Changes in chronic therapies for cystic fibrosis following initiation of CFTR modulators: A UK retrospective cohort study 2009 - 2023

IF 5.5 2区医学 Q1 RESPIRATORY SYSTEM

Journal of Cystic Fibrosis Pub Date : 2026-09-01 Epub Date: 2026-06-23 DOI:10.1016/j.jcf.2026.06.003

Jordan Busby, Tricia M McKeever, Andrew W Fogarty, Helen L Barr

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引用次数: 0

Abstract

Introduction

People with Cystic Fibrosis have a significant treatment burden from large numbers of maintenance therapies required to manage this multi-organ condition. Currently, there are limited data on the impact of CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) modulator use, including Elexacaftor/Tezacaftor/Ivacaftor (ETI), on prescribing of traditional maintenance therapies in the UK.

Methods

Retrospective analysis of chronic medications obtained from UK CF registry data, from 2009 to 2023. Impact of starting ETI on maintenance therapies was analysed using descriptive statistics. For those on ETI, risk factors associated with stopping maintenance treatments were identified by logistic regression models, adjusted for age, sex, and percentage predicted FEV₁. Socioeconomic status (SES) was determined by Townsend score quintiles.

Results

Overall, chronic medication use decreased for both those prescribed and not prescribed CFTR modulators. Those on ETI, who previously were on dual modulator therapy, had an absolute reduction of 15.1% in inhaled antibiotics, 14.7% in dornase alfa and 9.8% in hypertonic saline by 3 years after ETI initiation. Older age, higher body mass index, and poorer lung function were associated with lower odds of stopping inhaled antibiotics and azithromycin for those on ETI. Those in the lowest SES quintile had significantly lower odds of discontinuing azithromycin (OR:0.61; CI:0.40–0.91) and flucloxacillin (OR:0.54; CI:0.33–0.86), compared to the highest SES quintile.

Conclusions

Overall, prescriptions of traditional maintenance therapies reduced, irrespective of CFTR modulator use. These data suggest deprescribing may have occurred, despite a limited evidence base to support safe antibiotic withdrawal in this population.

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开始CFTR调节剂后囊性纤维化慢性治疗的变化：2009 - 2023年英国回顾性队列研究

简介：囊性纤维化患者需要大量的维持治疗来管理这种多器官疾病，这给他们带来了巨大的治疗负担。目前，关于使用CFTR（囊性纤维化跨膜传导调节剂）调节剂（包括Elexacaftor/Tezacaftor/Ivacaftor (ETI)）对英国传统维持疗法处方的影响的数据有限。方法：回顾性分析2009年至2023年英国CF登记数据中获得的慢性药物。采用描述性统计分析开始ETI对维持治疗的影响。对于接受ETI治疗的患者，通过逻辑回归模型确定与停止维持治疗相关的危险因素，并根据年龄、性别和预测FEV1百分比进行调整。社会经济地位（SES）由Townsend分数五分位数确定。结果：总体而言，处方CFTR调节剂和未处方CFTR调节剂的慢性药物使用均有所减少。那些接受ETI治疗的患者，先前接受双调节剂治疗，在ETI开始3年后，吸入抗生素的绝对减少15.1%，dornase α - fa的绝对减少14.7%，高渗盐水的绝对减少9.8%。年龄较大、身体质量指数较高和肺功能较差与ETI患者停止吸入抗生素和阿奇霉素的几率较低相关。与社会经济地位最高的五分位数相比，社会经济地位最低的五分位数患者停用阿奇霉素（OR:0.61; CI:0.40-0.91）和氟氯西林（OR:0.54; CI:0.33-0.86）的几率明显较低。结论：总体而言，与CFTR调节剂的使用无关，传统维持疗法的处方减少了。这些数据表明，尽管在这一人群中支持安全停用抗生素的证据基础有限，但处方可能已经减少。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Cystic Fibrosis 医学-呼吸系统

CiteScore

10.10

自引率

13.50%

发文量

1361

审稿时长

50 days

期刊介绍： The Journal of Cystic Fibrosis is the official journal of the European Cystic Fibrosis Society. The journal is devoted to promoting the research and treatment of cystic fibrosis. To this end the journal publishes original scientific articles, editorials, case reports, short communications and other information relevant to cystic fibrosis. The journal also publishes news and articles concerning the activities and policies of the ECFS as well as those of other societies related the ECFS.