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Kawasaki Disease

Key considerations: Coronary artery aneurysm, Myocarditis, Macrophage activation syndrome

Cannot miss

  • Coronary artery aneurysm
  • Myocarditis
  • Macrophage activation syndrome

Likely diagnoses

  • Kawasaki disease
  • Viral exanthem
  • Scarlet fever
  • Drug reaction
  • JIA

Red flags

  • Coronary artery dilation on echo
  • Shock
  • Persistent fever despite IVIG

Workup

  • history: Fever ≥5 days + 4 of 5 criteria: bilateral conjunctival injection, oral mucosal changes, extremity changes, rash, cervical LAD; exam: Irritability, rash, conjunctivitis without exudate, strawberry tongue, periungual peeling; labs: CBC (thrombocytosis after week 1), CRP, ESR, BMP, LFTs, UA (sterile pyuria); BNP/troponin if myocarditis concern; imaging: Echocardiogram at diagnosis, 2 weeks, 6-8 weeks; bedside: Echocardiogram

Management

  • immediate: IVIG 2g/kg single infusion over 10-12 hours; Aspirin 30-50mg/kg/day until afebrile, then 3-5mg/kg/day for 6-8 weeks; general: Second dose IVIG if persistent fever >36hr after first dose; Infliximab for IVIG-resistant cases; Follow-up echo to assess coronary arteries

Disposition

  • admit: All patients for IVIG administration and monitoring; discharge: After afebrile >48hr post-IVIG, no coronary changes, with close follow-up; consults: Pediatric cardiology for echo and follow-up

Clinical pearls

  • Incomplete Kawasaki: fever ≥5 days with 2-3 criteria + lab/echo findings — still treat!
  • Coronary artery aneurysms develop in 25% of untreated children — IVIG reduces this to <5%

Source and review

  • AHA Kawasaki Disease Guidelines 2017. Last reviewed: 2024-11-01