Kawasaki Disease

Kawasaki Disease is a topic covered in the 5-Minute Emergency Consult.

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  • Acute inflammatory process involving multiple organs
  • Leading cause of childhood-acquired heart disease in developed countries
  • Vasculitis is most severe in medium-sized arteries
  • Acute cardiac sequelae:
    • Coronary artery aneurysm:
      • Often lead to stenosis after healing
    • Giant aneurysm:
      • May rupture
    • Myocarditis
    • Pericarditis
  • Stages:
    • Acute (lasts 1–2 wk):
      • Fever
      • Oral mucosal erythema
      • Conjunctival injection
      • Erythema and edema of hands and feet
      • Cervical adenopathy
      • Aseptic meningitis
      • Hepatic dysfunction
      • Diarrhea
      • Myocarditis
      • Pericardial effusion (20–40%)
      • No aneurysms by ECHO
    • Subacute (when fever, rash, and lymphadenopathy resolve until about 4 wk):
      • Anorexia
      • Irritability
      • Desquamation of hands and feet
      • Thrombocytosis
      • Coronary artery aneurysms (20% if untreated)
      • Risk for sudden death is highest
    • Convalescent phase (about 6–8 wk):
      • Clinical signs are absent
      • ESR normalizes
    • Epidemiology:
      • 80% of cases occur in children <4 yr old; peak at 1–2 yr; rare in infants <3 mo old
      • Adult cases have been reported
      • Asians are at highest risk
      • Males > females 1.5:1
  • Genetics:
    • Possible genetic predisposition
  • Risks for nonresponse to standard therapy (10–15%):
    • Elevated band count
    • Low albumin level
    • Abnormal initial ECHO
  • Risks for development of coronary artery aneurysms:
    • Extremes of age
    • Male gender
    • Prolonged fever
    • Persistent fever after treatment
    • Delay in diagnosis
    • Increased WBC and/or band count
    • Low hematocrit
    • Significant increase in CRP and/or ESR


  • Unknown—believed to be infectious based on manifestations of disease, epidemics, and increased numbers of cases in winter and early spring
  • Current theory:
    • Activation of immune system in response to infection
    • Genetically susceptible host
    • May explain why certain ethnicities have higher incidence of disease
      • More prominent in Asian countries

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