Definition
• Syndrome of fever, conjunctivitis, lymphadenopathy, and exanthem, which can cause necrotizing coronary arteritis (also called mucocutaneous lymph node syndrome or infantile polyarteritis nodosa)
Clinical features
Epidemiology
• Affects up to 120 to 150 per 100,000 children younger than 5 years of age, depending on the geographic location
• Slight male predominance (male:female ratio, approximately 1.5:1)
• Affects infants and children, originally described in Asia but now increasingly recognized worldwide
Presentation
• Classic presentation: fever; exanthema; conjunctivitis; red tongue, lips, oral mucosa; cervical lymphadenopathy
• Diagnosis requires the presence of fever lasting at least 5 days and fulfilling four or more of the following diagnostic criteria (from Dajani AS, Taubert KA, Gerber MA, et al: The American Heart Association guidelines: diagnosis and therapy of Kawasaki disease in children, Circulation 87:1776-1780, 1993):
• Changes (swelling, redness) in the extremities
• Polymorphous exanthem
• Bilateral conjunctival injection
• Changes in lips and oral mucosa
• Cervical lymphadenopathy
• Other diseases with similar clinical findings must be excluded
• Patients with fever and fewer than four of the previously mentioned diagnostic criteria can be diagnosed with Kawasaki disease if coronary artery disease is demonstrated by two-dimensional echocardiography or coronary angiography
Prognosis and treatment
• Coronary arterial involvement results in 1% mortality (leading cause of acquired heart disease in the pediatric population)
• Intravenous immunoglobulin (IVIG) plus aspirin is a mainstay of therapy
• Other therapeutic options include: IVIG, high-dose aspirin, intravenous methylprednisolone, or infliximab (Remicade)
• Patients with coronary disease should receive aspirin, clopidogrel (Plavix), warfarin, or low molecular weight heparin
• Acute coronary thrombosis requires thrombolytic treatment with surgical or percutaneous intervention
Pathology
Gross
• Prominent coronary arteries with fusiform aneurysmal dilatation
• Markedly thickened coronary arterial walls on cross section
Histology
• Necrotizing polyarteritis, including involvement of coronary arteries, which leads to coronary aneurysm
• Myocardial infarction secondary to coronary involvement
Immunopathology/special stains
• Not contributory
Main differential diagnoses
• The clinical differential diagnosis includes
• Other viral exanthems
• Staphylococcal scalded-skin syndrome
• Toxic shock syndrome
• Scarlet fever
• Stevens-Johnson syndrome
• Drug reaction
• Measles
• Rocky Mountain spotted fever
• Juvenile rheumatoid arthritis
• Leptospirosis
• Mercury poisoning
• The pathological finding of coronary arteritis in a pediatric patient is diagnostic in the appropriate clinical context
Fig 1 Kawasaki disease. Gross photograph demonstrating aneurysmal dilatation of the coronary arteries in a patient with Kawasaki disease.
(Courtesy of Jerome Taxy, MD, University of Chicago, Ill.)
Fig 2 Kawasaki disease. Cross sections demonstrate marked thickening of the coronary arterial wall.
(Courtesy of Jerome Taxy, MD, University of Chicago, Ill.)