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.

中文翻译：

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川崎病合并冠状动脉瘤患者的药物治疗变化

客观的

评估国际川崎病登记处 (IKDR) 中药物管理的实践差异。

学习规划

描述了静脉内免疫球蛋白 (IVIG) 治疗、抗炎药、他汀类药物、β 受体阻滞剂、抗血小板治疗和抗凝剂的实践变化。

结果

我们纳入了来自 30 个 IKDR 中心的 1627 名患者，其中 848 名患者的最大冠状动脉瘤 (CAA) z 评分为 2.5-4.99，349 名患者为 5.0-9.99，430 名患者中≥10.0（大型/巨型）。所有中心均报告 IVIG 和乙酰水杨酸 (ASA) 作为主要治疗，并根据需要使用额外的 IVIG 或类固醇。30 个中心中有 23 个（77%）也使用了英夫利昔单抗；这 23 个中心中有 11 个报告在其患者中使用它的比例不到 10%，而 3 个中心在超过 20% 的患者中使用它。只有 9 个中心超过 10% 的患者使用了非甾体抗炎药。β-受体阻滞剂（8.8%，所有患者）和阿昔单抗（3.6%，所有患者）主要用于大型/巨大 CAA 患者。他汀类药物（2.7%，所有患者）主要在一个中心使用，并且仅用于大型/巨大 CAA 患者。ASA 是 99% 患者的主要抗血小板方法，在所有中心都使用。氯吡格雷（18%，所有患者）在 24 个中心使用，其中 11 个中心在超过 50% 的大型/巨大 CAA 患者中使用氯吡格雷。

结论

在 IKDR 中，IVIG 和 ASA 治疗作为主要治疗是普遍的，通常使用第二剂 IVIG 治疗持续发热。辅助治疗和抗凝策略中心之间存在实践差异，可能反映了持续的知识差距。嵌套在高质量协作注册表中的随机对照试验可能是减少实践差异的有效策略。