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Paediatrics

Kawasaki disease

An acute systemic medium-vessel vasculitis in childhood with persistent fever and mucocutaneous features; it is a time-critical diagnosis because coronary artery inflammation can lead to aneurysm, thrombosis and myocardial ischaemia.

In a nutshell

Kawasaki disease is an acute childhood medium-vessel vasculitis: persistent fever plus a changing combination of conjunctivitis, rash, oral mucosal change, cervical lymphadenopathy and red or swollen hands and feet. Consider it in every child with fever lasting 5 days or longer, including infants with incomplete features. Admit urgently for specialist assessment, early IVIG plus aspirin, and serial echocardiography because coronary aneurysm, thrombosis and myocardial ischaemia are the feared complications.

Classic presentation

A young child with fever lasting 5 days or longer and some combination of bilateral non-exudative conjunctivitis, polymorphous rash, cervical lymphadenopathy, red cracked lips or strawberry tongue, and red swollen hands and feet that may later peel.

Key points

  • NICE: consider Kawasaki disease in every child with fever lasting 5 days or longer; ask about features that have appeared and resolved.
  • CRASH-and-burn is a recognition aid, not a requirement to have all five features before referral or treatment.
  • Infants can have fewer clinical features and are at higher risk of coronary artery abnormalities.
  • The feared complication is coronary dilatation or aneurysm, with later thrombosis, myocardial infarction or sudden deterioration.
  • Early IVIG plus specialist-prescribed aspirin reduces inflammatory and thrombotic risk; follow current BNFc and local paediatric protocol.
  • Obtain ECG and echocardiography promptly without delaying treatment; repeat echo around 10–14 days and 6–8 weeks from illness onset.
  • Persistent fever, static CRP or continuing inflammation after IVIG needs tertiary paediatric cardiology/rheumatology discussion.

First-line investigation

ECG and echocardiography at presentation, alongside FBC, CRP/ESR, renal and liver tests, albumin and urinalysis; a normal early echo does not end serial follow-up.

Management

Consider it after 5 days of fever

  • Consider Kawasaki disease in every child with fever lasting 5 days or longer, including infants with fewer or transient principal features; document signs reported since fever onset.1,2

Admit and assess the heart

  • Admit for urgent paediatric specialist assessment, ECG and echocardiography; assess for shock, myocarditis, heart failure, PIMS-TS and serious infection in parallel.1,2

Treat early

  • Start IVIG as early as possible with specialist prescribing, plus aspirin under current BNFc/local paediatric protocol; do not delay treatment for an echo.2,4,3

Check response and repeat echo

  • Monitor fever, clinical state and inflammatory markers; repeat echo around 10–14 days and 6–8 weeks from illness onset, with more frequent cardiology imaging if coronary disease or ongoing inflammation is present.2,3

Escalate persistent inflammation

  • Persistent or recurrent fever, static CRP or continuing inflammation after IVIG requires tertiary cardiology/rheumatology discussion; further IVIG, corticosteroids or biologic treatment is specialist-led.2

Protect abnormal coronaries

  • Continue antiplatelet or anticoagulant treatment and long-term cardiology follow-up when coronary abnormalities are present; give post-IVIG vaccine advice and urgent-return safety-netting before discharge.2,3,5

Exam traps

  • Do not require fever plus four of five features: NICE says to consider Kawasaki disease after 5 days of fever even when additional features are absent.
  • Ask about transient signs because conjunctivitis, rash, oral changes and extremity changes may not be present together at review.
  • A normal early platelet count or inflammatory marker does not exclude Kawasaki disease; thrombocytosis is often later.
  • A normal initial echocardiogram does not exclude evolving coronary disease; repeat imaging is required.
  • Aspirin is normally avoided in children, but Kawasaki disease is a specialist-supervised exception; never advise unsupervised aspirin.
  • Persistent or recurrent fever after IVIG is a treatment-response problem requiring specialist escalation, not reassurance.

Illustrations

CRASH-and-burn feature mapDiagram of a child with Kawasaki disease annotated with conjunctival injection, rash, cervical lymphadenopathy, oral mucosal change, hand and foot changes, and persistent fever; label it as a recognition aid rather than a requirement to have every feature.PassFinals · original
Periungual desquamationClinical photograph or illustration of peeling around the fingers or toes in the subacute phase of Kawasaki disease; make clear that desquamation is a later feature and should not be awaited before referral.Dong Soo Kim, Wikimedia Commons · CC-BY-SA-4.0

Key sources

  1. NICE NG143: Fever in under 5s: assessment and initial management – recommendations 1.2.14 and 1.2.25–1.2.27 (Consider Kawasaki disease in children with fever lasting 5 days or longer; ask about features since onset; infants may present with fewer features and higher coronary risk.)
  2. North West Children’s Hospital Doctors Network: Kawasaki Disease Guidelines for Diagnosis & Management, February 2025 (UK regional specialist pathway covering incomplete disease, investigations, IVIG, aspirin, escalation, echocardiography, follow-up and immunisation.)
  3. University Hospitals Sussex NHS Foundation Trust: Kawasaki Disease, reviewed 16 February 2026 (Current NHS trust information on IVIG, aspirin, post-IVIG immunisation advice and cardiac follow-up.)
  4. BNFc: current paediatric prescribing information for aspirin and immunoglobulin (Use the current BNFc monographs and local IVIG policy for patient-specific dosing, contraindications, interactions and infusion requirements.)
  5. Great Ormond Street Hospital: Kawasaki disease (Specialist paediatric information on coronary complications, IVIG, aspirin and long-term cardiology follow-up.)

This page is exam revision material, not medical advice, and must not be used for patient care. Always check drug doses against the BNF and current guidance. Full disclaimer.