Combined pulmonary fibrosis and emphysema syndrome: clinical, functional, and radiological assessment

Abstract

The coexistence of pulmonary fibrosis and emphysema is increasingly recognized. To assess the clinical, physiological and radiological characteristics of patients with combined pulmonary fibrosis and emphysema (CPFE) and compare it with patients with chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) alone. One hundred-twenty patients were enrolled and divided into three groups; 40 had COPD based on poorly reversible airflow obstruction in spirometry; 40 had ILD based on high-resolution computed tomography (HRCT); and 40 had CPFE based on the presence of emphysematous changes in the upper lung zones and pulmonary fibrosis in lower zones in HRCT. Modified Medical Research Council dyspnea scale, arterial blood gas analysis, spirometry, diffusion capacity for carbon monoxide (DLCO), polythesmography, HRCT chest, and echocardiography were done. More than 57% of patients with CPFE were men and the majority of them were smokers. There was no significant difference in dyspnea grade between CPFE group and other groups (P>0.05). The rate of exacerbation per year was significantly higher in the CPFE group (4.2±1.02) compared with either COPD group (3.33±1.56) or ILD group (3.15 ±1.05). CPFE patients had both emphysematous and fibrotic changes on radiological examination. Lung volumes were preserved but DLCO% was significantly lower and the mean pulmonary artery systolic pressure was significantly higher in the CPFE group compared with COPD and ILD. CPFE is a distinct syndrome that has characteristic radiological findings and lung function profile with a significant reduction of DLCO and a significant increase in pulmonary artery systolic pressure.