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Elexacaftor/Tezacaftor/Ivacaftor Improves Bronchial Artery Dilatation Detected by Magnetic Resonance Imaging in Patients with Cystic Fibrosis.

Authors :
Wucherpfennig L
Triphan SMF
Wege S
Kauczor HU
Heussel CP
Sommerburg O
Stahl M
Mall MA
Eichinger M
Wielpütz MO
Source :
Annals of the American Thoracic Society [Ann Am Thorac Soc] 2023 Nov; Vol. 20 (11), pp. 1595-1604.
Publication Year :
2023

Abstract

Rationale: Magnetic resonance imaging (MRI) detects improvements in mucus plugging and bronchial wall thickening, but not in lung perfusion in patients with cystic fibrosis (CF) treated with elexacaftor/tezacaftor/ivacaftor (ETI). Objectives: To determine whether bronchial artery dilatation (BAD), a key feature of advanced lung disease, indicates irreversibility of perfusion abnormalities and whether BAD could be reversed in CF patients treated with ETI. Methods: A total of 59 adults with CF underwent longitudinal chest MRI, including magnetic resonance angiography twice, comprising 35 patients with CF (mean age, 31 ± 7 yr) before (MRI1) and after (MRI2) at least 1 month (mean duration, 8 ± 4 mo) on ETI therapy and 24 control patients with CF (mean age, 31 ± 7 yr) without ETI. MRI was assessed using the validated chest MRI score, and the presence and total lumen area of BAD were assessed with commercial software. Results: The MRI global score was stable in the control group from MRI1 to MRI2 (mean difference, 1.1 [-0.3, 2.4]; P  = 0.054), but it was reduced in the ETI group (-10.1 [-0.3, 2.4]; P  < 0.001). In the control and ETI groups, BAD was present in almost all patients at baseline (95% and 94%, respectively), which did not change at MRI2. The BAD total lumen area did not change in the control group from MRI1 to MRI2 (1.0 mm <superscript>2</superscript> [-0.2, 2.2]; P  = 0.099) but decreased in the ETI group (-7.0 mm <superscript>2</superscript> [-8.9, -5.0]; P  < 0.001). This decrease correlated with improvements in the MRI global score ( r  = 0.540; P  < 0.001). Conclusions: Our data show that BAD may be partially reversible under ETI therapy in adult patients with CF who have established disease.

Details

Language :
English
ISSN :
2325-6621
Volume :
20
Issue :
11
Database :
MEDLINE
Journal :
Annals of the American Thoracic Society
Publication Type :
Academic Journal
Accession number :
37579262
Full Text :
https://doi.org/10.1513/AnnalsATS.202302-168OC