Kawasaki disease

Abstract

Kawasaki disease is an acute vasculitis of childhood, complicated by coronary artery aneurysms in up to 25% of affected children. High-dose intravenous immunoglobulin (IVIG) administered in the acute phase of Kawasaki disease leads to a fivefold reduction in the prevalence of coronary aneurysms. When fever persists, a second IVIG infusion should be administered. Rescue therapies for IVIG-resistant Kawasaki disease include pulsed-dose methylprednisolone or other steroid regimens, as well as infliximab, a tumor necrosis factor-alpha antagonist. Aspirin is used initially in an anti-inflammatory dosage, then at a low dosage until approximately 6 weeks after illness onset. Patients with coronary aneurysms require chronic antithrombotic therapy. For small aneurysms, aspirin alone is sufficient. For larger aneurysms, agents added to aspirin include clopidogrel and, for giant aneurysms, warfarin or low molecular weight heparin. Long-term management is tailored to the degree of coronary artery involvement. Evidence-based data to guide indications for transcatheter and surgical intervention are limited.