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Kawasaki disease: Epidemiology and etiology

Robert Sundel, MD
Section Editors
Marisa Klein-Gitelman, MD, MPH
Sheldon L Kaplan, MD
Deputy Editor
Elizabeth TePas, MD, MS


Kawasaki disease (KD, previously called mucocutaneous lymph node syndrome) is one of the most common vasculitides of childhood, particularly in East Asia. It is typically a self-limited condition, with fever and other acute inflammatory manifestations lasting for an average of 12 days if not treated. The underlying etiology is unknown.

KD can cause a variety of cardiovascular complications, including coronary artery aneurysms, cardiomyopathy with depressed myocardial contractility and heart failure, myocardial infarction, arrhythmias, and peripheral arterial occlusion. These complications may cause significant morbidity and mortality, particularly in children who are inadequately treated. The frequency of aneurysm development and mortality has dramatically decreased as a result of intravenous immune globulin therapy. Early diagnosis is critical to achieve the optimal treatment result.

The epidemiology and etiology of KD are reviewed here. The clinical features, diagnosis, treatment, and cardiac sequelae are presented separately. Incomplete KD is also discussed separately. (See "Kawasaki disease: Clinical features and diagnosis" and "Kawasaki disease: Initial treatment and prognosis" and "Cardiovascular sequelae of Kawasaki disease: Clinical features and evaluation" and "Incomplete (atypical) Kawasaki disease".)


KD is one of the most common vasculitides of childhood [1].

Geographic variation — The incidence of KD is greatest in children who live in East Asia or are of Asian ancestry living in other parts of the world [2-5]. The incidence in underdeveloped countries is largely unknown and ascertainment may be incomplete. KD is particularly difficult to diagnose in areas where measles is still prevalent since the presentation is similar [6,7]. Many nations around the world have demonstrated an increase in the number of children diagnosed with KD since the early to mid 2000s. It is not clear, however, whether this represents an actual increase in the incidence of the disease, increased awareness of the condition, or a greater tendency to classify children with incomplete clinical features as having KD.

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Literature review current through: Nov 2017. | This topic last updated: May 05, 2017.
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