Combined pulmonary fibrosis and emphysema syndrome: a review

Chest. 2012 Jan;141(1):222-231. doi: 10.1378/chest.11-1062.


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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Humans
  • Incidence
  • Pulmonary Emphysema* / complications
  • Pulmonary Emphysema* / diagnosis
  • Pulmonary Emphysema* / epidemiology
  • Pulmonary Fibrosis* / complications
  • Pulmonary Fibrosis* / diagnosis
  • Pulmonary Fibrosis* / epidemiology
  • Respiratory Function Tests
  • Risk Factors
  • Sex Factors
  • Smoking / adverse effects
  • Survival Rate
  • Syndrome
  • Tomography, X-Ray Computed