Clinical and epidemiologic aspects of kawasaki disease

Abstract

Summary: Kawasaki disease (KD), first observed in Japan in 1967, is now distributed worldwide and occurs on all continents and in all ethnic groups. Almost exclusively, the syndrome affects children below the age of 8 years and is characterized by persistent high fever, conjunctival injection, mucosal changes to the oropharynx, changes in peripheral extremities, erythematous rash, and cervical lymphadenopathy. Despite the self-limited nature of KD, approximately 20% of patients manifest coronary artery aneurysms on echocardiogram. The development of giant coronary artery aneurysms larger than 8 mm leads to the greatest incidence of morbidity and mortality because of the association of such aneurysms with thrombotic occlusion or stenotic obstruction and subsequent myocardial infarction. Although the etiology of KD remains unknown, clinical features and epidemiologic patterns suggest the involvement of an infectious microbe with efficient transmission. Further research on suspicious agents, including various bacterial and viral species, has failed to establish a direct association between any organism and KD. Nevertheless, in the absence of a clearly defined etiology an effective therapy for KD has evolved. Trials conducted first in Japan and then in the United States have shown that treatment with intravenous immune globulin (IVIG), given at a dosage of 400 mg/kg/day for 4 consecutive days or in a single dosage of 2 g/kg, is associated with rapid resolution of the fever and other inflammatory manifestations of KD, in addition to the reduction in the frequency and severity of coronary artery abnormalities. The mechanism of action for IVIG in KD is not known.