The Value of CT for Disease Detection and Prognosis Determination in Combined Pulmonary Fibrosis and Emphysema (CPFE)

BACKGROUND AND PURPOSE: Several imaging-based indices were constructed quantitatively using the emphysema index (EI) and fibrosis score (FS) on high-resolution computed tomography (HRCT). We evaluated the ability of these indices to predict mortality compared to physiologic results. Additionally, prognostic predictive factors were compared among subgroups with biopsy-proven fibrotic idiopathic interstitial pneumonia (IIP) (biopsy-proven CPFE) and in a separate cohort with subclinical CPFE. MATERIALS AND METHODS: Three chest radiologists independently determined FS. EI was automatically quantified. PFTs, smoking history, and composite physiologic index (CPI) were reviewed. Predictors of time to death were determined based on clinico-physiologic factors and CT-based CPFE indices. RESULTS: The prevalence of biopsy-proven CPFE was 26% (66/254), with an EI of 9.1±7.1 and a FS of 19.3±14.2. In patients with CPFE, median survival and 5-year survival rates were 6.0 years and 34.8%, respectively, whereas those in fibrotic IIP without emphysema were 10.0 years and 60.9% (p = 0.013). However, the extent of fibrosis did not differ significantly between the two cohorts. In subclinical CPFE, prevalence was 0.04% (93/20,372), EI was 11.3±10.4, and FS was 9.1±7.1. FVC and a fibrosis-weighted CT index were independent predictors of survival in the biopsy-proven CPFE cohort, whereas only the fibrosis-weighted CT index was a significant prognostic factor in the subclinical CPFE cohort. CONCLUSIONS: Recognition and stratification using CT quantification can be utilized as a prognostic predictor. Prognostic factors vary according to fibrosis severity and among cohorts of patients with biopsy-proven and subclinical CPFE.