title: Combined pulmonary fibrosis and emphysema: The many aspects of a cohabitation contract.
abstract: Combined 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.
authors: 2nd Respiratory Medicine Department, "Attikon" University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece. Electronic address: papaioannouandriana@gmail.com.
authors: Andriana I
authors: AI
authors: Papaioannou
authors: 2nd Respiratory Medicine Department, "Attikon" University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece. Electronic address: ktk@otenet.gr.
authors: Konstantinos
authors: K
authors: Kostikas
authors: 2nd Respiratory Medicine Department, "Attikon" University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece. Electronic address: fmanali@otenet.gr.
authors: Effrosyni D
authors: ED
authors: Manali
authors: 2nd Respiratory Medicine Department, "Attikon" University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece. Electronic address: geo4pap@gmail.com.
authors: Georgia
authors: G
authors: Papadaki
authors: 2nd Respiratory Medicine Department, "Attikon" University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece. Electronic address: anezaroussou@gmail.com.
authors: Aneza
authors: A
authors: Roussou
authors: 7th Department of Pneumonology, "Sotiria" Chest Diseases Hospital, Athens, Greece. Electronic address: lykol@yahoo.gr.
authors: Likurgos
authors: L
authors: Kolilekas
authors: APHP, Hôpital Bichat, DHU FIRE Service de Pneumologie A, Centre de compétence des maladies pulmonaires rares, INSERM, Unité 1152, Université Paris Diderot, Paris, France. Electronic address: raphael.borie@aphp.fr.
authors: Raphaël
authors: R
authors: Borie
authors: 1st Respiratory Medicine Department, "Sotiria" Chest Diseases Hospital, Athens, Medical School, National and Kapodistrian University of Athens, Greece. Electronic address: dbouros@med.uoa.gr.
authors: Demosthenis
authors: D
authors: Bouros
authors: 2nd Respiratory Medicine Department, "Attikon" University Hospital, Athens Medical School, National and Kapodistrian University of Athens, Greece. Electronic address: papiris@otenet.gr.
authors: Spyridon A
authors: SA
authors: Papiris
mesh_terms: D000328:Adult; D000368:Aged; D004417:Dyspnea / Q000503:physiopathology; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000503:physiopathology; D008168:Lung / Q000503:physiopathology; D008175:Lung Neoplasms / Q000150:complications* / Q000503:physiopathology; D008297:Male; D015995:Prevalence; D011379:Prognosis; D011656:Pulmonary Emphysema / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology; D011658:Pulmonary Fibrosis / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology; D012129:Respiratory Function Tests / Q000379:methods; D012907:Smoking / Q000009:adverse effects / Q000453:epidemiology; D014057:Tomography, X-Ray Computed; D055815:Young Adult
keywords: Combined pulmonary fibrosis and emphysema; High resolution computed tomography; Pulmonary emphysema; Pulmonary fibrosis