Kawasaki disease
Medium-vessel vasculitis (unknown trigger)
Overview
A medium-vessel vasculitis of young children and the leading cause of acquired paediatric heart disease — coronary artery aneurysms. Diagnosed clinically: fever ≥5 days plus ≥4 of five features. Crucially it does NOT respond to antibiotics.
Recognise
- Fever ≥5 days PLUS ≥4 of: bilateral non-purulent CONJUNCTIVITIS; cracked lips/STRAWBERRY tongue; cervical lymphadenopathy; polymorphous rash; erythema/oedema of hands & feet then peeling
- Very irritable child
- Later: peeling of fingertips/toes
Red flags
- Coronary artery aneurysms — the feared complication; incomplete Kawasaki in infants
Differentials & how to tell them apart
Investigations
Clinical diagnosis. Bloods: raised CRP/ESR, thrombocytosis (2nd week), anaemia. ECHOCARDIOGRAM at diagnosis and follow-up for coronary aneurysms.
Management
IV immunoglobulin + aspirin
- 1IV immunoglobulin (single dose) PLUS aspirin. Echocardiogram for coronary aneurysms.Gate: Aspirin is normally avoided in children (Reye syndrome) — Kawasaki is a specific EXCEPTION
- 2Refractory: second IVIG dose, corticosteroids or infliximab. Long-term aspirin ± anticoagulation if aneurysms.
Key points
Two exam traps: (1) it mimics scarlet fever/measles but does NOT respond to antibiotics; (2) aspirin is used despite the usual Reye-syndrome caution.
Monitor & prognosis
Serial echocardiography for coronary aneurysms; inflammatory markers.
Good with early IVIG; aneurysms cause long-term cardiac risk.
Source: NICE; RCPCH