MDCalc

Kawasaki Disease Diagnostic Criteria

Diagnoses Kawasaki Disease.

Fever for ≥5 days
Acute change in extremities
Erythema of palms and soles, or edema of hands and feet
Subacute change in extremities
Periungual peeling of fingers and toes in weeks 2 and 3
Polymorphous exanthem
Bilateral bulbar conjunctival injection without exudate
Changes in lips and oral cavity
Erythema, lips cracking, strawberry tongue, diffuse injection of oral/pharyngeal mucosae
Cervical lymphadenopathy
>1.5 cm diameter, usually unilateral
Coronary artery disease detected by 2D echo or coronary angiogram

Result:

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Advice
  • If the diagnosis of KD is made in the acute phase, treatment should be initiated to prevent CA abnormalities.
  • If the patient does not meet the criteria for KD, consider incomplete KD.
Management
  • Prompt treatment in the acute phase with intravenous immune globulin (IVIG) and aspirin reduces the prevalence of the development of CA abnormalities and subsequent mortality and morbidity. The two appear to have an additive effect (Newburger 2004).
  • Treatment should be instituted within the first 10 days of the illness and if possible within 7 days of illness.
  • IVIG
    • The mechanism is unknown, but believed to be linked to the anti-inflammatory effect.
    • Different dosing regimens exist. A single, higher dose is most effective (Newburger 1991).
    • Based on timing of development of the CA abnormalities, therapy should be instituted within the first 10 days of illness and, if possible, within 7 days of illness.
    • IVIG should be considered in children presenting after the 10th day of illness if they have persistent fever without other explanation or aneurysms and ongoing systemic inflammation, as manifested by elevated ESR or CRP (Newburger 2004).
  • High dose aspirin
    • Administered with IVIG.
    • Has anti-inflammatory effects, but does not appear to lower the frequency of development of CA abnormalities (Newburger 2004).
    • Dosing regimens vary.
    • The risk of Reye Syndrome must be considered and weighed against the benefits of therapy.
    • Annual influenza vaccination is recommended in patients taking aspirin long term.
    • The risks and benefits of varicella vaccination in children receiving long term aspirin therapy must be considered.
    • Ibuprofen should be avoided because it antagonizes the irreversible platelet inhibition that is induced by aspirin.
  • Steroids may be considered as adjuvant treatment to help decrease the rate of CA abnormalities, although they are not routinely used.
  • Management algorithms exist for the treatment of incomplete and refractory KD.
Critical Actions
  • Suspect KD in any patient (particularly children <5 years old) presenting with several days of otherwise unexplained fever.
  • Early diagnosis and treatment during the acute phase is essential in preventing CA abnormalities.
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