GPAtlas🧭 When to suspect
Kawasaki disease is an acute, self-limiting systemic vasculitis of medium-sized arteries that almost exclusively affects young children.
• The peak is between 6 months and 5 years (and within that, 1–2 years).
• The defining feature is a high, persistent fever lasting 5 days or more that is characteristically unresponsive to antipyretics and antibiotics.
• The child is typically miserable and disproportionately irritable.
It matters because it is now the commonest cause of acquired heart disease in children in the UK:
• The inflammation has a predilection for the coronary arteries, and untreated, up to a quarter of children develop coronary artery aneurysms.
• Treatment with intravenous immunoglobulin (IVIG) within the first 10 days of illness cuts that risk to around 3–5%, so the whole prognosis can hinge on timely recognition.
The primary-care task is therefore simple but high-stakes:
• Think of Kawasaki disease in any young child with prolonged unexplained fever, and refer for same-day paediatric assessment.
• Have an especially low threshold in infants, who often present with incomplete features yet carry the greatest cardiac risk.
| Principal feature (fever ≥ 5 days + ≥ 4 of these 5) | What to look for |
|---|---|
| Bilateral conjunctival injection | Bilateral, bulbar and non-purulent (no discharge), with characteristic limbal sparing. |
| Lip and oral changes | • Red, dry, cracked or fissured lips • Strawberry tongue • Diffuse mucosal erythema, without ulcers or exudate |
| Polymorphous rash | • Widespread and non-vesicular • Often accentuated in the nappy/groin area, where early peeling may appear |
| Extremity changes | • Erythema and firm oedema of the palms and soles (acute) • Periungual desquamation of fingers and toes (subacute, around week 2–3) |
| Cervical lymphadenopathy | • Usually unilateral, ≥ 1.5 cm, firm and non-suppurative • Often the least common feature |
Classical Kawasaki disease is diagnosed with fever for ≥ 5 days plus ≥ 4 of the 5 features (or fewer features where echocardiography already shows coronary involvement).
Incomplete Kawasaki disease should be suspected in:
• Any infant under 6 months with unexplained fever for ≥ 7 days
• Any child with fever ≥ 5 days and only 2–3 features
These children are at the highest risk of coronary complications. Features evolve sequentially and may not all be present at once.
|
⚠️ Common pitfall • Waiting for the "full house": the classic error is withholding referral until all 4 features are present or the fever has been documented for a complete 5 days. • Features appear sequentially and may never all coexist. • Infants frequently have incomplete disease yet the highest coronary risk. • A persistently febrile, miserable young child with even 2–3 compatible features needs referral, not another course of antibiotics. |
Source: NICE NG143
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