A new study has found improvements on chest computed tomography (CT) scans of patients with cystic fibrosis (CF) after 1 year of treatment with elexacaftor-tezacaftor-ivacaftor (ETI).

The study, published in the European Journal of Radiology, provides further support for using cystic fibrosis transmembrane conductance regulator (CFTR) modulators as therapy for CF.

“Changes observed on chest CT in patients receiving previously available pharmacological therapies have been explored, and the mucous plugging and bronchial thickening scores seem to improve with ivacaftor and the combination of lumacaftor and ivacaftor,” the authors wrote. “Although improvement is also expected with ETI therapy, the effect of ETI on pulmonary and sinus morphological changes has not yet been evaluated on CT for over 1 year.”

The team conducted a retrospective, observational, noninterventional, single-center study on 12 adult patients with severe CF in France who were administered oral ETI therapy between 2018 and 2021. Chest CT scans were reviewed before starting ETI and then at least 1 year later.

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Mucus plugging and peribronchial thickening scores declined by 50%, a finding not seen previously to the best of the authors’ knowledge. Prior studies on the combination of lumacaftor and ivacaftor showed a 29% reduction in peribronchial thickening.

ETI therapy also significantly improved the visual Brody-II score after 1 year, achieving a reduction of 18 points, or 21%, and sinus obstruction was also improved during this period. However, bronchiectasis, air trapping, and parenchymal lesions were not improved, which has also occurred with previous CFTR treatments.

The authors note that these results emphasize the importance of starting CFTR treatment as early as possible to reduce the risk of lung damage and other sequelae.

Reference

Bec R, Reynaud-Gaubert M, Arnaud F, et al. Chest computed tomography improvement in patients with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor: early report. Eur J Radiol. Published online June 23, 2022. doi:10.1016/j.ejrad.2022.110421