Highlights
- •The chest MRI global score showed a long-term stability in patients under standard therapy without elexacaftor/tezacaftor/ivacaftor.
- •The chest MRI global score improved in patients under Elexacaftor/Tezacaftor/Ivacaftor, mainly due to reduction of bronchiectasis/wall thickening and mucus plugging.
- •The improved chest MRI global score correlated with proportional improvements in FEV1%.
- •The chronic rhinosinusitis MRI sum score improved under elexacaftor/tezacaftor/ivacaftor therapy through a reduction of mucopyoceles.
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.
Keywords
Abbreviations:
CF (cystic fibrosis), CFTRm (cystic fibrosis transmembrane conductance regulator modulator), CT (Computed tomography), CRS (chronic rhinosinusitis), ETI (Elexacaftor/Tezacaftor/Ivacaftor), FEV1% (Forced expiratory volume in 1 s percent predicted), MRI (magnetic resonance imaging)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: April 08, 2022
Accepted:
March 28,
2022
Received in revised form:
March 24,
2022
Received:
February 6,
2022
Identification
Copyright
© 2022 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
