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. 2024 Mar 28;63(3):2301794.
doi: 10.1183/13993003.01794-2023. Print 2024 Mar.

Reversal of cylindrical bronchial dilatations in a subset of adults with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor

Paul Cazier  1   2 , Guillaume Chassagnon  1   3   2 , Théo Dhote  4   5 , Jennifer Da Silva  4   5 , Reem Kanaan  4   5 , Isabelle Honoré  4   5 , Nicolas Carlier  4   5 , Marie-Pierre Revel  1   3 , Emma Canniff  1   3 , Clémence Martin  3   4   5 , Pierre-Régis Burgel  6   4   5
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Reversal of cylindrical bronchial dilatations in a subset of adults with cystic fibrosis treated with elexacaftor/tezacaftor/ivacaftor

Paul Cazier et al. Eur Respir J. .

Abstract

Background: 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.

Methods: 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.

Results: 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).

Conclusions: 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.

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Conflict of interest statement

Conflict of interest: G. Chassagnon reports lecture honoraria from Chiesi and Boehringer Ingelheim, outside the submitted work. N. Carlier reports consulting fees from Pfizer, outside the submitted work. M-P. Revel reports lecture honoraria from GE Healthcare, Bracco, Boehringer Ingelheim, MSD and Gleamer, and receipt of AI software from Aidence and Mevis, outside the submitted work; and is Past President of the European Society of Thoracic Imaging. C. Martin reports lecture honoraria from Chiesi, AstraZeneca, Vertex, GSK and Zambon, and travel support from Chiesi and Zambon, outside the submitted work. P-R. Burgel reports support for the present manuscript from Vaincre la Mucoviscidose, and reports grants from Boehringer Ingelheim, GSK and Vertex, and consulting fees and lecture honoraria from Boehringer Ingelheim, GSK, AstraZeneca, Vertex, Chiesi, Pfizer, Novartis, Zambon and Insmed, outside the submitted work. The remaining authors have no potential conflicts of interest to disclose.

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