Combined pulmonary fibrosis and emphysema characteristics in a Greek cohort

Abstract

Background Combined pulmonary fibrosis and emphysema (CPFE) has recently received great attention, with studies suggesting that it presents a distinct clinical entity while others have challenged this hypothesis. This nationwide study aimed to describe a large cohort of Greek CPFE patients and to examine potential prognostic factors for survival. Methods This retrospective study included 97 patients with CPFE. Demographic and clinical data, pulmonary function tests, echocardiography results and bronchoalveolar lavage analysis were recorded. Results Most patients were male (94.8%) and 92% were current or ex-smokers. Spirometry results were abnormal (forced vital capacity (FVC) 72.9±19.9% pred and forced expiratory volume in 1 s/FVC 82.9±9.7%) with reduced diffusing capacity of the lung for carbon monoxide (DLCO) (42.3±17.4% pred). Mean systolic pulmonary arterial pressure was 41.9±19.7 mmHg and pulmonary hypertension was present in 58.8% of patients. Mean 6-min walk distance was 335.4±159.4 m. Mean emphysema score was 14.23±8.69% and mean interstitial lung disease (ILD) extent was 39.58±19.82%. Mean survival was 84 months (95% CI 72–96 months). Patients with DLCO ≥39% pred had better survival than patients with DLCO <39% pred (p=0.031). Patients with ILD extent ≥30% had worse survival than patients with ILD extent <30% (p=0.037). Conclusions Our results indicate that CPFE patients have preserved lung volumes associated with disproportionately reduced DLCO, while reduced DLCO and increased ILD extent was associated with worse prognosis.