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

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) (p < 0.001). Spirometry parameters increased significantly: the percentage of the predicted forced expiratory volume in the first second (ppFEV(1)) increased from 66.5 +/- 19.8 to 77.0 +/- 20.4 (p = 0.005) and forced vital capacity (ppFVC) from 80.6 +/- 16.4 to 91.6 +/- 14.1 (p < 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 (n = 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. (Summary); Hernández-Muñiz, S (corresponding author), Univ Hosp La Princesa, Radiol Dept, Calle Diego Leon 62, Madrid 28006, Spain. (Author of contact); Hernández-Muñíz, Susana, Department of Radiology, Hospital Universitario de la Princesa, Madrid, Madrid, Spain; Caballero, Paloma, Department of Radiology, Hospital Universitario de la Princesa, Madrid, Madrid, Spain, Department of Medicine, Universidad Autónoma de Madrid, Madrid, Madrid, Spain; Peláez Laderas, Adrián, Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Madrid, Spain; Solís-García, Marta, Pneumonology Department, Hospital Universitario de la Princesa, Madrid, Madrid, Spain; de Benavides, Carmen, Department of Radiology, Hospital Universitario de la Princesa, Madrid, Madrid, Spain; Collada, Javier, Department of Radiology, Hospital Universitario de la Princesa, Madrid, Madrid, Spain; Díaz-Lorenzo, Ignacio, Department of Radiology, Hospital Universitario de la Princesa, Madrid, Madrid, Spain; Zorzo, Cristina, Department of Radiology, Hospital Universitario Sanitas La Moraleja, Madrid, Madrid, Spain; Gómez-Púnter, Rosa Mar, Pneumonology Department, Hospital Universitario de la Princesa, Madrid, Madrid, Spain; Girón-Moreno, Rosa María, Pneumonology Department, Hospital Universitario de la Princesa, Madrid, Madrid, Spain (Authors with Filk)