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. 2024 Jul;60(1):116-124.
doi: 10.1002/jmri.29041. Epub 2023 Oct 20.

Longitudinal Evaluation of Bronchial Changes in Cystic Fibrosis Patients Undergoing Elexacaftor/Tezacaftor/Ivacaftor Therapy Using Lung MRI With Ultrashort Echo-Times

Mathieu David  1 , Ilyes Benlala  1   2   3 , Stephanie Bui  1   2   3 , Thomas Benkert  4 , Patrick Berger  1   2   3 , François Laurent  1   2   3 , Julie Macey  1   2   3 , Gael Dournes  1   2   3
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Longitudinal Evaluation of Bronchial Changes in Cystic Fibrosis Patients Undergoing Elexacaftor/Tezacaftor/Ivacaftor Therapy Using Lung MRI With Ultrashort Echo-Times

Mathieu David et al. J Magn Reson Imaging. 2024 Jul.

Abstract

Background: 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.

Purpose: To analyze the effects of ETI on lung structural alterations using UTE-MRI, with a focus on bronchiectasis reversibility.

Study type: Retrospective.

Population: Fifty CF patients (mean age 24.3 ± 9.2; 23 males).

Field strength/sequence: 1.5 T, UTE-MRI.

Assessment: 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).

Statistical tests: Comparison was assessed using the paired t-test, correlation using the Spearman correlation test with a significance level of 0.05. Concordance and reproducibility were assessed using intraclass correlation coefficient (ICC).

Results: 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 (P = 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; r = 0.71) and M12 (N = 50; r = 0.72).

Data conclusion: 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.

Level of evidence: 4 TECHNICAL EFFICACY: Stage 2.

Keywords: CFTR; MRI; cystic fibrosis; lung.

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References

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