GPAtlas🧭 When to suspect
Kawasaki disease is an acute, self-limiting systemic vasculitis of medium-sized arteries that almost exclusively affects young children, with a peak 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, in a child who 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, or 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, and 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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