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

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 mm2 [-0.2, 2.2]; P = 0.099) but decreased in the ETI group (-7.0 mm2 [-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.

• Authors: Lena Wucherpfennig, Simon M F Triphan, Sabine Wege, Hans-Ulrich Kauczor, Claus P Heussel, Olaf Sommerburg, Mirjam Stahl, Marcus A Mall, Monika Eichinger, Mark O Wielpütz
• Journal: Annals of the American Thoracic Society (Ann Am Thorac Soc) Vol. 20 Issue 11 Pg. 1595-1604 (11 2023) ISSN: 2325-6621 [Electronic] United States
• PMID: 37579262 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• elexacaftor
• ivacaftor
• tezacaftor
• Cystic Fibrosis Transmembrane Conductance Regulator
• Aminophenols

Topics

• Adult
• Humans
• Young Adult
• Cystic Fibrosis (diagnostic imaging, drug therapy)
• Bronchial Arteries (diagnostic imaging)
• Dilatation
• Magnetic Resonance Imaging
• Cystic Fibrosis Transmembrane Conductance Regulator
• Mutation
• Aminophenols