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 – Japan – 184 per

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

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