Updated: 11/19/2020

Kawasaki Syndrome

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Snapshot
  • PhotoA 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
  • 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
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
  • 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
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
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

 

 

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