TY - JOUR
T1 - The assessment of short- and long-term changes in lung function in cystic fibrosis using 129Xe MRI
AU - Smith, Laurie J.
AU - Horsley, Alex
AU - Bray, Jody
AU - Hughes, Paul J. C.
AU - Biancardi, Alberto
AU - Norquay, Graham
AU - Wildman, Martin
AU - West, Noreen
AU - Marshall, Helen
AU - Wild, Jim M.
N1 - Funding Information:
Conflict of interest: L.J. Smith reports grants from National Institute for Health Research, during the conduct of the study. A. Horsley reports grants from National Institute of Health Research, during the conduct of the study; grants from Cystic Fibrosis Trust and Cystic Fibrosis Foundation, personal fees from Mylan Pharmaceuticals and Vertex Pharmaceuticals, outside the submitted work. J. Bray has nothing to disclose. P.J.C. Hughes reports grants from GlaxoSmithKline, outside the submitted work. A. Biancardi has nothing to disclose. G. Norquay has nothing to disclose. M. Wildman has nothing to disclose. N. West has nothing to disclose. H. Marshall has nothing to disclose. J.M. Wild has nothing to disclose.
Funding Information:
Support statement: This report is independent research supported by the National Institute for Health Research (NIHR) and Health Education England and also the Medical Research Council (MRC). This work was supported by the NIHR grants ICA-CDRF-2015-01-027 (awarded to L.J. Smith) and NIHR-RP-R3-12-027 (awarded to J.M. Wild) and MRC grant MR/M008894/1 (awarded to J.M. Wild). A. Horsley was supported by an NIHR Clinician Scientist award (NIHR-CS012-13) and by the NIHR Manchester Biomedical Research Centre. The views expressed in this publication are those of the author(s) and not necessarily those of the NHS, the National Institute for Health Research, Health Education England or the Department of Health. Funding information for this article has been deposited with the Crossref Funder Registry.
Publisher Copyright:
Copyright © ERS 2020
PY - 2020/12/1
Y1 - 2020/12/1
N2 - Introduction: Xenon-129 ( 129Xe) ventilation magnetic resonance imaging (MRI) is sensitive to detect early cystic fibrosis (CF) lung disease and response to treatment. 129Xe-MRI could play a significant role in clinical trials and patient management. Here we present data on the repeatability of imaging measurements and their sensitivity to longitudinal change. Methods: 29 children and adults with CF and a range of disease severity were assessed twice, a median (interquartile range (IQR)) of 16.0 (14.4–19.5) months apart. Patients underwent 129Xe-MRI, lung clearance index (LCI), body plethysmography and spirometry at both visits. 11 patients repeated 129Xe-MRI in the same session to assess the within-visit repeatability. The ventilation defect percentage (VDP) was the primary metric calculated from 129Xe-MRI. Results: At baseline, mean±SD age was 23.0±11.1 years and forced expiratory volume in 1 s (FEV 1) z-score was −2.2±2.0. Median (IQR) VDP was 9.5 (3.4–31.6)% and LCI was 9.0 (7.7–13.7). Within- and inter-visit repeatability of VDP was high. At 16 months there was no single trend of 129Xe-MRI disease progression. Visible 129Xe-MRI ventilation changes were common, which reflected changes in VDP. Based on the within-visit repeatability, a significant short-term change in VDP is >±1.6%. For longer-term follow-up, changes in VDP of up to ±7.7% can be expected, or ±4.1% for patients with normal FEV 1. No patient had a significant change in FEV 1; however, 59% had change in VDP >±1.6%. In patients with normal FEV 1, there were significant changes in ventilation and in VDP. Conclusions: 129Xe-MRI is a highly effective method for assessing longitudinal lung disease in patients with CF. VDP has great potential as a sensitive clinical outcome measure of lung function and end-point for clinical trials.
AB - Introduction: Xenon-129 ( 129Xe) ventilation magnetic resonance imaging (MRI) is sensitive to detect early cystic fibrosis (CF) lung disease and response to treatment. 129Xe-MRI could play a significant role in clinical trials and patient management. Here we present data on the repeatability of imaging measurements and their sensitivity to longitudinal change. Methods: 29 children and adults with CF and a range of disease severity were assessed twice, a median (interquartile range (IQR)) of 16.0 (14.4–19.5) months apart. Patients underwent 129Xe-MRI, lung clearance index (LCI), body plethysmography and spirometry at both visits. 11 patients repeated 129Xe-MRI in the same session to assess the within-visit repeatability. The ventilation defect percentage (VDP) was the primary metric calculated from 129Xe-MRI. Results: At baseline, mean±SD age was 23.0±11.1 years and forced expiratory volume in 1 s (FEV 1) z-score was −2.2±2.0. Median (IQR) VDP was 9.5 (3.4–31.6)% and LCI was 9.0 (7.7–13.7). Within- and inter-visit repeatability of VDP was high. At 16 months there was no single trend of 129Xe-MRI disease progression. Visible 129Xe-MRI ventilation changes were common, which reflected changes in VDP. Based on the within-visit repeatability, a significant short-term change in VDP is >±1.6%. For longer-term follow-up, changes in VDP of up to ±7.7% can be expected, or ±4.1% for patients with normal FEV 1. No patient had a significant change in FEV 1; however, 59% had change in VDP >±1.6%. In patients with normal FEV 1, there were significant changes in ventilation and in VDP. Conclusions: 129Xe-MRI is a highly effective method for assessing longitudinal lung disease in patients with CF. VDP has great potential as a sensitive clinical outcome measure of lung function and end-point for clinical trials.
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UR - https://www.mendeley.com/catalogue/cea6d197-bfad-3b0a-806d-17dc24b06936/
U2 - 10.1183/13993003.00441-2020
DO - 10.1183/13993003.00441-2020
M3 - Article
SN - 0903-1936
VL - 56
SP - 1
EP - 10
JO - European Respiratory Journal
JF - European Respiratory Journal
IS - 6
M1 - 2000441
ER -
