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Kawasaki disease: Clinical features and diagnosis

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

INTRODUCTION

Kawasaki disease (KD, previously called mucocutaneous lymph node syndrome) is one of the most common vasculitides of childhood [1]. KD also occurs rarely in adults. It is typically a self-limited condition, with fever and manifestations of acute inflammation lasting for an average of 12 days without therapy [2]. However, complications such as coronary artery (CA) aneurysms, depressed myocardial contractility and heart failure, myocardial infarction, arrhythmias, and peripheral arterial occlusion may develop and lead to significant morbidity and mortality. (See "Cardiovascular sequelae of Kawasaki disease".)

The clinical manifestations and diagnosis of KD are discussed in this review. The epidemiology, etiology, treatment, and complications of KD, including cardiac sequelae, are presented separately. Incomplete (atypical) KD and unique features in infants and adults are also reviewed separately. (See "Kawasaki disease: Epidemiology and etiology" and "Kawasaki disease: Initial treatment and prognosis" and "Cardiovascular sequelae of Kawasaki disease" and "Incomplete (atypical) Kawasaki disease" and "Kawasaki disease: Complications".)

CLINICAL MANIFESTATIONS

The clinical features of KD reflect widespread inflammation of primarily medium-sized muscular arteries. Diagnosis is based upon evidence of systemic inflammation (eg, fever) in association with signs of mucocutaneous inflammation. The characteristic bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, rash, extremity changes, and cervical lymphadenopathy typically develop after a brief nonspecific prodrome of respiratory or gastrointestinal symptoms [3-8] (see 'Other findings' below). These characteristic clinical signs are the basis for the diagnostic criteria for KD (table 1) [9].

Oral mucous membrane findings are seen in approximately 90 percent of cases of KD, polymorphous rash in 70 to 90 percent, extremity changes in 50 to 85 percent, ocular changes in >75 percent, and cervical lymphadenopathy in 25 to 70 percent [7,10-12].

These findings are often not present at the same time, and there is no typical order of appearance. As an example, some patients have only developed fever and cervical lymphadenopathy by the time of admission (so-called KD with isolated cervical lymphadenopathy, KDiL) [13]. In one case series, these patients tended to be older and to have a more severe course, with increased risk of coronary artery (CA) disease and lack of response to intravenous immune globulin (IVIG). Thus, repeated histories and physical examinations are important both for making a timely diagnosis of KD in children who fail to meet diagnostic criteria, as well as for appropriate consideration of alternative diagnoses. (See 'Diagnosis' below.)

                

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Literature review current through: Nov 2016. | This topic last updated: Mon Feb 22 00:00:00 GMT 2016.
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