CHU Bordeaux, Service d'Imagerie Thoracique et Cardiovasculaire, Service des Maladies Respiratoires, Service d'Exploration Fonctionnelle Respiratoire, Paediatric Cystic Fibrosis Reference Center (CRCM), Pessac, France.
Univ. Bordeaux, INSERM, Centre de Recherche Cardio-Thoracique de Bordeaux, Pessac, France.
J Magn Reson Imaging. 2024 Jul;60(1):116-124. doi: 10.1002/jmri.29041. Epub 2023 Oct 20.
Lung magnetic resonance imaging (MRI) with ultrashort echo-times (UTE-MRI) allows high-resolution and radiation-free imaging of the lung structure in cystic fibrosis (CF). In addition, the combination of elexacaftor/tezacaftor/ivacaftor (ETI) has improved CF clinical outcomes such as need for hospitalization. However, the effect on structural disease still needs longitudinal evaluation at high resolution.
To analyze the effects of ETI on lung structural alterations using UTE-MRI, with a focus on bronchiectasis reversibility.
Retrospective.
Fifty CF patients (mean age 24.3 ± 9.2; 23 males).
FIELD STRENGTH/SEQUENCE: 1.5 T, UTE-MRI.
All subjects completed both UTE-MRI and pulmonary function tests (PFTs) during two annual visits (M0 and M12), and 30 of them completed a CT scan. They initiated ETI treatment after M0 within a maximum of 3 months from the annual examinations. Three observers scored a clinical MRI Bhalla score on UTE-MRI. Bronchiectasis reversibility was defined as a reduction in both outer and inner bronchial dimensions. Correlations were searched between the Bhalla score and PFT such as the forced expiratory volume in 1 second percentage predicted (FEV1%p).
Comparison was assessed using the paired t-test, correlation using the Spearman correlation test with a significance level of 0.05. Concordance and reproducibility were assessed using intraclass correlation coefficient (ICC).
There was a significant improvement in MRI Bhalla score after ETI treatment. UTE-MRI demonstrated bronchiectasis reversibility in a subgroup of 18 out of 50 CF patients (36%). These patients with bronchiectasis reversibility were significantly younger, with lower severity of wall thickening but no difference in mucus plugging extent (P = 0.39) was found. The reproducibility of UTE-MRI evaluations was excellent (ICC ≥ 0.95), was concordant with CT scan (N = 30; ICC ≥ 0.90) and significantly correlated to FEV1% at PFT at M0 (N = 50; r = 0.71) and M12 (N = 50; r = 0.72).
UTE-MRI is a reproducible tool for the longitudinal follow-up of CF patients, allowing to quantify the response to ETI and demonstrating the reversibility of some structural alterations such as bronchiectasis in a substantial fraction of this study population.
4 TECHNICAL EFFICACY: Stage 2.
超短回波时间（UTE）磁共振成像（MRI）可实现肺部结构的高分辨率、无辐射成像，在囊性纤维化（CF）中具有重要应用。此外，三联药物（elexacaftor/tezacaftor/ivacaftor，ETI）的应用改善了 CF 患者的临床结局，例如减少住院需求。然而，其对结构性疾病的影响仍需要进行高分辨率的纵向评估。
采用 UTE-MRI 分析 ETI 对肺部结构改变的影响，重点关注支气管扩张的可逆转性。
回顾性研究。
50 例 CF 患者（平均年龄 24.3±9.2 岁，23 名男性）。
磁场强度/序列：1.5T，UTE-MRI。
所有患者在两次年度随访（M0 和 M12）期间均完成 UTE-MRI 和肺功能检查（PFT），其中 30 例患者完成 CT 扫描。他们在 M0 后开始 ETI 治疗，从年度检查到开始治疗的时间最长不超过 3 个月。三位观察者对 UTE-MRI 的临床 Bhalla 评分进行评分。支气管扩张的可逆转性定义为外、内径均减小。对 Bhalla 评分与 PFT （如 1 秒用力呼气容积占预计值的百分比（FEV1%p））之间的相关性进行搜索。
采用配对 t 检验进行比较，采用 Spearman 相关检验进行相关性分析，显著性水平为 0.05。采用组内相关系数（ICC）评估一致性和可重复性。
ETI 治疗后 MRI Bhalla 评分显著改善。在 50 例 CF 患者中，有 18 例（36%）患者的 UTE-MRI 显示支气管扩张可逆转。这些支气管扩张可逆转的患者年龄更小，管壁增厚程度更轻，但黏液栓的程度无差异（P=0.39）。UTE-MRI 评估的可重复性非常好（ICC≥0.95），与 CT 扫描一致（N=30；ICC≥0.90），与 M0（N=50；r=0.71）和 M12（N=50；r=0.72）时的 PFT FEV1%显著相关。
UTE-MRI 是 CF 患者纵向随访的一种可靠工具，可定量评估 ETI 治疗的效果，并在研究人群的很大一部分中显示出一些结构性改变（如支气管扩张）的可逆转性。
4 级 技术效能：2 级
