• Sarah C Scharm, Jens Vogel-Claussen, Cornelia Schaefer-Prokop, Sabine Dettmer, Lars Knudsen, Danny Jonigk, Jan Fuge, Rosa-Marie Apel, Tobias Welte, Frank Wacker, Antje Prasse, and Hoen-Oh Shin.
• Institute of Diagnostic and Interventional Radiology, Hannover Medical School, Carl-Neuberg-Str.1, 30625, Hannover, Germany.
• Eur Radiol. 2021 Sep 1; 31 (9): 6640-6651.

ObjectivesThe individual course of disease in idiopathic pulmonary fibrosis (IPF) is highly variable. Assessment of disease activity and prospective estimation of disease progression might have the potential to improve therapy management and indicate the onset of treatment at an earlier stage. The aim of this study was to evaluate whether regional ventilation, lung perfusion, and late enhancement can serve as early imaging markers for disease progression in patients with IPF.MethodsIn this retrospective study, contrast-enhanced dual-energy CT scans of 32 patients in inspiration and delayed expiration were performed at two time points with a mean interval of 15.4 months. The pulmonary blood volume (PBV) images obtained in the arterial and delayed perfusion phase served as a surrogate for arterial lung perfusion and parenchymal late enhancement. The virtual non-contrast (VNC) images in inspiration and expiration were non-linearly registered to provide regional ventilation images. Image-derived parameters were correlated with longitudinal changes of lung function (FVC%, DLCO%), mean lung density in CT, and CT-derived lung volume.ResultsRegional ventilation and late enhancement at baseline preceded future change in lung volume (R - 0.474, p 0.006/R - 0.422, p 0.016, respectively) and mean lung density (R - 0.469, p 0.007/R - 0.402, p 0.022, respectively). Regional ventilation also correlated with a future change in FVC% (R - 0.398, p 0.024).ConclusionCT-derived functional parameters of regional ventilation and parenchymal late enhancement are potential early imaging markers for idiopathic pulmonary fibrosis progression.Key Points• Functional CT parameters at baseline (regional ventilation and late enhancement) correlate with future structural changes of the lung as measured with loss of lung volume and increase in lung density in serial CT scans of patients with idiopathic pulmonary fibrosis. • Functional CT parameter measurements in high-attenuation areas (- 600 to - 250 HU) are significantly different from normal-attenuation areas (- 950 to - 600 HU) of the lung. • Mean regional ventilation in functional CT correlates with a future change in forced vital capacity (FVC) in pulmonary function tests.© 2021. The Author(s).

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