When viewing this topic in a different language, you may notice some differences in the way the content is structured, but it still reflects the latest evidence-based guidance.

Kawasaki disease

Last reviewed: 9 Nov 2024
Last updated: 14 Dec 2022

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
Full details

Other diagnostic factors

  • pericarditis with effusion
  • congestive heart failure
  • joint pain or edema
  • neurologic manifestations
  • gastrointestinal manifestations
  • urologic manifestations
  • other dermatologic manifestations
Full details

Risk factors

  • Asian ancestry
  • age 3 months to 4 years
  • male sex
Full details

Diagnostic tests

1st tests to order

  • CBC
  • erythrocyte sedimentation rate (ESR)
  • CRP
  • echocardiogram
Full details

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
Full details

Treatment algorithm

ACUTE

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

ONGOING

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.

  • Kawasaki disease images
  • Differentials

    • Staphylococcal or streptococcal infection
    • Systemic juvenile idiopathic arthritis (systemic JIA)
    • Scarlet fever
    More Differentials
  • Guidelines

    • Guideline for the management of Kawasaki disease
    • European consensus-based recommendations for the diagnosis and treatment of Kawasaki disease - the SHARE initiative
    More Guidelines
  • padlock-lockedLog in or subscribe to access all of BMJ Best Practice

Use of this content is subject to our disclaimer