Kawasaki Disease

Kawasaki disease is an acute vasculitis of unknown cause that occurs predominantly in infants and young children and produces coronary artery aneurysms in approximately 15% to 25% of those affected. In the United States, Kawasaki disease is more commonly the cause of noncongenital heart disease in children than is acute rheumatic fever. Therapy for Kawasaki disease in the acute phase is aimed at reducing inflammation of the coronary artery wall and preventing coronary thrombosis; treatment with high-dose intravenous gamma globulin and aspirin has become the standard of care and reduces the risk of development of coronary artery aneurysms by three- to fivefold. Even when treated with high-dose intravenous immune globulin G (IVIG) regimens within the first 10 days of illness, however, approximately 5% of children with Kawasaki disease develop at least transient coronary artery dilation, and 1% develop giant aneurysms. For those with persistent or recrudescent fever despite initial IVIG infusion, multiple courses of gamma globulin and treatment with cortico- steroids may be indicated. Early experience suggests that therapies aimed at reducing the amount of tumor necrosis factor alpha have a role. For those who develop coronary artery aneurysms, chronic antithrombotic regimens are instituted. When aneurysms are small or moderate in size, aspirin alone may be sufficient, but for patients with giant aneurysms, most experts choose to treat Kawasaki disease with aspirin plus warfarin. Acute coronary thromboses are treated with platelet IIb/IIIa antagonists and thrombolytic therapy. For children with coronary artery stenoses and consequent ischemic heart disease, the therapeutic armamentarium is similar to that used in adults with atherosclerotic coronary artery disease and includes coronary artery bypass grafting and transcatheter interventions.