Elexacaftor/Tazacaftor/Ivacaftor改善囊性纤维化患者磁共振成像检测到的支气管动脉扩张。

IF 6.8 2区 医学 Q1 RESPIRATORY SYSTEM
Lena Wucherpfennig, Simon M F Triphan, Sabine Wege, Hans-Ulrich Kauczor, Claus P Heussel, Olaf Sommerburg, Mirjam Stahl, Marcus A Mall, Monika Eichinger, Mark O Wielpütz
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引用次数: 0

摘要

理由:磁共振成像(MRI)可检测到接受依沙卡福/替扎卡福/依伐卡福(ETI)治疗的囊性纤维化(CF)患者粘液堵塞和支气管壁增厚的改善,但不能检测到肺灌注的改善。目的:确定支气管动脉扩张(BAD)(晚期肺部疾病的一个关键特征)是否表明灌注异常的不可逆性,以及在接受ETI治疗的CF患者中BAD是否可以逆转。方法:共有59名CF患者接受了胸部纵向MRI检查,包括两次磁共振血管造影术,其中包括35名CF患者(平均年龄31岁) ± 7. yr)之前(MRI1)和之后(MRI2)至少1 月(平均持续时间,8 ± 4. mo)和24名CF对照患者(平均年龄,31岁) ± 7. yr)而没有ETI。使用经验证的胸部MRI评分评估MRI,并使用商业软件评估BAD的存在和总管腔面积。结果:对照组MRI总体评分从MRI1到MRI2稳定(平均差异为1.1[-0.3,2.4];P = 0.054),但ETI组降低(-10.1[-0.3,2.4];P 2[-0.2,2.2];P = 0.099),但ETI组下降(-7.0 mm2[-8.9,-5.0];P r = 0.540;P 结论:我们的数据表明,在已经确定疾病的成年CF患者中,在ETI治疗下,BAD可能是部分可逆的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Elexacaftor/Tezacaftor/Ivacaftor Improves Bronchial Artery Dilatation Detected by Magnetic Resonance Imaging in Patients with Cystic Fibrosis.

Rationale: Magnetic resonance imaging (MRI) detects improvements in mucus plugging and bronchial wall thickening, but not in lung perfusion in patients with cystic fibrosis (CF) treated with elexacaftor/tezacaftor/ivacaftor (ETI). Objectives: To determine whether bronchial artery dilatation (BAD), a key feature of advanced lung disease, indicates irreversibility of perfusion abnormalities and whether BAD could be reversed in CF patients treated with ETI. Methods: A total of 59 adults with CF underwent longitudinal chest MRI, including magnetic resonance angiography twice, comprising 35 patients with CF (mean age, 31 ± 7 yr) before (MRI1) and after (MRI2) at least 1 month (mean duration, 8 ± 4 mo) on ETI therapy and 24 control patients with CF (mean age, 31 ± 7 yr) without ETI. MRI was assessed using the validated chest MRI score, and the presence and total lumen area of BAD were assessed with commercial software. Results: The MRI global score was stable in the control group from MRI1 to MRI2 (mean difference, 1.1 [-0.3, 2.4]; P = 0.054), but it was reduced in the ETI group (-10.1 [-0.3, 2.4]; P < 0.001). In the control and ETI groups, BAD was present in almost all patients at baseline (95% and 94%, respectively), which did not change at MRI2. The BAD total lumen area did not change in the control group from MRI1 to MRI2 (1.0 mm2 [-0.2, 2.2]; P = 0.099) but decreased in the ETI group (-7.0 mm2 [-8.9, -5.0]; P < 0.001). This decrease correlated with improvements in the MRI global score (r = 0.540; P < 0.001). Conclusions: Our data show that BAD may be partially reversible under ETI therapy in adult patients with CF who have established disease.

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来源期刊
Annals of the American Thoracic Society
Annals of the American Thoracic Society Medicine-Pulmonary and Respiratory Medicine
CiteScore
9.30
自引率
3.60%
发文量
0
期刊介绍: The Annals of the American Thoracic Society (AnnalsATS) is the official international online journal of the American Thoracic Society. Formerly known as PATS, it provides comprehensive and authoritative coverage of a wide range of topics in adult and pediatric pulmonary medicine, respiratory sleep medicine, and adult medical critical care. As a leading journal in its field, AnnalsATS offers up-to-date and reliable information that is directly applicable to clinical practice. It serves as a valuable resource for clinical specialists, supporting their formative and continuing education. Additionally, the journal is committed to promoting public health by publishing research and articles that contribute to the advancement of knowledge in these fields.
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