Open Access

ARTICLE

Kawasaki disease: State of the art

by

Department of Cardiology, Boston Children’s Hospital, Department of Pediatrics, Harvard Medical School, Boston, Massachusetts

* Corresponding Author: Jane W. Newburger, MD, MPH, Department of Cardiology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02468. Email:

Congenital Heart Disease 2017, 12(5), 633-635. https://doi.org/10.1111/chd.12498

Abstract

Kawasaki disease is an acute febrile arteritis of childhood that can result in coronary artery aneurysms if untreated in the first 10 and ideally 7 days of illness. Kawasaki disease begins as a necrotizing arteritis with neutrophilic infiltrate, followed by subacute/chronic changes and luminal myofibroblastic proliferation that can cause coronary artery stenosis. Manifestations include the presence of ≥5 days of fever, together with clinical criteria of extremity changes, rash, conjunctivitis, oral changes, and unilateral cervical lymphadenopathy. Echocardiography should be performed at the time of diagnosis, then 1–2 weeks and 4–6 weeks later, with more frequent studies in individuals with coronary artery dilation or persistent fever. Coronary artery dimensions are characterized both as z-scores and absolute measurements, and coronary architecture evolves over time in children who have aneurysms in the first weeks of illness. Systematic follow-up and therapies are tailored to the degree of coronary disease and to coronary ischemia.

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