Kawasaki disease

Last reviewed: 28 Dec 2022
Last updated: 14 Dec 2022

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • polymorphous rash
  • conjunctival injection
  • mucositis
  • skin changes in the peripheral extremities
  • enlarged cervical lymph nodes
  • coronary artery aneurysms
  • fever and extreme irritability
More key diagnostic factors

Other diagnostic factors

  • pericarditis with effusion
  • congestive heart failure
  • joint pain or oedema
  • neurological manifestations
  • gastrointestinal manifestations
  • urological manifestations
  • other dermatological manifestations
Other diagnostic factors

Risk factors

  • Asian ancestry
  • age 3 months to 4 years
  • male sex
More risk factors

Diagnostic investigations

1st investigations to order

  • FBC
  • erythrocyte sedimentation rate (ESR)
  • serum CRP
  • echocardiogram
More 1st investigations to order

Investigations to consider

  • serum LFTs
  • urinalysis
  • chest x-ray
  • electrocardiogram
  • ultrasonography of the gallbladder
  • ultrasonography of the testes
  • lumbar puncture
  • magnetic resonance angiography
  • cardiac catheterisation and angiography
  • natriuretic peptide tests
More investigations to consider

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.

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