Kawasaki Disease

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: • Polymorphous exanthema • Bilateral non-exudative conjunctival injection • Changes in lips and oral cavity • Changes in extremities • Erythema, indurative oedema, desquamation • 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

Kawasaki Disease