relative risk of disease. Pathognomonic changes in the coronary arteries discovered at autopsy should prompt counseling of family members.

KD should be considered in adolescents and young adults with prolonged unexplained fever and mucocutaneous findings, including rash, conjunctival injection, oral changes, and extremity edema and erythema, and transthoracic or transesophageal echocardiography should be performed to evaluate the coronary arteries. Clinicians evaluating a patient with suspected KD should obtain a history regarding family members with antecedent, unexplained, and prolonged febrile illness that might have been undiagnosed KD.