Unclassifiable-interstitial lung disease: Outcome prediction using CT and functional indices

Unclassifiable-interstitial lung disease (uILD) represents a heterogeneous collection of pathologies encompassing those fibrosing lung diseases which do not fulfill current diagnostic criteria. We evaluated baseline and longitudinal functional and CT (visual and quantitative computer [CALIPER] analysis) variables to identify outcome predictors in uILD. Consecutive patients with uILD on multidisciplinary review (n = 95) had baseline functional (FVC, DLco, CPI [composite physiologic index]) and CT features (visual evaluation: CT pattern, fibrosis extent, honeycombing presence, traction bronchiectasis severity, pulmonary artery (PA) diameter; CALIPER evaluation: fibrosis extent, pulmonary vessel volume (PVV)) examined in univariate and multivariate Cox regression models. Change in functional and CT variables were examined in a patient subset (n = 37), to identify indicators of outcome. On univariate analysis, CPI was the most powerful functional predictor of mortality (p