Abstract
A usual interstitial pneumonia (UIP) imaging pattern can be seen in both idiopathic pulmonary fibrosis (IPF) and connective tissue disease-related interstitial lung disease (CTD-ILD). The purpose of this multicenter study was to assess whether quantitative imaging data differ between IPF and CTD-ILD in the setting of UIP. Patients evaluated at two medical centers with CTD-ILD or IPF and a UIP pattern on CT or pathology served as derivation and validation cohorts. Chest CT data were quantitatively analyzed including total volumes of honeycombing, reticulation, ground-glass opacity, normal lung, and vessel related structures (VRS). VRS was compared with forced vital capacity percent predicted (FVC%) and percent predicted diffusing capacity of the lungs for carbon monoxide (DLCO%). There were 296 subjects in total, with 40 CTD-ILD and 85 IPF subjects in the derivation cohort, and 62 CTD-ILD and 109 IPF subjects in the validation cohort. VRS was greater in IPF across the cohorts on univariate (p < 0.001) and multivariable (p < 0.001–0.047) analyses. VRS was inversely correlated with DLCO% in both cohorts on univariate (p < 0.001) and in the derivation cohort on multivariable analysis (p = 0.003) but not FVC%. Total volume of normal lung was associated with DLCO% (p < 0.001) and FVC% (p < 0.001–0.009) on multivariable analysis in both cohorts. VRS appears to have promise in differentiating CTD-ILD from IPF. The underlying pathophysiological relationship between VRS and ILD is complex and is likely not explained solely by lung fibrosis.
Highlights
Idiopathic pulmonary fibrosis (IPF) is the most common form of the idiopathic inIdiopathic pulmonary is the most common form the idiopathic inter- in most terstitial pneumonias as fibrosis well as(IPF)the most common cause of of pulmonary fibrosis stitial pneumonias as well as the most common cause of pulmonary fibrosis in most pop-academic populations
The recent availability of agents for IPFfibrosis as wellwas as data showing thatwith immuosupthe recent availability of antifibrotic agents for as well as data showing that impression in idiopathic pulmonary fibrosis (IPF) is associated with worse outcomes, an accurate diagnosis in the setting of muosuppression in IPF is associated with worse outcomes, an accurate diagnosis in the pulmonary fibrosis has become essential [1,2,3]
There were 62 connective tissue disease-related interstitial lung disease (CTD-ILD) and 109 IPF subjects analyzed in the validation cohort
Summary
Idiopathic pulmonary fibrosis (IPF) is the most common form of the idiopathic inIdiopathic pulmonary is the most common form the idiopathic inter- in most terstitial pneumonias as fibrosis well as(IPF). Discerning the exact diagnosis was thought to be largely academic given that management of most types of pulmonary fibrosis was similar. The quantitative imaging tool known as Computer-Aided Lung Informatics for PaEvaluation and Rating (CALIPER) has been repeatedly shown to predict patient outcomes and thology Evaluation and Rating (CALIPER) has been repeatedly shown to predict patient has been associated withassociated pulmonary function [12,13,14,15,16,17,18,19]. Differentiate CTD-ILD from IPF even in the setting of UIP [6,20,21,22]. The purpose of this multicenter study was to assess whether quantitative imaging data differed multicenter study was to assess whether quantitative imaging data differed across IPF across IPF and CTD-ILD in the setting of UIP. Struction in a patient with connective tissue disease-related interstitial lung disease
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