Kawasaki disease is the second most common vasculitis in childhood (the most common being IgA vasculitis).
Presents with an acute self-limiting febrile illness usually in young children, and is the leading cause of acquired heart disease in children aged under 5 years in the developed world.
Classic features include fever, rash, conjunctivitis, lymphadenopathy, changes to the lips or oral mucosal, and peripheries, with initial swelling and erythema followed by desquamation in the second week.
The most serious sequelae is coronary artery aneurysm, affecting 20% to 25% of untreated patients and 19% of treated patients in the UK, with even higher coronary artery aneurysm complication rates in those aged under 1 year.
Current standard treatment includes intravenous immunoglobulin (IVIG), alongside aspirin. There remains clinical equipoise if up-front corticosteroids reduce coronary artery aneurysms in unselected cases, but they are usually added for high-risk cases as well as patients with Kawasaki shock syndrome.
In IVIG-resistant cases, other medications which may be of benefit include high-dose pulsed intravenous methylprednisolone, tumour necrosis factor (TNF)-alpha inhibitors (e.g., infliximab), interleukin (IL)-1 inhibitors, (e.g., anakinra), or ciclosporin.
Kawasaki disease (KD) is an acute, febrile, self-limiting, systemic vasculitis, the exact aetiology of which is not fully understood that almost exclusively affects young children. In an immunogenetically pre-disposed host, an environmental trigger, including infectious agents, results in an aberrant inflammatory host response leading to the clinical manifestations. Clinically, it is characterised by fever, polymorphic rash, conjunctivitis, mucosal erythema with strawberry tongue, erythema and swelling of the hands and feet, and unilateral cervical lymphadenopathy. The most serious complication is the development of coronary artery aneurysms, which account for significant morbidity and mortality and affect 20% to 25% of untreated patients. KD is the leading cause of acquired heart disease in children aged under 5 years in the US and other developed countries.
History and exam
Key diagnostic factors
- presence of risk factors
- polymorphous rash
- conjunctival injection
- skin changes in the peripheral extremities
- enlarged cervical lymph nodes
- coronary artery aneurysms
- fever and extreme irritability
Other diagnostic factors
- pericarditis with effusion
- congestive heart failure
- joint pain or oedema
- neurological manifestations
- gastrointestinal manifestations
- urological manifestations
- other dermatological manifestations
- Asian ancestry
- age 3 months to 4 years
- male sex
1st investigations to order
- erythrocyte sedimentation rate (ESR)
- serum CRP
Investigations to consider
- serum LFTs
- chest x-ray
- ultrasonography of the gallbladder
- ultrasonography of the testes
- lumbar puncture
- magnetic resonance angiography
- cardiac catheterisation and angiography
- natriuretic peptide tests
presentation ≤10 days from onset; or presentation >10 days from onset with evidence of ongoing inflammation
presentation >10 days from onset without evidence of ongoing inflammation
after initial episode: Z score always <2; no involvement at any time
after initial episode: Z score ≥2.0 to <2.5; dilation only
after initial episode: Z score ≥2.5 to <5.0; small aneurysm
after initial episode: Z score ≥5 to <10 (with absolute luminal dimension <8 mm); medium aneurysm
after initial episode: Z score ≥10 or absolute luminal diameter ≥8 mm; large or giant aneurysm
Paul Brogan, BSc(Hon), MBChB(Hon), FRCPCH, MSc, PhD
Professor of vasculitis
University College London
PB is co-chief investigator of the KDCAAP trial, and is an author of several references cited in this topic.
Kirsty McLellan, BMedSci, MBChB, MRCPCH
Specialist Registrar in Paediatric Rheumatology
Great Ormond Street Hospital
KM declares she has no competing interests.
Dr Paul Brogan and Dr Kirsty McLellan would like to gratefully acknowledge Professor Abraham Gedalia and Dr James Krulisky, previous contributors to this topic.
AG declares that he has no competing interests. JK declares that he is a paid consultant for Axia Medical Solutions, a small skincare company from Carlsbad, CA.
Michael Levin, null
Professor of International Child Health
Imperial College London
ML declares that he has no competing interests
Russell W. Steele, MD
Editor in Chief
Journal of Clinical Pediatrics
Department of Pediatrics
Division of Infectious Diseases
Ochsner Children's Health Center
RWS declares that he has no competing interests.
John L. Ey, MD
Clinical Professor of Pediatrics
Department of Pediatrics
Oregon Health Science University
JLE declares that he has no competing interests.
David Burgner, BSc(Hons), MBChB, MRCP, MRCPCH, FRACP, DTMH, PhD
Principal Research Fellow
Murdoch Childrens Research Institute
The Royal Children’s Hospital
DB has received competitive research funding from the National Heart Foundation Australia and from the Agency for Science, Technology and Research of the Singapore Government. He is co-inventor on a patent related to diagnostics submitted through the Genome Institute of Singapore.
- Staphylococcal or streptococcal infection
- Systemic juvenile idiopathic arthritis (systemic JIA)
- Scarlet fever
- Guideline for the management of Kawasaki disease
- European consensus-based recommendations for the diagnosis and treatment of Kawasaki disease - the SHARE initiative
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