Background

Combined Pulmonary Fibrosis Emphysema (CPFE) is a smoking-related syndrome with poor prognosis.

Objectives

To analyze clinic and functional features of CPFE compared to Idiopathic Pulmonary Fibrosis (IPF) with different extent of emphysema.

Methods

Retrospective, observational and longitudinal study collecting data (epidemiology, comorbidities, lung function and mortality) of 155 patients from University Hospital of Bellvitge at diagnosis, 1, 3 and 5 years: 43 CPFE (>10% E), 31 IPF with emphysema (5% and 10% emphysema), 31 IPF without emphysema and 50 COPD-emphysema.

Results

CPFE and IPF with emphysema patients had a higher smoke exposure and a higher prevalence of pulmonary hypertension (PH), lung cancer, paraseptal emphysema and pneumothorax. CPFE patients presented more dyspnea and higher oxygen (O2) need at diagnosis, with a higher progression rate during the first year after diagnosis (assessed by walking distance and O2 therapy at 6MWT). Patients had worse survival rate proportional to the extent of emphysema (E) and even worse in CPFE-IPF (CPFE-IPF > IPF+E > IPF without E; p<0.001). At diagnosis, CPFE compared to IPF patients had higher values of FVC, RV, FRC, which increased gradually with the extent of emphysema; and lower values of MEF50% and 25% (maximal expiratory flow), FEV1 and KCO (carbon monoxide transfer coefficient) that decreased gradually with the extent of emphysema.

Conclusions

Mortality of CPFE-IPF and IPF patients was proportional to the extent of emphysema. CPFE had a higher prevalence of comorbidities and a higher rate progression during the first year after diagnosis. Comorbidities (PH and pneumothorax) were also directly proportional to the extent of emphysema.