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接受依列卡福妥/替扎卡福妥/依伐卡托治疗的成年囊性纤维化患者肺部疾病的计算机断层扫描研究进展

Evolution of Lung Disease Studied by Computed Tomography in Adults with Cystic Fibrosis Treated with Elexacaftor/Tezacaftor/Ivacaftor.

作者信息

Hernández-Muñiz Susana, Caballero Paloma, Peláez Adrián, Solís-García Marta, de Benavides Carmen, Collada Javier, Díaz-Lorenzo Ignacio, Zorzo Cristina, Gómez-Punter Rosa Mar, Girón Rosa María

机构信息

Radiology Department, University Hospital La Princesa, Calle Diego de Leon nº 62, 28006 Madrid, Spain.

Medicine Department, Universidad Autónoma, 28049 Madrid, Spain.

出版信息

J Imaging. 2025 Apr 21;11(4):124. doi: 10.3390/jimaging11040124.

Abstract

Elexacaftor-tezacaftor-ivacaftor (ETI) has shown clinical and spirometric benefits in cystic fibrosis (CF). CT remains a vital tool for diagnosing and monitoring structural lung disease. This study aimed to assess the evolution of lung disease, as evaluated through CT, in adults with CF after at least one year of ETI treatment. This ambispective observational analysis assessed lung CT scans performed before initiating ETI and after at least one year of treatment, using the modified Bhalla scoring system. For those patients with an earlier CT scan, a pre-treatment phase analysis was performed. Epidemiological, clinical, and functional parameters were evaluated. Results: Sixty-two patients were included (35 males, median age 30.4 ± 7.87 years). After at least one year of ETI, significant improvements were observed in the global CT Bhalla score (12.2 ± 2.8 vs. 14.0 ± 2.8), peribronchial thickening (1.4 ± 0.6 vs. 1.0 ± 0.4), and mucus plugging (1.6 ± 0.7 vs. 0.8 ± 0.6) ( < 0.001). Spirometry parameters increased significantly: the percentage of the predicted forced expiratory volume in the first second (ppFEV) increased from 66.5 ± 19.8 to 77.0 ± 20.4 ( = 0.005) and forced vital capacity (ppFVC) from 80.6 ± 16.4 to 91.6 ± 14.1 ( < 0.001). Additionally, body mass index showed a significant increase. A moderate correlation was found between the Bhalla score and spirometry results. In the pre-treatment phase ( = 52), mucus plugging demonstrated a significant worsening, whereas global CT score, other subscores, and spirometry did not change significantly. Conclusions: In adults with CF, after at least one year of ETI, a significant improvement in structural lung disease was achieved, as reflected by the CT Bhalla score.

摘要

依列卡福妥-替扎卡福妥-依伐卡托(ETI)已在囊性纤维化(CF)患者中显示出临床和肺功能测定方面的益处。CT仍然是诊断和监测肺部结构性疾病的重要工具。本研究旨在评估接受ETI治疗至少一年的成年CF患者通过CT评估的肺部疾病进展情况。这项双向观察性分析使用改良的巴拉评分系统,对开始ETI治疗前和治疗至少一年后的肺部CT扫描进行了评估。对于那些有更早CT扫描的患者,进行了治疗前阶段分析。评估了流行病学、临床和功能参数。结果:纳入62例患者(35例男性,中位年龄30.4±7.87岁)。接受ETI治疗至少一年后,全球CT巴拉评分(12.2±2.8对14.0±2.8)、支气管周围增厚(1.4±0.6对1.0±0.4)和黏液嵌塞(1.6±0.7对0.8±0.6)均有显著改善(<0.001)。肺功能测定参数显著增加:第一秒用力呼气容积预测值百分比(ppFEV)从66.5±19.8增加到77.0±20.4(=0.005),用力肺活量(ppFVC)从80.6±16.4增加到91.6±14.1(<0.001)。此外,体重指数显著增加。巴拉评分与肺功能测定结果之间存在中度相关性。在治疗前阶段(=52),黏液嵌塞有显著恶化,而全球CT评分、其他子评分和肺功能测定无显著变化。结论:在成年CF患者中,接受ETI治疗至少一年后,CT巴拉评分反映出肺部结构性疾病有显著改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7b78/12028220/c593fe57a53e/jimaging-11-00124-g001.jpg

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