Kawasaki disease is defined as a medium-vessel vasculitis predominantly affecting children

Aetiology

• The aetiology of Kawasaki disease is not fully understood
• It's thought to be multifactorial, potentially involving genetic factors, an infectious trigger, and a subsequent abnormal immune response

Clinical features

• The diagnosis of Kawasaki disease is based on the presence of high-grade fevers persisting for more than five days, accompanied by four out of five 'CREAM' features:
  • Conjunctivitis (bilateral, non-exudative)
  • Rash (any non-bullous rash)
  • Edema/Erythema of hands and feet
  • Adenopathy (cervical, commonly unilateral and non-tender)
  • Mucosal involvement (strawberry tongue, oral fissures etc)

Investigations

• The primary investigation for Kawasaki disease is an echocardiogram due to the risk of coronary aneurysms
• Additional investigations may include blood tests looking for elevated inflammatory markers, such as CRP and ESR

Management

• Intravenous immunoglobulin (IVIg) and high-dose aspirin to reduce inflammation and prevent coronary artery aneurysms
• Regular echocardiograms for ongoing surveillance of coronary artery aneurysms
• Patients should be closely monitored as recovery from the acute episode can take several weeks