Practice Essentials Kawasaki disease (KD) is an acute febrile illness of early childhood characterized by vasculitis of the medium-sized arteries. Given its predilection for the coronary arteries, there is a potential for the development of coronary artery aneurysms (CAAs) and thus sudden death. CAAs develop in approximately 25% of untreated cases; appropriate treatment decreases this risk to 3-5%. KD is the leading cause of acquired heart disease in developed nations. The incidence of KD in the continental United States is approximately 25/100,000 children under 5 years of age; in Japan, the incidence has been estimated at approximately 250/100,000 children < 5 years of age. The etiology of this disorder remains unknown. Diagnosis There are two forms of KD: complete and incomplete. Diagnosis of complete KD requires fever of at least 5 days' duration along with 4 or 5 of the principal clinical features. The principal clinical features are as follows: · Extremity changes · Polymorphous rash · Oropharyngeal changes · Bilateral, nonexudative, limbic sparing, painless bulbar conjunctival injection · Acute unilateral nonpurulent cervical lymphadenopathy with lymph node diameter greater than 1.5 cm The acronym "FEBRILE" is used to remember the criteria as follows: · Fever · Enanthem (mucous membrane rash) · Bulbar conjunctivitis · Rash · Internal organ involvement (not part of the criteria) · Lymphadenopathy · Extremity changes Incomplete KD is diagnosed when a patient presents with fever for 5 days or longer, 2 or 3 of the principal clinical features, and laboratory findings suggestive of the disease or echocardiographic abnormalities. Suggestive laboratory findings include elevated erythrocyte sedimentation rate (ESR), elevated C-reactive protein (CRP), hypoalbuminemia, anemia, elevated alanine aminotransferase (ALT), thrombocytosis, leukocytosis, and pyuria. The American Heart Association (AHA) suggests an algorithm for the diagnosis of incomplete KD in the most recent guideline. Echocardiography is the study of choice to evaluate for CAAs. Serial echocardiograms should be obtained as follows: · At the time of KD diagnosis · 1-2 weeks after the onset of the illness · 5-6 weeks after the onset of the illness Management The principal goal of treatment is to prevent coronary artery disease. Intravenous immunoglobulin (IVIG) and aspirin are the mainstays of treatment. Patients should be treated with IVIG within 10 days after the onset of fever to prevent the development of cardiac disease. Other medications that are used variably as adjunctive treatments or for IVIG-resistant KD include corticosteroids, infliximab, cyclophosphamide, methotrexate, and ulinastatin. In addition to aspirin, other anticoagulants are sometimes utilized, including clopidogrel, dipyridamole, warfarin, and heparin. Source
