Kawasaki Disease

Centers for Disease Control (CDC)

There have been reports of multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 (COVID-19), which may present with Kawasaki disease-like features.

Kawasaki disease (KD), also known as Kawasaki syndrome, is an acute febrile illness of unknown etiology that primarily affects children younger than 5 years of age. The disease was first described in Japan by Tomisaku Kawasaki in 1967, and the first cases outside of Japan were reported in Hawaii in 1976.

Clinical signs include fever, rash, swelling of the hands and feet, irritation and redness of the whites of the eyes, swollen lymph glands in the neck, and irritation and inflammation of the mouth, lips, and throat.

KD is a leading cause of acquired heart disease in the United States. Serious complications include coronary artery dilatations and aneurysms. The standard treatment, intravenous immunoglobulin and aspirin, substantially decreases the development of these coronary artery abnormalities.

KD occurs worldwide, with the highest incidence in Japan, and it most often affects boys and younger children. KD may have a winter-spring seasonality, and community-wide outbreaks have been reported occasionally.

In the continental United States, population-based and hospitalization studies estimate an incidence of KD ranging from about 9 to 20 per 100,000 children under 5 years of age. In the year 2016, approximately 5440 hospitalizations with KD were reported among children under 18 years of age in the US; 3935 of these children were under 5 years of age, for a hospitalization rate of 19.8 per 100,000 children in that age group.

Case Definition

For epidemiologic surveillance, CDC defines a case of KD as illness in a patient with fever of 5 or more days duration (or fever until the date of administration of intravenous immunoglobulin if it is given before the fifth day of fever), and the presence of at least 4 of the following 5 clinical signs:

Rash
Cervical lymphadenopathy (at least 1.5 cm in diameter)
Bilateral conjunctival injection
Oral mucosal changes
Peripheral extremity changes

Patients whose illness does not meet the above KD case definition but who have fever and coronary artery abnormalities are classified as having atypical or incomplete KD.