REVIEW article
Front. Med.
Sec. Pulmonary Medicine
Volume 12 - 2025 | doi: 10.3389/fmed.2025.1683252
Combined Pulmonary Fibrosis and Emphysema:A Narrative Review
Provisionally accepted
- Department of Respiratory and Critical Care Medicine, West China Hospital, Sichuan University, Chengdu, China
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Combined pulmonary fibrosis and emphysema (CPFE) is defined as a clinical-radiological-physiological syndrome characterized by upper-lobe emphysema and fibrosis predominantly in the lower lobes. The diagnosis of CPFE remains challenging due to the opposing pathophysiological effects of emphysema and fibrosis, which can mask their characteristic clinical and imaging features. Although an international committee proposed standardized terminology for CPFE in 2022, uniform diagnostic criteria and optimal management strategies have not yet been established. Patients with CPFE exhibit reduced overall survival and higher mortality compared to those with chronic obstructive pulmonary disease (COPD) or idiopathic pulmonary fibrosis (IPF). This may be due to increased disease severity, as assessed by CT findings ( the extent of fibrosis and emphysema), advanced age, and associated complications such as lung cancer, acute exacerbations, and pulmonary arterial hypertension. This narrative review synthesizes the literature on CPFE from 1990 to 2024, covering its historical background, epidemiology, pathogenesis, clinical presentation, diagnostic features (imaging and pulmonary function), disease course (diagnosis, prognosis, complications), and management.
Keywords: combined pulmonary fibrosis and emphysema, diagnosis, prognosis, complications, Management
Received: 10 Aug 2025; Accepted: 11 Sep 2025.
Copyright: © 2025 ZENG, Liang and Ou. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Xuemei Ou, ouxuemeihx123@126.com
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