Article Text
Abstract
Kawasaki disease (KD) is an inflammatory disorder of young children, associated with vasculitis of the coronary arteries with subsequent aneurysm formation in up to one-third of untreated patients. Those who develop aneurysms are at life-long risk of coronary thrombosis or the development of stenotic lesions, which may lead to myocardial ischaemia, infarction or death. The incidence of KD is increasing worldwide, and in more economically developed countries, KD is now the most common cause of acquired heart disease in children. However, many clinicians in the UK are unaware of the disorder and its long-term cardiac complications, potentially leading to late diagnosis, delayed treatment and poorer outcomes. Increasing numbers of patients who suffered KD in childhood are transitioning to the care of adult services where there is significantly less awareness and experience of the condition than in paediatric services. The aim of this document is to provide guidance on the long-term management of patients who have vascular complications of KD and guidance on the emergency management of acute coronary complications. Guidance on the management of acute KD is published elsewhere.
- Kawasaki disease
- lifetime cardiovascular management
- coronary artery aneurysm
- late sequelae
- acute coronary syndrome
- cardiovascular risk
- person specific protocol
- transitional care
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Footnotes
Contributors All authors contributed to the design of the manuscript and attended multiple meetings over a 2-year period in order to achieve a consensus document. All authors have approved the final version of the manuscript. Members of the writing group are; Brogan PA, Burns JC, Cornish J, Diwakar V, Eleftheriou D, Gordon JB, Gray HH, Johnson T, Levin M, Malik I, MacCarthy P, McCormack R, Miller OI, Tulloh RMR.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests PB has received institutional grants from SOBI, Roche, Novartis and Novimmune and consultancy fees from SOBI, Novartis, Roche and UCB. RMRT has received grants and speaker fees from Actelion, Abbvie, GSK, Bayer, Pfizer, Jansen. Societi Foundation (RMcC) has received grants from SOBI and Roche.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.