Longitudinal Evaluation of Bronchial Changes in Cystic Fibrosis Patients Undergoing Elexacaftor/Tezacaftor/Ivacaftor Therapy Using Lung MRI With Ultrashort Echo‐Times
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Media type:
E-Article
Title:
Longitudinal Evaluation of Bronchial Changes in Cystic Fibrosis Patients Undergoing Elexacaftor/Tezacaftor/Ivacaftor Therapy Using Lung MRI With Ultrashort Echo‐Times
Published in:Journal of Magnetic Resonance Imaging
Language:
English
DOI:
10.1002/jmri.29041
ISSN:
1053-1807;
1522-2586
Origination:
Footnote:
Description:
<jats:sec><jats:title>Background</jats:title><jats:p>Lung magnetic resonance imaging (MRI) with ultrashort echo‐times (UTE‐MRI) allows high‐resolution and radiation‐free imaging of the lung structure in cystic fibrosis (CF). In addition, the combination of elexacaftor/tezacaftor/ivacaftor (ETI) has improved CF clinical outcomes such as need for hospitalization. However, the effect on structural disease still needs longitudinal evaluation at high resolution.</jats:p></jats:sec><jats:sec><jats:title>Purpose</jats:title><jats:p>To analyze the effects of ETI on lung structural alterations using UTE‐MRI, with a focus on bronchiectasis reversibility.</jats:p></jats:sec><jats:sec><jats:title>Study Type</jats:title><jats:p>Retrospective.</jats:p></jats:sec><jats:sec><jats:title>Population</jats:title><jats:p>Fifty CF patients (mean age 24.3 ± 9.2; 23 males).</jats:p></jats:sec><jats:sec><jats:title>Field Strength/Sequence</jats:title><jats:p>1.5 T, UTE‐MRI.</jats:p></jats:sec><jats:sec><jats:title>Assessment</jats:title><jats:p>All subjects completed both UTE‐MRI and pulmonary function tests (PFTs) during two annual visits (M0 and M12), and 30 of them completed a CT scan. They initiated ETI treatment after M0 within a maximum of 3 months from the annual examinations. Three observers scored a clinical MRI Bhalla score on UTE‐MRI. Bronchiectasis reversibility was defined as a reduction in both outer and inner bronchial dimensions. Correlations were searched between the Bhalla score and PFT such as the forced expiratory volume in 1 second percentage predicted (FEV1%p).</jats:p></jats:sec><jats:sec><jats:title>Statistical Tests</jats:title><jats:p>Comparison was assessed using the paired <jats:italic>t</jats:italic>‐test, correlation using the Spearman correlation test with a significance level of 0.05. Concordance and reproducibility were assessed using intraclass correlation coefficient (ICC).</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>There was a significant improvement in MRI Bhalla score after ETI treatment. UTE‐MRI demonstrated bronchiectasis reversibility in a subgroup of 18 out of 50 CF patients (36%). These patients with bronchiectasis reversibility were significantly younger, with lower severity of wall thickening but no difference in mucus plugging extent (<jats:italic>P</jats:italic> = 0.39) was found. The reproducibility of UTE‐MRI evaluations was excellent (ICC ≥ 0.95), was concordant with CT scan (N = 30; ICC ≥ 0.90) and significantly correlated to FEV1% at PFT at M0 (N = 50; <jats:italic>r</jats:italic> = 0.71) and M12 (N = 50; <jats:italic>r</jats:italic> = 0.72).</jats:p></jats:sec><jats:sec><jats:title>Data Conclusion</jats:title><jats:p>UTE‐MRI is a reproducible tool for the longitudinal follow‐up of CF patients, allowing to quantify the response to ETI and demonstrating the reversibility of some structural alterations such as bronchiectasis in a substantial fraction of this study population.</jats:p></jats:sec><jats:sec><jats:title>Level of Evidence</jats:title><jats:p>4</jats:p></jats:sec><jats:sec><jats:title>Technical Efficacy</jats:title><jats:p>Stage 2</jats:p></jats:sec>