التفاصيل البيبلوغرافية
| العنوان: |
Longitudinal Evaluation of Bronchial Changes in Cystic Fibrosis Patients Undergoing Elexacaftor/Tezacaftor/Ivacaftor Therapy Using Lung MRI With Ultrashort Echo‐Times. |
| المؤلفون: |
David, Mathieu, Benlala, Ilyes, Bui, Stephanie, Benkert, Thomas, Berger, Patrick, Laurent, François, Macey, Julie, Dournes, Gael |
| المصدر: |
Journal of Magnetic Resonance Imaging; Jul2024, Vol. 60 Issue 1, p116-124, 9p |
| مصطلحات موضوعية: |
CYSTIC fibrosis, FORCED expiratory volume, MAGNETIC resonance imaging, LUNGS, INTRACLASS correlation, ECHO-planar imaging |
| مستخلص: |
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 [ABSTRACT FROM AUTHOR] |
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