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Kawasaki’s disease 

Kawasaki’s disease

Chapter:
Kawasaki’s disease
Author(s):

Brian W. McCrindle

DOI:
10.1093/med/9780199204854.003.191108_update_001

November 28, 2012: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.

Update:

Pathogenesis—expanded discussion to include specific evidence regarding genetic susceptibility.

Clinical features—new illustration of all of the typical physical features of the diagnostic criteria.

Risk stratification—inclusion of stress echocardiography, MR angiography, and CT angiography. Classification of coronary artery abnormalities updated to emphasize the use of z-scores and identified cutpoints.

Management—(1)General—encouragement of physical activity within the context of any restrictions. (2) Immunosuppressive—enhanced discussion of evidence regarding the role of corticosteroids, also of both risk prediction and alternative treatments for patients not responding to initial IVIG treatment. (3) Long term—discussion a role for intravascular ultrasound to characterize arterial wall structure, particularly in adults suspected of coronary artery complications related to Kawasaki’s disease.

Updated on 30 Nov 2011. The previous version of this content can be found here.
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date: 28 March 2017

Kawasaki’s disease is an acute, self-limited, inflammatory vasculitis of unknown aetiology, with a peak incidence under 5 years of age.

Clinical features—the diagnosis is made in the presence of persistent fever for 5 days or more and at least four of the following five clinical signs: (1) nonpurulent conjunctivitis, (2) oropharyngeal inflammation, (3) cervical lymphadenopathy, (4) polymorphous exanthem, and (5) erythema of the palms and soles with subsequent desquamation. Incomplete presentations occur in approximately 25% of patients. The primary complications are cardiac, with coronary artery dilation and aneurysms evident in approximately 15 to 25% of untreated patients.

Management and prognosis—primary therapy consists of a single high dose of intravenous gammaglobulin, reducing the prevalence of coronary artery complications to approximately 4% if given within 10 days of the onset of fever. Persistent coronary artery lesions, with an ongoing risk of thrombosis and stenosis, are the predominant long-term morbidity. In adults, new presentations of myocardial ischemia with the associated finding of coronary artery aneurysms may suggest a previous episode of Kawasaki’s disease during childhood.

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