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Kawasaki disease is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in infants and young children. First described in Japan in l967 by Tomisaku Kawasaki, the disease is now known to occur in both endemic and community-wide epidemic forms in the Americas, Europe, and Asia in children of all races. Kawasaki disease is 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 15% to 25% of untreated children with the disease and may lead to myocardial infarction (MI), sudden death, or ischemic heart disease. In the United States, Kawasaki  disease has surpassed acute rheumatic fever as the leading cause of acquired heart disease in children.4 Treatment of Kawasaki disease in the acute phase is directed at reducing inflammation in the coronary artery wall and preventing coronary thrombosis, whereas long-term therapy in individuals who develop coronary aneurysms is aimed at preventing myocardial ischemia or infarction.

A new feature of these recommendations is an algorithm for the evaluation and treatment of patients in whom incomplete or atypical Kawasaki disease is suspected. We attempt to summarize the current state of knowledge of the management of patients with Kawasaki disease. The recommendations are evidence based and derived from published data wherever possible. The levels of evidence on which recommendations are based are classified as follows: level A (highest), multiple randomized clinical trials; level B (intermediate), limited number of randomized trials, nonrandomized studies, and observational registries; and level C (lowest), primarily expert consensus.