Kawasaki Disease: Current Therapeutic Perspectives

Kawasaki disease is an acute systemic vasculitis of unknown etiology that has become the most common form of acquired heart disease in young children in developing countries. The most serious threat from Kawasaki disease is the development of coronary vasculitis, with subsequent development of aneurysms, thrombosis, and coronary compromise. Standard treatment during the acute phase of Kawasaki disease now consists of intravenous gamma globulin, 2 g/kg, given as a single dose, and high-dose aspirin therapy, 80 to 100 mg/kg daily. When instituted within 10 days of the onset of illness, this approach has reduced the incidence of coronary artery abnormalities from 20% to 25% to approximately 5% at 6 to 8 weeks after initiation of treatment. Despite these therapeutic successes, the optimal management of certain patient groups with Kawasaki disease remains unclear or controversial. This includes patients with persistent or recrudescent fever and inflammation despite prompt initiation of standard therapy, and patients developing coronary arterial aneurysms. For patients with persistent or recrudescent fever, there are increasing reports suggesting that corticosteroid therapy, in addition to retreatment with intravenous gamma globulin, may be useful. Newer antiplatelet agents are being introduced that may improve outcome in children who develop acute coronary artery lesions. Long-term therapy of patients with coronary artery aneurysms typically consists of long-term aspirin therapy; the use of additional antiplatelet agents and anticoagulants is often used in clinical practice, but not universally accepted.