Corticosteroid therapy in Kawasaki Disease; Author(s): Carl Galloway, MD, FHM, FAAP Frontline Medical News

Date: July 2017
Correspondence to: Carl Galloway MD: Published Online

A systematic review and meta analysis considering the clinical question - What is the efficacy of corticosteroid therapy in Kawasaki disease? This study suggests that corticosteroids combined with IVIG as initial therapy for KD showed a more protective effect against CAA, compared with conventional IVIG therapy, and the efficacy was more pronounced in the high-risk patient group

To access the paper synopsis: click here

Corticosteroid therapy in Kawasaki Disease; Carl Galloway, MD, FHM, FAAP

Frontline Medical News

Date: July 2017
Correspondence to: Carl Galloway, MD, FHM, FAAP 

This study suggests that corticosteroids combined with IVIG as initial therapy for KD showed a more protective effect against CAA, compared with conventional IVIG therapy, and the efficacy was more pronounced in the high-risk patient group.

To access the resource: click here

Management of Kawasaki disease in adults

Kara J Denby, Daniel E Clark, Larry W Markham

Date: July 2017

Kawasaki disease is the most common childhood vasculitis in the USA and the most common cause of acquired cardiac disease in children in developed countries. Since the vast majority of Kawasaki disease initially presents at <5 years of age, many adult cardiologists are unfamiliar with the pathophysiology of this disease. This vasculitis has a predilection for coronary arteries with a high complication rate across the lifespan for those with medium to large coronary artery aneurysms. An inflammatory cascade produces endothelial dysfunction and damage to the vascular wall, leading to aneurysmal dilatation. Later, pseudonormalisation of the vascular lumen occurs through vascular remodelling and layering thrombus, but this does not necessarily indicate resolution of disease or reduction of risk for future complications. There is a growing prevalence of Kawasaki disease, making it increasingly relevant for adult cardiologists as this population transitions into adulthood. As the 2017 American Heart Association (AHA) and 2014 Japanese Circulation Society (JCS) guidelines emphasise, Kawasaki disease requires rigorous follow-up with cardiac stress testing and non-invasive imaging to detect progressive stenosis, thrombosis and luminal occlusion that may lead to myocardial ischaemia and infarction. Due to differences in disease mechanisms, coronary disease due to Kawasaki disease should be managed with different pharmacological and non-pharmacological treatment algorithms than atherosclerotic coronary disease. This review addresses gaps in the current knowledge of the disease and its optimal treatment, differences in the AHA and JCS guidelines, targets for future research and obstacles to transition of care from adolescence into adulthood..

To access the resource: click here

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Kawasaki Disease Increases the Incidence of Myopia; Yung-Jen Kung,1 Chang-Ching Wei,2,3 Liuh An Chen,4 Jiin Yi Chen,4 Ching-YaoChang,5 Chao-Jen Lin,6,7 Yun-Ping Lim,8 Peng-Tai Tien,2,9 Hsuan-Ju Chen,10Yong-San Huang,1 Hui-Ju Lin,4,9 and Lei Wan5,9,11,12

Date: July 2017
Correspondence to: Yong-San Huang, Hui-Ju Lin, and Lei Wan 

The prevalence of myopia has rapidly increased in recent decades and has led to a considerable global public health concern. In this study, we elucidate the relationship between Kawasaki disease (KD) and the incidence of myopia. We used Taiwan’s National Health Insurance Research Database to conduct a population-based cohort study. We identified patients diagnosed with KD and individuals without KD who were selected by frequency matched based on sex, age, and the index year. The Cox proportional hazards regression model was used to estimate the hazard ratio and 95% confidence intervals for the comparison of the 2 cohorts. The log-rank test was used to test the incidence of myopia in the 2 cohorts. A total of 532 patients were included in the KD cohort and 2128 in the non-KD cohort. The risk of myopia (hazard ratio, 1.31; 95% confidence interval, 1.08–1.58; ) was higher among patients with KD than among those in the non-KD cohort. The Cox proportional hazards regression model showed that irrespective of age, gender, and urbanization, Kawasaki disease was an independent risk factor for myopia. Patients with Kawasaki disease exhibited a substantially higher risk for developing myopia.

To access the resource: click here

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 

​​​​Research Resources - Kawasaki Disease 

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

​​​​Resources for Clinicians - Kawasaki Disease Research 

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

...together we will

We are building what we wish to be the most comprehensive on line research resource for clinicians.  That's an ambitions we'll keep working towards - adding to the information here as often as possible.

If there are key papers that you think it is important that have links to, please do contact usand we will add them.  If you are working on research into Kawasaki Disease and want to promote that here - or discuss it with us, please get in touch.

​If you'd like us to post information from your organisation on our website, links to relevant papers or articles, please contact us.

The UK Kawasaki Disease Foundation

Dissecting Kawasaki disease: a state-of-the-art review S. M. Dietz 1 & D. van Stijn1 & D. Burgner2,3,4 & M. Levin5 & I. M. Kuipers 6 & B. A. Hutten7 & T. W. Kuijpers1

Date: June 2017
Correspondence to: S. M. Dietz 1 & D. van Stijn1 & D. Burgner2,3,4 & M. Levin5 & I. M. Kuipers 6 & B. A. Hutten7 & T. W. Kuijpers1

Kawasaki disease (KD) is a pediatric vasculitis with coronary artery aneurysms (CAA) as its main complication. The diagnosis is based on the presence of persistent fever and clinical features including exanthema, lymphadenopathy, conjunctival injection, and changes to the mucosae and extremities. Although the etiology remains unknown, the current consensus is that it is likely caused by an (infectious) trigger initiating an abnormal immune response in genetically predisposed children. Treatment consists of high dose intravenous immunoglobulin (IVIG) and is directed at preventing the development of CAA. Unfortunately, 10–20% of all patients fail to respond to IVIG and these children need additional anti-inflammatory treatment. Coronary artery lesions are diagnosed by echocardiography in the acute and subacute phases. Both absolute arterial diameters and z-scores, adjusted for height and weight, are used as criteria for CAA. Close monitoring of CAA is important as ischemic symptoms or myocardial infarction due to thrombosis or stenosis can occur. These complications are most likely to arise in the largest, socalled giant CAA. Apart from the presence of CAA, it is unclear whether KD causes an increased cardiovascular risk due to the vasculitis itself. Conclusion: Many aspects of KD remain unknown, although there is growing knowledge on the etiology

To access the resource: click here

​​​​​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: click 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: click here

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: click here

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: click here


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 10 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:
click here  

For the abstract and link to paper: click here  

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: click 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: click here

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.

​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: click here