What is Kawasaki disease?

Updated: Jul 29, 2018
  • Author: Tina K Sosa, MD; Chief Editor: Russell W Steele, MD  more...
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Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome and Kawasaki syndrome, is an acute febrile illness of early childhood characterized by vasculitis of the medium-sized arteries. Given its predilection for the coronary arteries, there is a potential for the development of coronary artery aneurysms (CAAs) and thus sudden death. CAAs develop in approximately 25% of untreated cases; appropriate treatment decreases this risk to 3-5%. [1]  Echocardiography is the study of choice to evaluate for CAAs. KD is the leading cause of acquired heart disease in developed nations. [2]

The incidence of KD in the continental United States is approximately 25/100,000 children under 5 years of age; in Japan, the incidence has been estimated at approximately 250/100,000 children < 5 years of age. [1]

The etiology of this disorder remains unknown.

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