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Self-limited vasculitic syndrome of unknown etiology
characterized by fever, cervical adenopathies, and cardiac involvement.
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Mucocutaneous Lymph Node Syndrome.
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Asian people are at greater risk: 5000-6000 new cases per
year occur in Japan versus 3000 in the United States. Males are affected 1.5
times more often than females. Kawasaki disease (KD) is currently the leading
cause of acquired heart disease in the United States among children younger
than 5 years. Mortality from coronary artery abnormalities, including
coronary aneurysms, is reported in 20 to 25% of untreated individuals.
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Etiology of KD is unknown. Several
possibilities are considered, including an infectious etiology (retrovirus?), an
immunologic abnormality, and even a toxic cause (carpet shampoo). Clinical
and epidemiologic data support an infectious etiology, but many authors
think that an autoimmune component or genetic predisposition also exists.
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Necrotizing vasculitis of medium-size muscular
arteries, including coronary and cerebral vessels. Arteries show focal
segmental destruction, with subsequent ectasia and formation of aneurysms in
15 to 25% of children. Several infectious causes of KD have been
theorized and include Ebstein-Barr virus, retroviruses, Streptococcus pyogenes and viridans, Staphylococcus, Chlamydia, Propionibacterium, and Pseudomonas species. However, conventional bacterial and viral cultures and
serologic studies have not confirmed an infectious cause. Other postulated
etiologic agents are immunizations, medications, and environmental agents,
such as exposure to rug shampooing agents or house dust mites. The finding
of a skewed T-cell receptor response in the myocardium and the coronary arteries
led to a search for superantigens, such as toxic shock syndrome toxin-1
produced by Staphylococcus aureus. Immunohistochemical findings lend support to the hypothesis of
a proinflammatory cell-mediated immune reaction possibly triggered by a
superantigen or a conventional antigen.
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Clinical diagnosis. No diagnostic laboratory tests are
available. The Centers for Disease Control and Prevention in Atlanta (USA) defines a case
of KD as illness in a patient with fever of 5 or more days' duration (or fever until the
of administration of intravenous immunoglobulin, if this is given before the fifth day
of fever), and the presence of at least 4 of the following 5 clinical signs:
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Rash
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Cervical lymphadenopathy (at least one lymph node ≥ 1.5 cm in diameter)
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Bilateral conjunctival injection
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Oral mucosal changes (injected or fissured lips, injected pharynx, “strawberry” tongue)
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Peripheral extremity changes (erythema of palms or soles, edema of the hands or feet,
or generalized or periungual desquamation).
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Patients whose illness does not meet the above KD case definition but who have
fever and coronary artery ...