Summary
Definition
History and exam
Key diagnostic factors
- polymorphous rash
- conjunctival injection
- mucositis
- 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 edema
- neurologic manifestations
- gastrointestinal manifestations
- urologic manifestations
- other dermatologic manifestations
Risk factors
- Asian ancestry
- age 3 months to 4 years
- male sex
Diagnostic tests
1st tests to order
- CBC
- erythrocyte sedimentation rate (ESR)
- CRP
- echocardiogram
Tests to consider
- serum LFTs
- urinalysis
- chest x-ray
- electrocardiogram
- ultrasonography of the gallbladder
- ultrasonography of the testes
- lumbar puncture
- magnetic resonance angiography
- cardiac catheterization and angiography
- natriuretic peptide tests
Treatment algorithm
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
Contributors
Authors
Paul Brogan, BSc(Hon), MBChB(Hon), FRCPCH, MSc, PhD
Professor of vasculitis
University College London
London
UK
Disclosures
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
London
UK
Disclosures
KM declares she has no competing interests.
Acknowledgements
Dr Paul Brogan and Dr Kirsty McLellan would like to gratefully acknowledge Professor Abraham Gedalia and Dr James Krulisky, previous contributors to this topic.
Disclosures
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.
Peer reviewers
Michael Levin, null
Professor of International Child Health
Imperial College London
London
Disclosures
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
New Orleans
LA
Disclosures
RWS declares that he has no competing interests.
John L. Ey, MD
Clinical Professor of Pediatrics
Department of Pediatrics
Oregon Health Science University
Portland
OR
Disclosures
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
Victoria
Australia
Disclosures
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.
References
Key articles
McCrindle BW, Rowley AH, Newburger JW, et al; American Heart Association. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation. 2017 Apr 25;135(17):e927-99.Full text Abstract
Kawasaki T, Kosaki T, Okawa S, et al. A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan. Pediatrics. 1974 Sep;54(3):271-6. Abstract
de Graeff N, Groot N, Ozen S, et al. European consensus-based recommendations for the diagnosis and treatment of Kawasaki disease - the SHARE initiative. Rheumatology (Oxford). 2019 Apr 1;58(4):672-82.Full text Abstract
Gorelik M, Chung SA, Ardalan K, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of Kawasaki disease. Arthritis Care Res (Hoboken). 2022 Apr;74(4):538-48. Abstract
Newburger JW, Takahashi M, Beiser AS, et al. A single intravenous infusion of gamma globulin as compared with four infusions in the treatment of acute Kawasaki syndrome. N Engl J Med. 1991 Jun 6;324(23):1633-9. Abstract
Lei WT, Chang LS, Zeng BY, et al. Pharmacologic interventions for Kawasaki disease in children: A network meta-analysis of 56 randomized controlled trials. EBioMedicine. 2022 Apr;78:103946.Full text Abstract
McCrindle BW, Rowley AH, Newburger JW, et al; American Heart Association. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation. 2017 Apr 25;135(17):e927-99.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Staphylococcal or streptococcal infection
- Systemic juvenile idiopathic arthritis (systemic JIA)
- Scarlet fever
More DifferentialsGuidelines
- 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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