Kawasaki Disease

Danielle Hann

ST2 GPVTS 2010

Kawasaki Disease

80% cases aged 6/12 to 5 years Acute inflammatory vasculitis of medium sized

arteries Incidence varies worldwide

– England - 8 per 100 000– Japan – 184 per 100 000

Causes

?? Probably infectious agent triggering an

inflammatory response Likely genetic predisposition

Signs/Symptoms

Fever Extreme irritability Rash Swollen hands and feet Desquamation Conjunctival injection Cervical lymphadenopathy Severe peripheral vasculitis

Investigations

Haematology WCC, anaemia, plt

Urine– Leucocytes

Biochemistry CRP/ESR

CSF ECG Echo

Diagnostic Criteria

Fever of at least 5 days duration plus 4 of the following:

1. Polymorphous exanthema

2. Bilateral non-exudative conjunctival injection

3. Changes in lips and oral cavity

4. Changes in extremities– Erythema, indurative oedema, desquamation

5. Cervical lymphadenopathy

Differential Diagnosis

Differential Diagnosis

Scarlet fever Toxic shock syndrome Measles Glandular fever Stevens-Johnson syndrome

Complications

30-50% develop mild diffuse dilatation of coronary arteries– Develops on average 10 days after onset of fever– Often regresses within 6-8 weeks

20% of coronary artery lesions become aneurysmal– Reduced to 5% with IVIG treatment

May also affect other arteries

Treatment

IV Immunoglobulin– Single dose 5-10 days after onset of fever

Aspirin– Lack of trial evidence but widely accepted use– Dose varies given in acute and sub-acute phase

Corticosteroids– Inconclusive evidence

Immunisations

Not to have live vaccines until 3 months after IVIG

Summary

Acute febrile illness mainly in under 5s Most common cause of acquired heart disease

in children Fever of at least 5 days and 4/5 criteria Clinical features appear sequentially