Variation in Pharmacologic Management of Patients with Kawasaki Disease with Coronary Artery Aneurysms Academic Article uri icon

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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.


    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.


    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.


  • Selamet Tierney, Elif Seda
  • Runeckles, Kyle
  • Tremoulet, Adriana H
  • Dahdah, Nagib
  • Portman, Michael A
  • Mackie, Andrew S
  • Harahsheh, Ashraf S
  • Lang, Sean M
  • Choueiter, Nadine F
  • Li, Jennifer S
  • Manlhiot, Cedric
  • Low, Tisiana
  • Mathew, Mathew
  • Friedman, Kevin G
  • Raghuveer, Geetha
  • Norozi, Kambiz
  • Szmuszkovicz, Jacqueline R
  • McCrindle, Brian W
  • Altman, Carolyn A
  • Braunlin, Elizabeth
  • Burns, Jane C
  • Carr, Michael R
  • Colyer, Jessica H
  • Dallaire, Frederic
  • Dempsey, Adam
  • Desjardins, Laurent
  • Dillenburg, Rejane
  • Dionne, Audrey
  • Gewitz, Michael
  • Giglia, Therese M
  • Harris, Kevin C
  • Hill, Kevin D
  • Jain, Supriya
  • Jone, Pei-Ni
  • Kimball, Thomas R
  • Kutty, Shelby
  • Lai, Lillian
  • Lee, Simon
  • Lin, Ming-Tai
  • Mahle, William T
  • McHugh, Kimberly E
  • Mondal, Tapas
  • Newburger, Jane W
  • Renaud, Claudia
  • Sexson Tejitel, S Kristen
  • Texter, Karen M
  • Thacker, Deepika
  • Thomas, Thomas
  • Wagner-Lees, Sharon
  • Wong, Kenny K
  • Wu, Mei-Hwan
  • Yetman, Anji T
  • Baker, Annette L
  • Collins, Tanveer
  • De Ferranti, Sarah D
  • Fournier, Anne
  • Gould, Patrick
  • O’Shea, Sunita
  • Sable, Craig A
  • Sabouni, Sam
  • Tinker, Devin D

publication date

  • January 2022