Kawasaki disease. Epidemiology, late prognosis, and therapy.

Kawasaki disease is an immunologically mediated diffuse vasculitis of childhood of unknown etiology. While most of the clinical features--including diffuse mucosal inflammation, indurative edema, rash, and lymphadenopathy--are self-limiting, coronary artery aneurysms and the possibility of thrombotic occlusion occurs in up to 20% of children. The epidemiologic and clinical features of this disease suggest an infectious etiology; however, a specific organism has not been consistently identified. An abnormal immune response to this as yet to be defined organism plays a critical role in the progression of this disease. The morbidity and mortality of this disease are related primarily to the potential cardiovascular complications. The natural history of the coronary artery aneurysms is that most lesions regress with time. Factors leading to a higher probability of regression include age less than 1 year, female sex, fusiform aneurysm, and maximum diameter less than 4 mm. Current recommendations for therapy include aspirin and IVIG. The range of dosages regimens for each medication are discussed in the text.