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

Journal Article (Journal Article;Multicenter Study)

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.

Full Text

Duke Authors

Cited Authors

  • Selamet Tierney, ES; Runeckles, K; Tremoulet, AH; Dahdah, N; Portman, MA; Mackie, AS; Harahsheh, AS; Lang, SM; Choueiter, NF; Li, JS; Manlhiot, C; Low, T; Mathew, M; Friedman, KG; Raghuveer, G; Norozi, K; Szmuszkovicz, JR; McCrindle, BW; International Kawasaki Disease Registry,

Published Date

  • January 2022

Published In

Volume / Issue

  • 240 /

Start / End Page

  • 164 - 170.e1

PubMed ID

  • 34474088

Electronic International Standard Serial Number (EISSN)

  • 1097-6833

Digital Object Identifier (DOI)

  • 10.1016/j.jpeds.2021.08.072


  • eng

Conference Location

  • United States