The UK Kawasaki Disease Foundation

Kawasaki Disease is the leading cause of acquired heart disease in children in the UK. It's time we changed that....

...together we will

2013 UK Management of  Kawasaki Disease; D Eleftheriou, M Levin, D Shingadia, 
R Tulloh, NJ Klein, PA Brogan

Date: October 2013
Correspondence to: Dr D Eleftheriou, Institute of Child Health, London, Open Access: Published Online

This paper sets out the UK recommended guidance for patient management and clinical care for acute Kawasaki Disease and indicates long term care needs, together with summarising recent advances in the understanding of KD pathogenesis and therapeutics.  

To access the resource: click here

​​​​1. Kawasaki Disease is the leading cause of acquired heart disease in children in the U.K.   ​

2. Kawasaki Disease is the second most common paediatric vasculitis

3. Early diagnosis and treatment is critical and can improve outcomes for children with Kawasaki Disease - there is a direct link between treatment times and outcomes, in particular heart damage

4. A child with Kawasaki Disease will present with a persistent high fever and a combination of TWO OR MORE other symptoms including rash, bloodshot eyes, swollen fingers and toes, swollen glands and cracked lips/'strawberry' tongue.  If some of these symptoms exist with a high fever, please THINK Kawasaki Disease and treat early

5. Increasingly common, Kawasaki Disease cases are doubling every ten years across the globe and U.K. - and hospital admissions increased fourfold in the last decade - doctors should be ready to see it / ready to treat

6. Long term care is essential for ALL patients with CAA, whether these resolve or do not. UK Management guidelines include recommendations (see below). 

7. The cause of Kawasaki Disease is unknown - much international research is ongoing but 50 years since its identification in Japan its cause is still uncertain​​

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

​Date: March 2017

Background: Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries.

Methods and Results: To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and long-term outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up.

Conclusions: These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances.

Refer to the guidelines here: click here 

Kawasaki Disease – Pearls and Pitfalls

An article on the diagnosis and treatment of Kawasaki Disease

Published on 17 January 2017

Once seen, never forgotten – but still a diagnosis that can be easily missed. Kawasaki disease is reasonably uncommon, and that ‘textbook’ case of a miserable child, five days febrile and bright red all over, ticking all the Kawasaki boxes, is even more uncommon. Here is a short guide to the ins and outs of Kawasaki disease, including some common pitfalls to avoid when diagnosing and managing this potentially devastating condition. Refer to the article here: click here

We're working to share knowledge and best practice, evidence based information.

Why?  Because Societi research and our conversations with clinicians across the UK have revealed sometimes inconsistent management approaches to both acute Kawasaki Disease and long term management  / clinical care arrangements. 

​Information to Share?

Included below are documents and links with current management recommendations for UK Kawasaki Disease clinical care.  If you'd like us to include information from your organisation on our website, links to relevant papers or articles, please contact us.

​​​​Resources for Clinicians - Kawasaki Disease Management 

​​​​Clinical Resources - Kawasaki Disease Management

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​​​​Kawasaki Disease - Key Facts

​​​​​​​NHS Improvement Service – Patient Safety Alert – Stage One Warning

Correspondence to: NHS Improvement Service

Publication date: May 2016

Requires action by all providers of NHS funded care to assess and act on risks re failure to recognise acute coronary syndromes in Kawasaki Disease patients.

​​Acute Cardiovascular Management of Patients with previous Kawasaki Disease – NHS London Guidance
Correspondence to: Dr M Levin, Imperial College London

Published: by circulation September 2015

Those who have a past history of Kawasaki Disease (KD) are at life-long risk of future cardiovascular events, particularly acute coronary syndromes. This guidance identifies risk factors, introduces the requirement for a person-specific protocol, and notes the need to ensure life-long follow up for those patients with coronary artery injury by a cardiology team with specialist interest in Kawasaki Disease. To download the Guidelines click above.