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Kawasaki disease 

Kawasaki disease
Chapter:
Kawasaki disease
DOI:
10.1093/med/9780198729228.003.0082
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date: 26 June 2019

Kawasaki disease is an acute self-limiting inflammatory disorder, associated with vasculitis affecting predominantly medium-sized arteries, particularly the coronary arteries. In developed countries, Kawasaki disease is the commonest cause of acquired heart disease in childhood. The aetiology of Kawasaki disease remains unknown, and it is currently believed that one or more as yet unidentified infectious agents induce an intense inflammatory host response in genetically susceptible individuals. Genetic studies have identified several susceptibility genes for Kawasaki disease and its sequelae in different ethnic populations. Intravenous immunoglobulin (IVIG) and aspirin are effective therapeutically, but recent clinical trials and meta-analyses have demonstrated that the addition of corticosteroids to IVIG is beneficial for the prevention of coronary artery aneurysms in severe cases with the highest risk of IVIG resistance. Other proposed therapies, including anti-tumour necrosis factor alpha, could also have a role for IVIG-resistant Kawasaki disease. This chapter summarizes recent advances in the understanding of Kawasaki disease pathogenesis and therapeutics and provides an approach for managing Kawasaki disease patients in the light of these advances.

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