Kawasaki disease

Last edited 03/2022 and last reviewed 03/2022

Kawasaki disease was first described in 1967 by Tomisaku Kawasaki.

Kawasaki disease is a febrile systemic vasculitis predominantly affecting children under the age of 5. The highest incidence is in Japan but is becoming increasingly recognised in the West.

The most serious complication of Kawasaki disease is coronary arteritis and aneurysm formation which occurs in a third of untreated patients.

In Japan the mortality is less than 0.1%, in the UK the mortality is 3.7%.

NICE note that clinical features suggestive of Kawasaki disease, in a child less than 5 years, are:

Be aware of the possibility of Kawasaki disease in children with fever that has lasted 5 days or longer.

Additional features of Kawasaki disease may include:

  • bilateral conjunctival injection without exudate
  • erythema and cracking of lips; strawberry tongue; or erythema of oral and pharyngeal mucosa
  • oedema and erythema in the hands and feet
  • polymorphous rash
  • cervical lymphadenopathy

Ask parents or carers about the presence of these features since the onset of fever, because they may have resolved by the time of assessment.

Be aware that children under 1 year may present with fewer clinical features of Kawasaki disease in addition to fever, but may be at higher risk of coronary artery abnormalities than older children

Administration of intravenous immunoglobulin (IVIG) and aspirin has greatly reduced the incidence of coronary lesions in affected children (2)

Prognosis for children diagnosed with Kawasaki disease is primarily based upon extent and severity of coronary artery involvement at diagnosis and at follow-up (2)

  • case-fatality rate in the United States and Japan is less than 0.2%, and the principal cause of death is myocardial infarction resulting from coronary artery occlusion

Reference:

  1. NICE (November 2019). Feverish illness in children Assessment and initial management in children younger than 5 years
  2. Rife E, Gedalia A. Kawasaki Disease: an Update. Curr Rheumatol Rep. 2020;22(10):75. Published 2020 Sep 13. doi:10.1007/s11926-020-00941-4