In:
Thorax, BMJ, Vol. 76, No. 2 ( 2021-02), p. 144-151
Abstract:
Idiopathic pulmonary fibrosis (IPF) is a fatal disease of lung scarring. Many patients later develop raised pulmonary vascular pressures, sometimes disproportionate to the interstitial disease. Previous therapeutic approaches that have targeted pulmonary vascular changes have not demonstrated clinical efficacy, and quantitative assessment of regional pulmonary vascular involvement using perfusion imaging may provide a biomarker for further therapeutic insights. Methods We studied 23 participants with IPF, using dynamic contrast-enhanced MRI (DCE-MRI) and pulmonary function tests, including forced vital capacity (FVC), transfer factor (TL CO ) and coefficient (K CO ) of the lungs for carbon monoxide. DCE-MRI parametric maps were generated including the full width at half maximum (FWHM) of the bolus transit time through the lungs. Key metrics used were mean (FWHM mean ) and heterogeneity (FWHM IQR ). Nineteen participants returned at 6 months for repeat assessment. Results Spearman correlation coefficients were identified between TL CO and FWHM IQR (r=−0.46; p=0.026), K CO and FWHM mean (r=−0.42; p=0.047) and K CO and FWHM IQR (r=−0.51; p=0.013) at baseline. No statistically significant correlations were seen between FVC and DCE-MRI metrics. Follow-up at 6 months demonstrated statistically significant decline in FVC (p=0.040) and K CO (p=0.014), with an increase in FWHM mean (p=0.040), but no significant changes in TL CO (p=0.090) nor FWHM IQR (p=0.821). Conclusions DCE-MRI first pass perfusion demonstrates correlations with existing physiological gas exchange metrics, suggesting that capillary perfusion deficit (as well as impaired interstitial diffusion) may contribute to gas exchange limitation in IPF. FWHM mean showed a significant increase over a 6-month period and has potential as a quantitative biomarker of pulmonary vascular disease progression in IPF.
Type of Medium:
Online Resource
ISSN:
0040-6376
,
1468-3296
DOI:
10.1136/thoraxjnl-2019-214375
Language:
English
Publisher:
BMJ
Publication Date:
2021
detail.hit.zdb_id:
1481491-2
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