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JAMA Network Clinical Guideline Synopsis
January 22, 2019

Diagnosis and Management of Kawasaki Disease

Author Affiliations
  • 1Division of Hospital Medicine, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
  • 2Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio
  • 3The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
JAMA Pediatr. 2019;173(3):278-279. doi:10.1001/jamapediatrics.2018.3307

Kawasaki disease (KD) is an acute, self-limited vasculitis of medium-sized arteries that leads to coronary artery aneurysms in approximately 25% of untreated patients. Timely treatment with intravenous immunoglobulin (IVIG) has decreased this risk to 3% to 5%. Incomplete KD is a risk factor for delayed diagnosis and treatment, thus increasing the risk for coronary artery abnormalities (CAAs). The American Heart Association (AHA) guideline provides a diagnostic algorithm for suspected cases of incomplete KD, which has retrospectively been shown to hasten treatment.1,2 There remains a subset of children, particularly infants younger than 6 months and adolescents, in whom the diagnosis is exceedingly challenging. Once the diagnosis is made, the treatment of complete and incomplete KD is identical. Intravenous immunoglobulin and acetylsalicylic acid are the hallmarks of therapy. The use of adjunctive agents and strategies for treatment of IVIG-resistant disease remains controversial.1

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