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Kawasaki Disease at a Glance
  • Most common cause of acquired heart disease in children in developed nations.
  • Highest incidence in Asian children; 1 in 100 Japanese children develops Kawasaki disease (KD) by age 5 years.
  • KD is a multisystem inflammatory process of unknown but suspected infectious etiology.
  • KD affects all blood vessels in the body, particularly medium-sized 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. About 25% of untreated children develop coronary artery abnormalities, including dilatation 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 IVIG and 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 four of five other clinical findings (Box 167-1). However, incomplete forms of illness are well-recognized, in which a child manifests prolonged fever with fewer than four other clinical features of the illness and subsequently develops coronary artery abnormalities. 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.

Box 167-1 Diagnostic Criteria for Classic Kawasaki Disease

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