This study sought to evaluate the impact of elexacaftor/tezacaftor/ivacaftor (ETI) on lung structural abnormalities in adults with cystic fibrosis (awCF) with a specific focus on the reversal of bronchial dilatations.

Chest computed tomography (CT) scans performed prior to and 12 months after initiation of ETI were visually reviewed for possible reversal of bronchial dilatations. AwCF with and without reversal of bronchial dilatations (the latter served as controls, with three controls per case) were selected. Visual Brody score, bronchial and arterial diameters, and lung volume were measured on CT.

Reversal of bronchial dilatations was found in 12/235 (5%) awCF treated with ETI. 12 awCF with and 36 without reversal of bronchial dilatations were further analysed (male 56%, mean±sd age 31.6±8.5 years, F508del/F508del CFTR 54% and mean forced expiratory volume in 1 s 58.8±22.3% predicted). The Brody score improved overall from 79.4±29.8 to 54.8±32.3 (p<0.001). Reversal of bronchial dilatations was confirmed by a decrease in bronchial lumen diameter in cases from 3.9±0.9 to 3.2±1.1 mm (p<0.001), whereas it increased in awCF without reversal of bronchial dilatations (from 3.5±1.1 to 3.6±1.2 mm; p=0.002). Reversal of bronchial dilatations occurred in cylindrical (not varicose or saccular) bronchial dilatations. Lung volumes decreased by –6.6±10.7% in awCF with reversal of bronchial dilatations but increased by +2.3±9.6% in controls (p=0.007).

Although bronchial dilatations are generally considered irreversible, ETI was associated with reversal, which was limited to the cylindrical bronchial dilatation subtype, and occurred in a small subset of awCF. Initiating ETI earlier in life may reverse early bronchial dilatations.

本研究旨在评估 elexacaftor/tezacaftor/ivacaftor (ETI) 对成人囊性纤维化 (awCF) 肺结构异常的影响，特别关注逆转支气管扩张。

在开始 ETI 之前和之后 12 个月进行胸部计算机断层扫描 (CT) 扫描，以目视检查支气管扩张可能逆转的情况。选择有或没有支气管扩张逆转的 AwCF（后者作为对照，每个病例三个对照）。通过 CT 测量视觉 Brody 评分、支气管和动脉直径以及肺体积。

在用 ETI 治疗的 12/235 (5%) awCF 中发现支气管扩张逆转。进一步分析了 12 名有支气管扩张逆转的 awCF 和 36 名没有逆转支气管扩张的 awCF（男性 56%，平均值±标准差年龄 31.6±8.5 岁，F508del/F508del CFTR 54%，1 秒内平均用力呼气量 58.8±22.3%（预测值）。 Brody 评分总体从 79.4±29.8 提高到 54.8±32.3 (p<0.001)。支气管扩张的逆转通过病例中支气管管腔直径从 3.9±0.9 减小到 3.2±1.1 mm (p<0.001) 来证实，而 awCF 中支气管管腔直径增加而支气管扩张没有逆转 (从 3.5±1.1 到 3.6±1.2 mm) ；p=0.002)。支气管扩张的逆转发生在圆柱形（非曲张性或囊状）支气管扩张中。随着支气管扩张的逆转，awCF 中的肺体积减少了 –6.6±10.7%，但对照组则增加了 +2.3±9.6% (p=0.007)。

尽管支气管扩张通常被认为是不可逆的，但 ETI 与逆转相关，这种逆转仅限于圆柱形支气管扩张亚型，并且发生在 awCF 的一小部分中。尽早开始 ETI 可能会逆转早期支气管扩张。