Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor/tezacaftor/ivacaftor therapy in adults 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: Schmitt, Niclas [VerfasserIn]
• Verfasst von: Wünnemann, Felix [VerfasserIn]
• Verfasst von: Mayer, Victoria Louise [VerfasserIn]
• Verfasst von: Sommerburg, Olaf [VerfasserIn]
• Verfasst von: Mall, Marcus A. [VerfasserIn]
• Verfasst von: Eichinger, Monika [VerfasserIn]
• Verfasst von: Wielpütz, Mark Oliver [VerfasserIn]
• Titel: Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaftor/tezacaftor/ivacaftor therapy in adults with cystic fibrosis
• Verf.angabe: Lena Wucherpfennig, Simon M. F. Triphan, Sabine Wege, Hans-Ulrich Kauczor, Claus P. Heussel, Niclas Schmitt, Felix Wuennemann, Victoria L. Mayer, Olaf Sommerburg, Marcus A. Mall, Monika Eichinger, Mark O. Wielpütz
• E-Jahr: 2022
• Jahr: 29 November 2022
• Umfang: 8 S.
• Fussnoten: Online verfügbar 8 April 2022, Artikelversion 29 November 2022 ; Gesehen am 21.03.2023
• Titel Quelle: Enthalten in: Journal of cystic fibrosis
• Ort Quelle: Amsterdam [u.a.] : Elsevier Science, 2002
• Jahr Quelle: 2022
• Band/Heft Quelle: 21(2022), 6 vom: Nov., Seite 1053-1060
• ISSN Quelle: 1873-5010
• DOI: doi:10.1016/j.jcf.2022.03.011
• Datenträger: Online-Ressource
• Sprache: eng
• Sach-SW: Airway disease
• Sach-SW: Cystic fibrosis
• Sach-SW: Functional MRI
• Sach-SW: Lung perfusion
• Sach-SW: Mucus obstruction, CFTR-modulator
• K10plus-PPN: 1839669578

Abstract

Background - Therapy with Elexacaftor/Tezacaftor/Ivacaftor (ETI) was recently approved for adult cystic fibrosis (CF) patients with at least one F508del mutation. However, its effects on structural and functional lung abnormalities and chronic rhinosinusitis have not been studied by imaging. - Methods - 19 adults with CF (mean age 31±9y, range 19-55y) underwent standardized chest magnetic resonance imaging (MRI), and nine also same-session sinonasal MRI, before (MRI1) and after (MRI2) at least one month (mean duration 5 ± 3mon) on ETI. 24 control CF patients (30±7y, range 20-44y) without ETI underwent longitudinal chest MRI, and eleven also sinonasal MRI, twice (mean interval 40±15mon). MRI was assessed using the validated chest MRI score and chronic rhinosinusitis (CRS)-MRI score. Forced expiratory volume in 1 s percent predicted (FEV1%) was measured in all patients. - Results - In controls, the chest MRI global score and CRS-MRI sum score were stable from MRI1 to MRI2. In patients under ETI, the chest MRI global score improved (-11.4 ± 4.6, P<0.001), mainly due to reduction of bronchiectasis/wall thickening and mucus plugging subscores (-3.3 ± 2.2 and -5.2 ± 1.5, P<0.001, respectively). The improvement in chest MRI score correlated well with improved FEV1% (r=-0.703, P<0.001). The CRS-MRI sum score also improved in patients under ETI (-6.9 ± 3.0, P<0.001), mainly due to a reduction of mucopyoceles in the maxillary and ethmoid sinus (-50% and -39%, P<0.05, respectively). - Conclusions - MRI detects improvements of chest MRI and CRS-MRI scores in adult CF patients who first received ETI, demonstrating reversibility of structural lung and paranasal sinus abnormalities in patients with established disease.