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Master Thesis

Un score IRM de l’atteinte pulmonaire basé sur la séquence ZTE dans la mucoviscidose : application sous lumacaftor/ivacaftor chez des adolescents et de jeunes adultes

Abstract : Thanks to recent improvements in lung parenchymal evaluation by sequences such as tridimensional Zero-Time Echo (3D-ZTE), MRI is an emerging alternative compared to the cumulated radiation risk of CT for the routine assessment of lung disease in patients with cystic fibrosis (CF). MRI could be applied as endpoint in clinical trials of Cystic Fibrosis Transmembrane conductance Regulator (CFTR) modulator therapy. The aim of this work was to evaluate the inter-reader reproducibility and correlation with pulmonary function tests (PFTs) and sweat tests of a 3D-ZTE-based morphological severity score of lung disease in CF, in patients with different severity range, treated with CFTR modulator therapy. From July 2016 to December 2017, 28 teenagers and young adults (age > 12 years), homozygous for the F508del-CFTR mutation were included in a monocentric prospective study, and underwent MRI at baseline and/or at 6 months of CFTR modulator therapy. A lobe based analysis was performed for bronchiectasis, wall thickening, mucus plugging, consolidation, mosaic attenuation. Inter-reader agreement of the MRI-CF score and correlation with PFTs and sweat tests were assessed. 45 MRI exams, from 28 patients (median age 14.5 years, 53.6% males), were analyzed. Our CF-MRI score showed good to excellent inter-reader agreement (Intraclass Correlation Coefficient of 0.87, CI95%= 0.78-0.93) and was significantly correlated with FEV1% (ρ= -0.68; p<0.001). 17 patients underwent MRI at baseline and 6 months of CFTR modulator therapy. Their MRI score did not improve significantly, and this correlated to the absence of significant change in their FEV1% (p=0.98 and 0.32, respectively). MRI, including a 3D-ZTE sequence, is a valuable tool to monitor lung disease in CF. Our CF-MRI score is reproducible and applicable for evaluation of a large spectrum of CF lung disease severity.
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Master Thesis
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https://dumas.ccsd.cnrs.fr/dumas-03200264
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Submitted on : Thursday, May 6, 2021 - 4:04:26 PM
Last modification on : Tuesday, September 7, 2021 - 3:23:51 AM

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Aurélie Hansmann. Un score IRM de l’atteinte pulmonaire basé sur la séquence ZTE dans la mucoviscidose : application sous lumacaftor/ivacaftor chez des adolescents et de jeunes adultes. Médecine humaine et pathologie. 2019. ⟨dumas-03200264⟩

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