Imaging Department, Hopital Nord, APHM, Aix Marseille University, France.
Service de Pneumologie et équipe de transplantation pulmonaire, Centre de Ressources et de Compétences de la Mucoviscidose (CRCM) Adulte, AP-HM Hôpital Nord, 13015 Marseille, France; Aix Marseille Université, Marseille, France.
Eur J Radiol. 2022 Sep;154:110421. doi: 10.1016/j.ejrad.2022.110421. Epub 2022 Jun 23.
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have revolutionised the treatment of cystic fibrosis (CF). Chest computed tomography (CT) is key in the diagnosis and follow-up of anatomical damage to the lungs. Our study aimed to evaluate changes on lung CT scans of patients with CF after receiving elexacaftor-tezacaftor-ivacaftor (ETI) therapy for one year.
We conducted a retrospective, observational, single-centre study between 2018 and 2021 on adult patients with CF administered ETI. We reviewed chest CT scans before and at least one year after starting ETI. The Brody-II score (BSII) was measured by two experienced radiologists who were blinded to the treatment. Paranasal sinus CT scans and clinical and functional data were also compared. Wilcoxon tests were used to compare differences, and Spearman's correlation coefficient was used to evaluate changes in forced expiratory volume in one second (FEV) and total BSII.
In the period, 63 patients were given ETI, and 12 met the criteria for analysis. The inter-observer reproducibility of BSII was satisfactory (intraclass correlation coefficient = 0.83, 95% confidence interval 0.57-0.91). The BSII decreased after one year of treatment (-18 ± 16, p = 0.002) due to lower mucous plugging (-7 ± 4, p < 0.001) and peribronchial thickening (-9 ± 10, p = 0.002) scores. Bronchial, parenchymal, and hyperinflation scores were unchanged. Clinical and functional parameters were significantly improved, except for total lung capacity. The correlation between ΔFEV and Δtotal BSII was strong (r = 0.88, p < 0.001). The paranasal sinus CT score significantly improved with ETI treatment.
ETI decreased pulmonary and sinus morphological abnormalities after one year of treatment.
囊性纤维化跨膜电导调节因子（CFTR）调节剂彻底改变了囊性纤维化（CF）的治疗方法。胸部计算机断层扫描（CT）是诊断和随访肺部解剖结构损伤的关键。我们的研究旨在评估接受依伐卡托-泰他卡托-维兰特罗（ETI）治疗一年后的 CF 患者的肺部 CT 扫描结果的变化。
我们进行了一项回顾性、观察性、单中心研究，纳入了 2018 年至 2021 年间接受 ETI 治疗的成年 CF 患者。我们评估了开始 ETI 治疗前和至少一年后的胸部 CT 扫描。Brody-II 评分（BSII）由两位经验丰富的放射科医生测量，他们对治疗方案不知情。还比较了副鼻窦 CT 扫描和临床及功能数据。采用 Wilcoxon 检验比较差异，Spearman 相关系数评估用力呼气量（FEV）和总 BSII 的变化。
在此期间，有 63 名患者接受了 ETI 治疗，其中 12 名符合分析标准。BSII 的两位观察者之间的可重复性令人满意（组内相关系数为 0.83，95%置信区间为 0.57-0.91）。治疗一年后，BSII 下降（-18±16，p=0.002），这是由于黏液栓形成减少（-7±4，p<0.001）和支气管周围增厚（-9±10，p=0.002）评分降低所致。支气管、肺实质和过度充气评分无变化。除了肺活量外，临床和功能参数均显著改善。ΔFEV 和Δ总 BSII 之间具有较强的相关性（r=0.88，p<0.001）。ETI 治疗后，副鼻窦 CT 评分显著改善。
ETI 治疗一年后可减少肺部和鼻窦形态学异常。
