Abstract

Objective An image analysis of high-resolution computed tomography (HRCT) can provide objective quantitation of the disease status in idiopathic pulmonary fibrosis (IPF). However, to our knowledge, no reports have investigated the utility of the normal lung volume for evaluating mortality from IPF. This study aimed to evaluate the relationship between the normally attenuated lung volume on HRCT as a percentage of whole-lung volume (NL%) and IPF mortality. Methods The NL% was determined by HRCT (between -950 and -701 Hounsfield units) using a density mask technique and volumetric software. The NL%, visual assessments of the normal lung by two radiologists, pulmonary function variables, and the gender, age, and physiology (GAP) index were retrospectively evaluated for 175 patients with IPF. Uni- and multivariate Cox proportional hazards analyses and C statistics for mortality were performed. Results The univariate Cox proportional hazards analysis identified the NL% as a prognostic factor [hazard ratio, 0.949; 95% confidence interval (CI), 0.936-0.964; p<0.0001]. In the multivariate analysis, the NL% was a prognostic factor, but the radiologists' visual assessment scores of normal lung were not. The C index increased when the NL% was included in the models of the pulmonary function variables. Furthermore, the C index for a combined model of GAP stage and categorized NL% (0.758; 95% CI, 0.751-0.762) was higher than for the model with the GAP stage alone (0.689; 95% CI, 0.672-0.709). Conclusion The NL% was a prognostic factor in our study population. Quantification of the normal lung using our method may help improve the IPF staging systems.

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