Kawasaki Disease

Kawasaki disease (KD) is an acute systemic vasculitis syndrome in infants and young children of unknown etiology, which mainly affects small and medium-sized arteries, particularly the coronary arteries. First described in Japan in 1967, Kawasaki disease has now been described worldwide and in all racial groups. It is the leading cause of acquired heart disease in children in the United States. Clinical features include an acute febrile illness associated with mucocutaneous involvement and unilateral cervical lymphadenopathy. Without early treatment, coronary aneurysms develop in approximately 20 % of patients. Treatment with high-dose intravenous immunoglobulin (IVIG) and aspirin in acute KD reduces the risk of coronary aneurysms to less than 5 %. Patients with coronary aneurysms may develop coronary thrombosis in the acute phase, and, over time, progressive coronary artery obstruction, with angina, myocardial infarction, or sudden death. Bypass surgery and percutaneous coronary intervention are used to treat myocardial ischemia secondary to coronary artery aneurysms and stenosis. In the U.S., approximately 5 % of young adults presenting with acute coronary syndromes had Kawasaki disease in childhood.