Kawasaki disease

Specialties > Child health

Background knowledge 🧠

Definition

Kawasaki disease (KD) is an acute, self-limiting vasculitis primarily affecting children under 5 years of age.
It is the leading cause of acquired heart disease in children in developed countries.
The disease predominantly affects medium-sized arteries, especially the coronary arteries.

Epidemiology

Incidence is highest in Japan, with lower rates in Europe and North America.
Affects approximately 8-67 per 100,000 children under 5 years annually in the UK.
Peak incidence is between 6 months and 2 years of age.
Slightly more common in males than females.

Aetiology and Pathophysiology

Exact cause is unknown, but an infectious trigger in genetically predisposed children is suspected.
Inflammation affects the vascular endothelium, leading to vasculitis.
Coronary artery aneurysms can develop in untreated cases, leading to long-term cardiac complications.
Autoimmune mechanisms are thought to play a role.
Genetic factors include polymorphisms in immune-related genes.

Types

Typical Kawasaki Disease: Meets the classic criteria (fever plus at least four of the five principal clinical features).
Atypical (or incomplete) Kawasaki Disease: Fewer than four principal clinical features, more common in younger infants.
Chronic Kawasaki Disease: Persistent inflammation and coronary artery complications beyond the acute phase.

Clinical Features 🌡️

Symptoms

Prolonged fever (lasting more than 5 days), unresponsive to antipyretics.
Irritability and malaise.
Non-purulent conjunctivitis.
Rash: Polymorphous, typically starting on the trunk.
Abdominal pain, vomiting, or diarrhoea (gastrointestinal involvement).

Signs

Bilateral non-purulent conjunctivitis.
Oropharyngeal changes: Reddened, cracked lips, “strawberry” tongue, erythema of the oral mucosa.
Cervical lymphadenopathy (usually unilateral).
Extremity changes: Erythema and swelling of hands and feet, followed by desquamation (skin peeling).
Cardiac involvement: Tachycardia, gallop rhythm, signs of heart failure.

Investigations 🧪

Tests

Blood tests: Raised inflammatory markers (ESR, CRP), anaemia, leucocytosis, thrombocytosis.
Echocardiogram: Essential to assess coronary artery involvement.
Urinalysis: Sterile pyuria may be present.
Liver function tests: May show mild elevation in transaminases.
ECG: To assess for arrhythmias or signs of myocardial ischemia.

Management 🥼

Management

Intravenous immunoglobulin (IVIG): Given within the first 10 days to reduce coronary artery complications.
Aspirin: High-dose in the acute phase, then low-dose for antiplatelet effect.
Corticosteroids: Considered in refractory cases or high-risk patients.
Close monitoring: Regular echocardiograms to detect coronary artery changes.
Long-term follow-up: For those with coronary artery aneurysms, managed with cardiology input.

Complications

Coronary artery aneurysms: Occurs in 15-25% of untreated cases.
Myocardial infarction: Due to coronary thrombosis or aneurysm rupture.
Heart failure: Resulting from myocarditis or coronary artery involvement.
Arrhythmias: Secondary to myocardial involvement.
Peripheral arterial occlusion: Due to thrombosis.

Prognosis

With early treatment, the prognosis is generally good.
Coronary artery abnormalities resolve in most cases treated early.
Long-term cardiac sequelae can occur in those with significant coronary involvement.
Mortality rate is low with appropriate treatment.

Key Points

Early diagnosis and treatment with IVIG are crucial to prevent coronary complications.
Consider Kawasaki Disease in any child with prolonged fever and signs of systemic inflammation.
Long-term follow-up is essential for those with coronary artery involvement.
Kawasaki Disease is a clinical diagnosis supported by laboratory and imaging findings.

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