[THE SYNDROME OF COMBINED PULMONARY FIBROSIS AND EMPHYSEMA - CPFE].

There is an increasing recognition of patients with radiologic features of both pulmonary fibrosis and emphysema. This association has enabled the description of a syndrome termed combined pulmonary fibrosis and emphysema (CPFE) with unique features. Patients are mostly male smokers who suffer from effort dyspnea and hypoxemia, with near-normal spirometric and lung volumes measurements, and abnormalities of gas exchange. On the computerized tomography there is upper-lobe emphysema and lower-lobe interstitial fibrosis. CPFE is frequently complicated by pulmonary hypertension, lung cancer, acute lung injury and coronary artery disease. Mortality is significant, especially in the presence of pulmonary hypertension. In this article we will review the CPFE syndrome.