Kawasaki disease

PURPOSE OF REVIEW: Kawasaki disease is an acute, self-limited vasculitis of childhood characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in approximately 15 to 25% of untreated children with the disease and may lead to myocardial infarction, sudden death, or ischemic heart disease. RECENT FINDINGS: In the United States, Kawasaki disease has now surpassed acute rheumatic fever as the leading cause of acquired heart disease in children. The cause of Kawasaki disease remains unknown, but fortunately intravenous immune globulin therapy has proved to be effective at reducing the prevalence of coronary aneurysms in most children treated in the acute phase. Therapy for Kawasaki disease resistant to intravenous immune globulin therapy is an area of research and controversy. The long-term treatment of children with Kawasaki disease is dependent on coronary artery status. SUMMARY: This review covers key data on the etiology, pathogenesis, treatment, and long-term outcomes of Kawasaki disease, highlighting recent publications.