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

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) (<i>p</i> < 0.001). Spirometry parameters increased significantly: the percentage of the predicted forced expiratory volume in the first second (ppFEV<sub>1</sub>) increased from 66.5 ± 19.8 to 77.0 ± 20.4 (<i>p</i> = 0.005) and forced vital capacity (ppFVC) from 80.6 ± 16.4 to 91.6 ± 14.1 (<i>p</i> < 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 (<i>n</i> = 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.

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Journal
Journal of Imaging
Published
2025-04-21
DOI
https://doi.org/10.3390/jimaging11040124
Citations
1
Primary Topic
Cystic Fibrosis Research Advances
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article
Field-Weighted Citation Impact
1.52
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article

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

Cristina Zorzo, J. C. García Collada, Marta Solís-García, Ignacio Lorenzo et al.
1 citations
Journal of Imaging
Cystic Fibrosis Research Advances
1.52
article

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

Cristina Zorzo, J. C. García Collada, Marta Solís-García, Ignacio Lorenzo, Rosa Mar Gómez-Punter, Adrián Peláez, Rosa Girón, Paloma Caballero, Carmen de Benavides, Susana Hernández-Muñíz
article en
1 citations

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) (<i>p</i> < 0.001). Spirometry parameters increased significantly: the percentage of the predicted forced expiratory volume in the first second (ppFEV<sub>1</sub>) increased from 66.5 ± 19.8 to 77.0 ± 20.4 (<i>p</i> = 0.005) and forced vital capacity (ppFVC) from 80.6 ± 16.4 to 91.6 ± 14.1 (<i>p</i> < 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 (<i>n</i> = 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.

Journal of ImagingVol. 11(4)
Spanish National Centre for Cardiovascular Research (ES), Hospital Universitario de La Princesa (ES), Hospital General de La Rioja (ES), Universidad Autónoma de Madrid (ES)
Good health and well-being
Openalex Percentile: Top 20%
Cystic Fibrosis Research Advances
1.52
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