Kawasaki disease

Kimon Bekelis MD (Dr. Bekelis of Dartmouth-Hitchcock Medical Center has no relevant financial relationships to disclose.)
Robert J Singer MD (Dr. Singer of Dartmouth-Hitchcock Medical Center/Geisel School of Medicine at Dartmouth has no relevant financial relationships to disclose.)
Nina Schor MD PhD, editor. (Dr. Schor of the University of Rochester Medical Center and Chair of the Department of Pediatrics at Golisano Children’s Hospital at Strong has no relevant financial relationships to disclose.)
Originally released April 17, 2006; last updated September 12, 2016; expires September 12, 2019

Overview

Kawasaki disease is one of the most common vasculitides in childhood. This inflammatory condition attacks the medium and small arteries with a propensity to damage the coronary arteries, thus, making it the most common cause of acquired heart disease in childhood. Treatment with intravenous immunoglobulin is effective at preventing damage to coronary arteries. In this article, the authors review the symptoms, signs, and pathophysiology of Kawasaki disease. The latest treatment recommendations are also reviewed.

Historical note and terminology

Kawasaki disease is a vasculitis of the medium and small arteries with a propensity to damage the coronary arteries. Dr. Tomisaku Kawasaki first reported 7 cases of “Non-scarlet fever desquamation syndrome” in 1962 followed by “20 cases of ocular-mucocutaneous syndrome” in 1964. His classic report was published in 1967 and included a description of 50 children with what he described as “pediatric acute febrile mucocutaneous lymph node syndrome with characteristic desquamation of the fingers and toes” (Kawasaki 1967). In this paper, Dr. Kawasaki described the cardinal characteristics that have since come to classify the illness. In 1975, Kato and colleagues published the first large case series describing the coronary artery abnormalities associated with Kawasaki disease (Kato et al 1975). The first published case series describing “mucocutaneous lymph node syndrome” in the United States was reported in 1976 (Melish et al 1976). The connection between Kawasaki disease and ensuing coronary artery aneurysm has proven significant. Kawasaki disease has replaced acute rheumatic fever as the most common cause of acquired heart disease in industrialized countries.

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