Institute of Diagnostic and Interventional Radiology, Hannover Medical School, Hannover, Germany.
Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research (DZL), Hannover, Germany.
Eur Radiol. 2024 Jan;34(1):80-89. doi: 10.1007/s00330-023-09912-6. Epub 2023 Aug 7.
To investigate whether 3D phase-resolved functional lung (PREFUL)-MRI parameters are suitable to measure response to elexacaftor/tezacaftor/ivacaftor (ETI) therapy and their association with clinical outcomes in cystic fibrosis (CF) patients.
Twenty-three patients with CF (mean age: 21; age range: 14-46) underwent MRI examination at baseline and 8-16 weeks after initiation of ETI. Morphological and 3D PREFUL scans assessed pulmonary ventilation. Morphological images were evaluated using a semi-quantitative scoring system, and 3D PREFUL scans were evaluated by ventilation defect percentage (VDP) values derived from regional ventilation (RVent) and cross-correlation maps. Improved ventilation volume (IVV) normalized to body surface area (BSA) between baseline and post-treatment visit was computed. Forced expiratory volume in 1 second (FEV) and mid-expiratory flow at 25% of forced vital capacity (MEF25), as well as lung clearance index (LCI), were assessed. Treatment effects were analyzed using paired Wilcoxon signed-rank tests. Treatment changes and post-treatment agreement between 3D PREFUL and clinical parameters were evaluated by Spearman's correlation.
After ETI therapy, all 3D PREFUL ventilation markers (all p < 0.0056) improved significantly, except for the mean RVent parameter. The BSA normalized IVV was significantly correlated to relative treatment changes of MEF25 and mucus plugging score (all |r| > 0.48, all p < 0.0219). In post-treatment analyses, 3D PREFUL VDP values significantly correlated with spirometry, LCI, MRI global, morphology, and perfusion scores (all |r| > 0.44, all p < 0.0348).
3D PREFUL MRI is a very promising tool to monitor CFTR modulator-induced regional dynamic ventilation changes in CF patients.
3D PREFUL MRI is sensitive to monitor CFTR modulator-induced regional ventilation changes in CF patients. Improved ventilation volume correlates with the relative change of mucus plugging, suggesting that reduced endobronchial mucus is predominantly responsible for regional ventilation improvement.
• 3D PREFUL MRI-derived ventilation maps show significantly reduced ventilation defects in CF patients after ETI therapy. • Significant post-treatment correlations of 3D PREFUL ventilation measures especially with LCI, FEV %pred, and global MRI score suggest that 3D PREFUL MRI is sensitive to measure improved regional ventilation of the lung parenchyma due to reduced inflammation induced by ETI therapy in CF patients. • 3D PREFUL MRI-derived improved ventilation volume (IVV) correlated with MRI mucus plugging score changes suggesting that reduced endobronchial mucus is predominantly responsible for regional ventilation improvement 8-16 weeks after ETI therapy.
研究 3D 相位分辨功能肺（PREFUL）-MRI 参数是否适合测量囊性纤维化（CF）患者对依伐卡托/泰他卡托/维利卡托（ETI）治疗的反应及其与临床结局的关系。
23 例 CF 患者（平均年龄：21 岁；年龄范围：14-46 岁）在 ETI 治疗开始前和 8-16 周后进行 MRI 检查。形态学和 3D PREFUL 扫描评估肺通气。形态学图像采用半定量评分系统进行评估，3D PREFUL 扫描通过区域通气（RVent）和互相关图得出的通气缺陷百分比（VDP）值进行评估。计算治疗前后以体表面积（BSA）标准化的通气体积改善（IVV）。用力呼气量（FEV）和用力肺活量 25%时的中间流量（MEF25），以及肺清除指数（LCI）也进行了评估。采用配对 Wilcoxon 符号秩检验分析治疗效果。采用 Spearman 相关分析评估 3D PREFUL 和临床参数之间的治疗变化和治疗后一致性。
ETI 治疗后，除平均 Rvent 参数外，所有 3D PREFUL 通气标志物均显著改善（均 p < 0.0056）。以 BSA 标准化的 IVV 与 MEF25 和黏液栓子评分的相对治疗变化显著相关（均 |r| > 0.48，均 p < 0.0219）。在治疗后分析中，3D PREFUL VDP 值与肺功能、LCI、MRI 整体、形态和灌注评分显著相关（均 |r| > 0.44，均 p < 0.0348）。
3D PREFUL MRI 是一种很有前途的工具，可监测 CFTR 调节剂诱导的 CF 患者的区域性动态通气变化。
3D PREFUL MRI 可敏感监测 CFTR 调节剂诱导的 CF 患者的区域性通气变化。改善的通气量与黏液栓子的相对变化相关，提示减少支气管内黏液主要负责改善区域性通气。
· ETI 治疗后，3D PREFUL MRI 衍生的通气图显示 CF 患者的通气缺陷明显减少。
· 3D PREFUL 通气测量的显著治疗后相关性，特别是与 LCI、FEV%pred 和整体 MRI 评分相关，提示 3D PREFUL MRI 敏感，可测量 CF 患者由于 ETI 治疗减轻炎症引起的肺实质区域通气改善。
· 3D PREFUL MRI 衍生的改善通气量（IVV）与 MRI 黏液栓子评分变化相关，提示 ETI 治疗后 8-16 周支气管内黏液减少主要负责改善区域性通气。
