Elexacaftor/Tezacaftor/Ivacaftor improves bronchial artery dilatation detected by magnetic resonance imaging in patients with cystic fibrosis

• Verfasst von: Wucherpfennig, Lena [VerfasserIn]
• Verfasst von: Triphan, Simon M. F. [VerfasserIn]
• Verfasst von: Wege, Sabine [VerfasserIn]
• Verfasst von: Kauczor, Hans-Ulrich [VerfasserIn]
• Verfasst von: Heußel, Claus Peter [VerfasserIn]
• Verfasst von: Sommerburg, Olaf [VerfasserIn]
• Verfasst von: Stahl, Mirjam [VerfasserIn]
• Verfasst von: Mall, Marcus A. [VerfasserIn]
• Verfasst von: Eichinger, Monika [VerfasserIn]
• Verfasst von: Wielpütz, Mark Oliver [VerfasserIn]
• Titel: Elexacaftor/Tezacaftor/Ivacaftor improves bronchial artery dilatation detected by magnetic resonance imaging in patients with cystic fibrosis
• Verf.angabe: Lena Wucherpfennig, Simon M.F. Triphan, Sabine Wege, Hans-Ulrich Kauczor, Claus P. Heussel, Olaf Sommerburg, Mirjam Stahl, Marcus A. Mall, Monika Eichinger, and Mark O. Wielpütz
• E-Jahr: 2023
• Jahr: November 2023
• Umfang: 10 S.
• Fussnoten: Gesehen am 26.02.2024
• Titel Quelle: Enthalten in: American Thoracic SocietyAnnals of the American Thoracic Society
• Ort Quelle: New York, NY : American Thoracic Society, 2013
• Band/Heft Quelle: 20(2023), 11 vom: Nov., Seite 1595-1604
• ISSN Quelle: 2325-6621
• DOI: doi:10.1513/AnnalsATS.202302-168OC
• Datenträger: Online-Ressource
• Sprache: eng
• Sach-SW: airway disease
• Sach-SW: cystic fibrosis
• Sach-SW: cystic fibrosis transmembrane conductance regulator modulator
• Sach-SW: functional magnetic resonance imaging
• Sach-SW: lung perfusion
• K10plus-PPN: 1881570843

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.