Magnetic resonance imaging detects improvements of pulmonary and paranasal sinus abnormalities in response to elexacaf tor/tezacaf tor/ivacaf tor therapy in adults with cystic fibrosis

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 & ndash;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 & ndash;4 4y) 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, respec-tively). 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 re-duction 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. (c) 2022 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Automated summary

This study found that therapy with Elexacaftor/Tezacaftor/Ivacaftor can improve chest and sinus abnormalities in adult cystic fibrosis patients, indicating the reversibility of structural lung and sinus abnormalities in patients with established disease.