Variation in Pharmacologic Management of Patients with Kawasaki Disease with Coronary Artery Aneurysms

International Kawasaki Disease Registry

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Objective: To evaluate practice variation in pharmacologic management in the International Kawasaki Disease Registry (IKDR). Study design: Practice variation in intravenous immunoglobulin (IVIG) therapy, anti-inflammatory agents, statins, beta-blockers, antiplatelet therapy, and anticoagulation was described. Results: We included 1627 patients from 30 IKDR centers with maximum coronary artery aneurysm (CAA) z scores 2.5-4.99 in 848, 5.0-9.99 in 349, and ≥10.0 (large/giant) in 430 patients. All centers reported IVIG and acetylsalicylic acid (ASA) as primary therapy and use of additional IVIG or steroids as needed. In 23 out of 30 centers, (77%) infliximab was also used; 11 of these 23 centers reported using it in <10% of their patients, and 3 centers used it in >20% of patients. Nonsteroidal anti-inflammatory agents were used in >10% of patients in only nine centers. Beta-blocker (8.8%, all patients) and abciximab (3.6%, all patients) were mainly prescribed in patients with large/giant CAAs. Statins (2.7%, all patients) were mostly used in one center and only in patients with large/giant CAAs. ASA was the primary antiplatelet modality for 99% of patients, used in all centers. Clopidogrel (18%, all patients) was used in 24 centers, 11 of which used it in >50% of their patients with large/giant CAAs. Conclusions: In the IKDR, IVIG and ASA therapy as primary therapy is universal with common use of a second dose of IVIG for persistent fever. There is practice variation among centers for adjunctive therapies and anticoagulation strategies, likely reflecting ongoing knowledge gaps. Randomized controlled trials nested in a high-quality collaborative registry may be an efficient strategy to reduce practice variation.

Original languageEnglish (US)
Pages (from-to)164-170.e1
JournalJournal of Pediatrics
StatePublished - Jan 2022

Bibliographical note

Funding Information:
Funding for the data coordinating center was partially provided by the CIBC World Market Chair in Child Health Research (to B.M.) and the Labatt Family Heart Centre at SickKids Hospital (to B.M.). Additional local funding for participation in the IKDR was provided by the les Fonds BoBeau Coeur of the Ste-Justine Hospital Foundation (to N.D.), the Milken Family Foundation (to Jane Newburger), R01HL143130 from the National Institutes of Health (to M.P.). The other authors declare no conflicts of interest.

Publisher Copyright:
© 2021


  • Kawasaki
  • management
  • variation

PubMed: MeSH publication types

  • Evaluation Study
  • Journal Article
  • Multicenter Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't


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