Kawasaki disease

Last reviewed: 27 Apr 2022
Last updated: 10 Sep 2019

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
More key diagnostic factors

Other diagnostic factors

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

  • CBC
  • serum erythrocyte sedimentation rate (ESR)
  • 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
More investigations to consider

Emerging tests

  • magnetic resonance angiography
  • cardiac catheterization and angiography

Treatment algorithm

ACUTE

presentation ≤10 days from onset; or presentation >10 days from onset with risk factors for complications

presentation >10 days from onset without risk factors for complications

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

Abraham Gedalia, MD

Professor of Pediatrics and Chief

Division of Pediatric Rheumatology

LSU Health Sciences Center and Children's Hospital

New Orleans

LA

Disclosures

AG declares that he has no competing interests.

James Krulisky, MD

Assistant Professor of Clinical Pediatrics

Department of Pediatric Cardiology

Children's Hospital

New Orleans

LA

Disclosures

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

    • Diagnosis, treatment, and long-term management of Kawasaki disease
    More Guidelines
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