An update on Kawasaki disease.

Kawasaki disease (KD) is a febrile systemic vasculitis complicated by coronary and peripheral arterial aneurysms in 20-35% of untreated patients. It is reported as the commonest cause of acquired heart disease in children in developed countries, and may be a risk for adult ischaemic heart disease. Although KD has been reported all over the world, it is overexpressed among Asian populations, especially Japanese. The disease pathogenesis is still unknown and several theories have been proposed, including the possibility of an infection by a toxin-secreting microorganism and of a superantigen-driven process. Despite numerous efforts there is still no diagnostic test available for KD, and the diagnosis is based on clinical criteria after the exclusion of other diseases presenting with high persistent fever. Prompt diagnosis is critical, since the early administration of intravenous immunoglobulins and aspirin reduces the rate of coronary abnormalities to less than 5% of patients.