Journal
CHEST
Volume 141, Issue 1, Pages 222-231Publisher
ELSEVIER
DOI: 10.1378/chest.11-1062
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Funding
- Department of Medicine, Alpert Medical School of Brown University
- VA
- National Heart, Lung, and Blood Institute [R01 HL64936]
- Providence VA Medical Center
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There is increasing clinical, radiologic, and pathologic recognition of the coexistence of emphysema and pulmonary fibrosis in the same patient, resulting in a clinical syndrome known as combined pulmonary fibrosis and emphysema (CPFE) that is characterized by dyspnea, upper-lobe emphysema, lower-lobe fibrosis, and abnormalities of gas exchange. This syndrome frequently is complicated by pulmonary hypertension, acute lung injury, and lung cancer. The CPFE syndrome typically occurs in male smokers, and the mortality associated with this condition, especially if pulmonary hypertension is present, is significant. In this review, we explore the current state of the literature and discuss etiologic factors and clinical characteristics of the CPFE syndrome. CHEST 2012; 141(1):222-231
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