About Kawasaki Disease

Kawasaki disease (KD) is a vasculitic disease, with onset typically in early childhood (77% of patients are <5 yo).

  • More common in boys (~1.5:1 M to F ratio)
  • Younger (under 1 yo) = highest risk of coronary artery aneurysm (also most likely to present as incomplete!)
  • Rapid onset, may be preceded by URI or AGE
  • Acute phase (first 7-11 days)
    Subacute phase (12+ days)
    Convalescent phase

Fever >5 days (poorly responsive to NSAIDs) with 4+ of the following:

  • Red eye (without exudate) (view image)
  • Mucosal changes:
  • Changes in extremities:
    • Erythema
    • Edema of hands/feet in acute phase
    • Periungual desquamation in convalescent phase (view image)
  • Polymorphous rash
    • Primarily truncal
    • Non-vesicular
  • Cervical LAD (at least one node >1.5 cm)

Lab Workup

  • Elevated ESR, CRP, WBCs w/left shift
  • Thrombocytosis by the 2nd week (reactive)
  • Normocytic, normochromic anemia (in ~50%)
  • Sterile pyuria (but LE negative), common
  • Elevated ALT/AST
  • Abnormal lipid profiles

Rule out other causes of fever/sxs!

  • Admission for evaluation and treatment of inflammation and prevention of cardiac sequelae
  • Echocardiogram
  • Early treatment with:
    • Intravenous immunoglobulin (IVIg) (often a single dose)
    • High dose aspirin (80-100 mg/kg divided QID)
  • Consider adding steroids or TNF-inhibitor for refractory cases
  • Baseline echocardiogram
  • Repeat echo 2 weeks after diagnosis
  • Further monitoring determined by cardiology
  • Treatment within 10 days of onset is very effective.
  • Can treat after 10 days with IVIg but less effective at preventing aneurysms
    • No live virus vaccines for 11 months post IVIg
  • Mortality is ~0.1% in the US.

Related Links

References

 

Kawasaki Disease Case Study

 

ACR Vasculitis Treatment Guideline

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