Treatment of Kawasaki disease

The epidemiology, etiology, diagnosis, and treatment of Kawasaki disease are reviewed. Kawasaki disease, or mucocutaneous lymph node syndrome, is an acute, usually self-limiting, multiple-organ-system disease of childhood that occurs both epidemically and endemically worldwide. The etiology of the disease is unknown but may involve an infectious agent. To be diagnosed, a patient must be febrile for at least five days and show four of five additional clinical features: bilateral conjunctivitis, changes in the oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. The most important complications are cardiac; patients may develop aneurysms or thrombosis of the coronary arteries or myocarditis. Other complications include arthritis, conjunctivitis, and hydrops of the gallbladder. Aspirin, intravenous immune globulin, corticosteroids, and antithrombotic agents have been investigated for use in the treatment of Kawasaki disease with varying results. Current recommendations suggest therapy with aspirin 80-100 mg/kg/day every six hours for the first 14 days after diagnosis and intravenous immune globulin 400 mg/kg/day for the first four days. The dose of aspirin should then be reduced and continued for six to eight weeks if no coronary artery abnormalities are present. Treatment guidelines for Kawasaki disease are being refined. Current evidence supports early use of aspirin and intravenous immune globulin to prevent cardiac complications.