​​For Clinicians

The UK Kawasaki Disease Foundation

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 information leaflet aimed at GPs, pharmacists and primary care practitioners

Date: February 2017

​NIHR Signal Corticosteroids given early reduce risk of heart problems in children with Kawasaki disease
Published on 14 February 2017

Early treatment with corticosteroids on top of standard therapy reduces the risk of serious heart problems in children under five with the rare vascular disease, Kawasaki disease.

​Refer to the abstract 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

Coronary Artery Complication in Kawasaki Disease and the Importance of Early Intervention : A Systematic Review and Meta-analysis. 
Chen S, et al. JAMA Pediatr.
Correspondence to: Chen S; Department of Cardiology, Shanghai First People's Hospital, Shanghai Jiao Tong University School of Medicine

Date: 1 December 2016

Importance: The timing and selection of patients with Kawasaki disease for corticosteroid use to prevent coronary artery complications remain controversial.
Objective: To evaluate the effect of corticosteroid therapy in KD.
Refer to the abstract here: 

Early corticosteroid therapy associated with better outcomes in Kawasaki Disease

Anees Daud, MD and James England, MD

Correspondence to: Anees Daud, University of Utah Medical School

Date: 19 October, 2016

The presented study is a meta-analysis of trials that compared adjuvant corticosteroid therapy in addition to IVIG/ASA to conventional therapy alone. Trials that had steroids as part of initial therapy and those that used steroids as rescue therapy for IVIG-resistant disease were included.

Refer to the study here: http://www.2minutemedicine.com/early-corticosteroid-therapy-associated-with-better-outcomes-in-kawasaki-disease

Coronary Artery Complication in Kawasaki Disease and the Importance of Early Intervention

Shaojie Chen, MD, MM, PhD1,2; Ying Dong, MD, MM, PhD3; Marcio Galindo Kiuchi, MD, MS, PhD4; et al

Correspondence to:

Date: December 2016

This systematic review and meta-analysis demonstrates that adjunctive corticosteroid therapy was associated with a significantly lower rate of coronary artery complication compared with intravenous immunoglobulin therapy, particularly among high-risk patients. Meta-regression based on known variables demonstrated that the overall efficacy was negatively correlated with the duration of illness before corticosteroids therapy.

Refer to the study here:http://jamanetwork.com/journals/jamapediatrics/article-abstract/2565679

Breastfeeding and Risk of Kawasaki Disease: A Nationwide Longitudinal Survey in Japan

Yorifuji T, Tsukahara H, Doi H

Correspondence to: Associate Prof Dr. Takashi Yorifuji Okayama University

Date: June 2016

Kawasaki disease (KD) is the most common cause of childhood-acquired heart disease in developed countries. However, the etiology of KD is not known. Aberrant immune responses are considered to play key roles in disease initiation and breastfeeding can mature immune system in infants. We thus examined the association between breastfeeding and the development of KD.

​Refer to the study here: 


NHS Improvement Service – Patient Safety Alert – Stage One Warning Correspondence to: NHS Improvement Service (patientsafety.enquiries@nhs.net)
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.

Societi Summer Newsletter
Date: August 2016

For an update on our work over the period December 2015, when we began, to June 2016 - together with plans for the next six months or so, please download a copy of our newsletter here! 

Issues and Needs - A Societi Infographic for Kawasaki Disease
Date: May 2016

We have prepared a clear summary statement, helped by the inclusion of 'infographics' to set out clearly what the current position with regard to Kawasaki Disease is in the UK - and what needs to be done.  This can be used as an awareness raising poster/teaching aid or discussion point.

BPSU Surveillance of All Kawasaki Disease, UK & Ireland 2013-2015
Correspondence to: Dr R Tulloh, Bristol Children's Hospital

Publication date: Abstracts / papers presented April 2016

Funded by KSSG UK, this research survey covered all children in the UK and Ireland who had a new diagnosis of KD between February 2013 and February 2015. Kawasaki disease (KD) is the commonest acquired heart disease in the western world. The incidence is rising in all countries, doubling every 20 years. The last BPSU survey was 25 years ago and we wished to update the data and find if there was a link between phenotype, ethnicity, location, and outcome. More information about the study can be found here:
www.rcpch.ac.uk/bpsu/kd  For the abstract and link to paper go to http://bit.ly/Societi-1U7baP4. For information on the associated enquiry area of 'Can we predict the severity of coronary artery changes in the BPSU survey of Kawasaki Disease from the phenotypic presentation' refer to the abstract here http://bit.ly/Societi-25LzMpo

Societi UK National Strategy for Kawasaki Disease
Date: March 2016

We have prepared an ambitious strategy to enable a co-ordinated approach to tackling Kawasaki Disease across the UK. Download our strategy here.

Kawasaki Disease incidence in children and adolescents
Correspondence to: Dr R Tulloh, Bristol Children’s Hospital

Published: Online BMGP February 2016

Kawasaki Disease is reported to be increasing in incidence and is the commonest childhood cause of acquired heart disease in the Western world. The aim of this study was to determine the current UK incidence of Kawasaki disease across childhood and adolescence; and investigate trends over time and season. To download the full paper go to

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.

Cardiovascular status after Kawasaki Disease in the UK
Correspondence to: Dr PA Brogan, UCL Institute of Child Health

Published: Online BMJ 27 August 2015

Kawasaki Disease (KD) is a self-limiting medium vessel vasculitis of unknown aetiology, affecting predominantly children under the age of 5 years, resulting in coronary artery aneurysms (CAA) in approximately 25% of untreated patients. There is a need to identify patients at risk of late KD vasculopathy to inform clinical strategies for surveillance and prevention of late cardiovascular events.  This study examined circulating endothelial cells (CECs) years after KD. CECs were increased in patients with KD, were highest in those with CAA, but were also elevated in some patients without CAA, compatible with a state of persistent subclinical vasculitis years after the acute disease. In contrast, arterial stiffness, carotid intima media thickness and conventional cardiovascular risk factors were no different from controls. Impact on clinical practice? The significance of patients having high CECs is unknown. Our study does, however, provide a scientific rationale for recommending lifelong follow-up of all patients with KD.  This study was funded by the British Heart Foundation and KSSG UK.  To download the full paper click the Download button.

Management of  Kawasaki Disease; D Eleftheriou, M Levin, D Shingadia,

R Tulloh, NJ Klein, PA Brogan
Correspondence to: Dr D Eleftheriou, Institute of Child Health, London

Open Access: Published Online First 25 October 2013

This paper summarises recent advances in the understanding of KD pathogenesis and therapeutics, and provides an approach for managing KD patients in the UK in the light of these advances.

​Behaviour sequelae following acute Kawasaki disease

Daniel Carlton-Conway, Raju Ahluwalia, Lucy Henry, Colin Michie, Louise Wood and Robert Tulloh

Correspondence to:

Published: May 25 2005

Kawasaki disease is a systemic vasculitis and may affect cerebral function acutely. The aim of the present study was to measure a number of behaviour and social parameters within a cohort of Kawasaki disease patients.

Refer to the study here:   http://bmcpediatr.biomedcentral.com/articles/10.1186/1471-2431-5-14