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Updated: May 6 2022

Kawasaki Syndrome

Images disease.jpg
  • Snapshot
    • A 5-year-old boy presents to the emergency room with 5 days of fevers, ranging from 102-104°F (38.9-40°C). His mother reports that he also has a bad rash that developed on day 3. On physical exam, he has bilateral conjunctivitis, an extensive morbilliform rash on his trunk with desquamation, a bright red tongue, and swollen hands and feet. Labs are remarkable for elevated C-reactive protein, white blood cell count, and erythrocyte sedimentation rate. He is immediately given intravenous immunoglobulin and aspirin and sent for an echocardiogram.
  • Introduction
    • Clinical definition
      • acute febrile medium-vessel vasculitis, also known as mucocutaneous lymph node syndrome, characterized by CRASH and burn
        • Conjunctival injection
        • Rash
        • Adenopathy
        • Strawberry tongue
        • Hand and foot rash
        • fever (burn)
  • Epidemiology
    • Demographics
      • children < 5 years of age
      • more common in those of Asian descent
    • Risk factors
      • family history
      • being of Asian or Pacific Islander descent
    • Pathogenesis
      • unknown but thought to involve infection, environmental factors, immunologic abnormalities, and genetics
  • Presentation
    • Symptoms
      • 5 days or more of high fever
      • arthritis may be reported
    • Physical exam
      • bilateral non-purulent conjunctival injection
      • rash
        • erythematous morbilliform rash with desquamation on the trunk that may spread
          • may also be urticarial or erythema multiforme-like
          • non-vesicular
        • erythema and swelling of the hands and feet with desquamation
      • oral mucositis
        • red cracked lips
        • strawberry tongue
      • asymmetric cervical adenopathy
        • firm, unilateral, and nontender
  • Imaging
    • Echocardiography
      • indications
        • for all patients with Kawasaki disease to assess for cardiac abnormalities
        • obtained at the time of diagnosis and again at 2 and 6-8 weeks after diagnosis
  • Studies
    • Labs
      • ↑ inflammatory markers
        • ↑ C-reactive protein
        • ↑ erythrocyte sedimentation rate
        • ↑ platelet count (often at weeks 2-3)
        • ↑ white blood cells
      • ↑ liver transaminases
  • Differential
    • Takayasu arteritis
      • distinguishing factors
        • weak upper extremity pulses
        • no hand/foot rash or strawberry tongue
    • Scarlet fever
      • distinguishing factors
        • sandpaper-like rash
        • in the setting of group A streptococcal pharyngitis
    • Staphylococcal scalded skin syndrome
      • distinguishing factors
        • + Nikolsky sign
    • Making the diagnosis
      • diagnostic criteria
        • fever for 5 or more days (burn)
        • 4/5 of CRASH
          • Conjunctival injection
          • Rash
          • Adenopathy
          • Strawberry tongue
          • Hand and foot rash
  • Treatment
    • Management approach
      • treatment includes both intravenous immunoglobulin and aspirin
    • Medical
      • intravenous immunoglobulin (IVIG)
        • indications
          • for all patients
          • prevention of coronary artery involvement
      • high-dose aspirin
        • indications
          • for all patients
        • high dose initially with subsequent switch to low-dose aspirin
        • continued if coronary artery abnormalities are present
        • discontinued only if imaging confirms no coronary artery abnormalities weeks after onset of Kawasaki disease
      • anticoagulation
        • indications for patients
          • at risk of thrombosis
          • with thrombocytosis
        • drugs
          • warfarin
      • vaccinations
        • indications
          • prevention of viral infections while on aspirin therapy
            • recall that viral infection with aspirin may cause Reye syndrome in children
  • Complications
    • Cardiac sequelae
      • coronary artery aneurysm in 25% of patients
        • may lead to death
      • myocarditis
      • myocardial infarction
  • Prognosis
    • Usually self-limited and resolves with treatment
    • 25% of patients have cardiac sequelae
      • may lead to fatal or nonfatal myocardial infarctions years after initial onset of disease
      • may lead to fatal rupture of aneurysm
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