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- Title
Differentiation of Idiopathic Pulmonary Fibrosis from Connective Tissue Disease-Related Interstitial Lung Disease Using Quantitative Imaging.
- Authors
Chung, Jonathan H.; Adegunsoye, Ayodeji; Cannon, Brenna; Vij, Rekha; Oldham, Justin M.; King, Christopher; Montner, Steven M.; Thirkateh, Prahasit; Barnett, Scott; Karwoski, Ronald; Bartholmai, Brian J.; Strek, Mary; Nathan, Steven D.
- 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.
- Subjects
IDIOPATHIC pulmonary fibrosis; INTERSTITIAL lung diseases; CONNECTIVE tissues; VITAL capacity (Respiration); PULMONARY fibrosis
- Publication
Journal of Clinical Medicine, 2021, Vol 10, Issue 12, p2663
- ISSN
2077-0383
- Publication type
Article
- DOI
10.3390/jcm10122663