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AT-A-GLANCE

AT-A-GLANCE

  • Kawasaki disease (KD) is a multisystem inflammatory process of unknown but suspected infectious etiology.

  • Highest incidence is in Asian children; 1 in 80 Japanese children develops KD by age 5 years.

  • KD is the most common cause of acquired heart disease in children in developed nations.

  • KD affects all blood vessels in the body, but primarily damages medium-sized muscular arteries such as the coronary arteries.

  • Major symptoms are prolonged high fever, conjunctival injection, oral mucosal changes such as red lips and pharynx and strawberry tongue, redness and swelling of the hands and feet, erythematous polymorphic rash, and cervical lymphadenopathy.

  • Inflammation in the coronary arteries can lead to aneurysms with subsequent myocardial infarction, aneurysm rupture, and sudden death.

  • Treatment with intravenous immunoglobulin (IVIG) and aspirin, when given in the first 10 days of fever, reduces the prevalence of coronary artery abnormalities from 25% in those treated with aspirin alone, to 5% in those who receive IVIG with aspirin.

  • Long-term complications are confined to the heart and vascular tree, primarily thrombosis and stenosis of the major coronary arteries with myocardial ischemia.

Kawasaki disease (KD), the leading cause of acquired heart disease in children in developed nations, is a multisystem inflammatory illness that particularly affects blood vessels, especially the coronary arteries. Approximately 25% of untreated children develop coronary artery abnormalities, including dilation and aneurysms that can lead to myocardial infarction and sudden death.1,2 The etiology is unknown, but clinical and epidemiologic data support an infectious cause. In KD, an intense inflammatory cell response develops in a wide array of organs and tissues3; in medium-sized arteries such as the coronary arteries, this response can damage collagen and elastin fibers in the vessel walls and lead to loss of their normal structural integrity with resultant ballooning or aneurysm formation. Despite a limited understanding of KD pathogenesis, a very effective therapy exists in the form of intravenous immunoglobulin (IVIG) with aspirin; when given in the first 10 days of fever, this therapy reduces the prevalence of coronary artery abnormalities from 25% in untreated patients to 5% in those who receive the therapy.4 Because the etiology is unknown, no diagnostic test exists, and the diagnosis is made clinically. Classic KD is diagnosed in a patient with prolonged fever and 4 of 5 other clinical features. However, incomplete forms of illness, in which a child manifests prolonged fever with fewer than 4 other clinical features of the illness and subsequently develops coronary artery abnormalities, are well-recognized. The existence of these incomplete forms of illness results in a major diagnostic dilemma for physicians in establishing the diagnosis accurately in children with prolonged fever of uncertain cause.

HISTORICAL PERSPECTIVE

KD is named for Dr. Tomisaku Kawasaki, a Japanese pediatrician who first recognized the clinical features of the illness. He described 50 cases of a new illness in 1967 in the Japanese-language literature that he termed ...

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