• Andriana I Papaioannou, Konstantinos Kostikas, Effrosyni D Manali, Georgia Papadaki, Aneza Roussou, Likurgos Kolilekas, Raphaël Borie, Demosthenis Bouros, and Spyridon A Papiris.
• 2nd Respiratory Medicine Department, "Attikon" University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece. Electronic address: papaioannouandriana@gmail.com.
• Respir Med. 2016 Aug 1; 117: 14-26.

AbstractCombined pulmonary fibrosis and emphysema (CPFE) is a clinical entity characterized by the coexistence of upper lobe emphysema and lower lobe fibrosis. Patients with this condition experience severe dyspnea and impaired gas exchange with preserved lung volumes. The diagnosis of the CPFE syndrome is based on HRCT imaging, showing the coexistence of emphysema and pulmonary fibrosis both in varying extent and locations within the lung parenchyma. Individual genetic background seem to predispose to the development of the disease. The risk of the development of pulmonary hypertension in patients with CPFE is high and related to poor prognosis. CPFE patients also present a high risk of lung cancer. Mortality is significant in patients with CPFE and median survival is reported between 2.1 and 8.5 years. Currently, no specific recommendations are available regarding the management of patients with CPFE. In this review we provide information on the existing knowledge on CPFE regarding the pathophysiology, clinical manifestations, imaging, complications, possible therapeutic interventions and prognosis of the disease.Copyright © 2016 Elsevier Ltd. All rights reserved.

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