Acute febrile illness lasting 5 or more days.
Typical signs include fever, polymorphic rash, injected eyes, and mucosal erythema with strawberry tongue.
Swelling and erythema of the hands and feet occur in the acute stage, followed by desquamation in the second week.
Unilateral non-purulent cervical lymphadenopathy is present in about 40% of cases.
Coronary aneurysms develop in 20% to 25% of untreated patients.
Standard treatment includes intravenous immunoglobulin and/or aspirin. In resistant cases, corticosteroids or a tumour necrosis factor (TNF)-alpha inhibitor may be necessary.
Kawasaki disease (KD) is an acute, febrile, self-limiting, systemic vasculitis of unknown origin that almost exclusively affects young children. In an immunogenetically pre-disposed host, one or more infectious agents may play a role in triggering the clinical manifestations of the disease. Clinically, it is characterised by fever, polymorphic rash, conjunctivitis, mucosal erythema with strawberry tongue, induration of the hands and feet, and unilateral cervical lymphadenopathy. Morbidity and mortality depend on coronary aneurysms that develop in 20% to 25% of untreated patients. KD is the leading cause of acquired heart disease in children under 5 years of age 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
- serum 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
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
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
Abraham Gedalia, MD
Professor of Pediatrics and Chief
Division of Pediatric Rheumatology
LSU Health Sciences Center and Children's Hospital
AG declares that he has no competing interests.
James Krulisky, MD
Assistant Professor of Clinical Pediatrics
Department of Pediatric Cardiology
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
- Fever in under 5s: assessment and initial management
- Diagnosis, treatment, and long-term management of Kawasaki disease
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