With Kevin S. Roman

Definition and Classification

• Also known as mucocutaneous lymph node syndrome
  
• Acute febrile illness associated with a generalized vasculitis in infants and young children
  
• Leading cause of acquired heart disease in children in developed world
  
• Disease course divided into three phases:
  
  
  
  • • Acute stage: 1 to 2 weeks
    
    
    
    • – Acute febrile period
      
    • – Pancarditis with pericarditis, myocarditis, valvulitis, and coronary vasculitis
    
    
  • • Subacute stage: 2 to 4 weeks
    
    
    
    • – Begins with elevation of platelet count and ends with a return to nearly normal
      
    • – Coronary artery aneurysms reach peak
    
    
  • • Convalescent or chronic phase: months to possibly years
    
    
    
    • – Abnormalities of coronary arteries most apparent
      
    • – Expansion of giant aneurysms, smaller aneurysms tend to resolve, possible myocardial infarction

Pathophysiology