Diagnosis and management of Kawasaki disease in children

Kawasaki disease is the most common cause of acquired heart disease in children in the UK. It is an acute self-limiting systemic vasculitis, with a predilection for the coronary arteries. Coronary arteritis, thrombotic occlusion of coronary vessels, coronary artery aneurysms and even sudden death can occur with endocardial, myocardial or pericardial inflammation occurring during the acute stages of the illness or up to many years following initial presentation. The cause of Kawasaki disease remains unknown and there is no diagnostic test for it. Diagnosis is made on the basis of certain clinical criteria and non-specific laboratory findings. Prompt treatment with both high-dose aspirin and intravenous immunoglobulin is essential as it has been shown to reduce the incidence of cardiac complications from 25% to 4.7% in the UK. Abnormalities in myocardial blood flow can result in patients requiring long-term therapy in the form of drugs or, in some cases, invasive cardiac interventions.