Kawasaki Disease is an idiopathic multisystem disease characterized by vasculitis of small & medium blood vessels, including coronary arteries. It is a self-limited vasculitis of unknown etiology that predominantly affects children younger than 5 years and is now the most common cause of acquired heart disease in children in the United States and Japan.
80% of cases in children < 4 yrs, 5% of cases in children > 10 yrs. The etiology is most likely infectious and there has been some association with a novel coronavirus suggesting a viral etiology, however no definitive causative agent has been identified.
About 90% of patients will have fever, conjunctival erythema, and oral mucosal changes and 70% will have lymphadenopathy. Other common symptoms include strawberry tongue and lip fissures, anterior uveitis, perianal erythema, hepatic, renal, and GI dysfunction, myocarditis, pericarditis and lymphadenopathy.
In the acute phase, the most common blood laboratory findings include: a leukocytosis with left shift, mild anemia, an increased erythrocyte sedimentation rate or C-reactive protein, hypoalbuminemia, elevation of liver transaminases.
Imaging: Echocardiography is used to rule out coronary artery aneurysms and myocarditis, valvulitis, or pericardial effusion. Diffuse dilatation of coronary lumina can be observed in 50% of patients by the 10th day of illness. Echocardiography should be repeated in the second or third week of illness and 1 month after all other laboratory results have normalized.
Treatment includes a one-time dose of IVIG and high-dose aspirin. Although it has a good prognosis with treatment, Kawasaki disease can lead to death from coronary artery aneurysm in a small percentage of patients.
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