Clinical characteristics of combined pulmonary fibrosis and emphysema

Respirology. 2010 Feb;15(2):265-71. doi: 10.1111/j.1440-1843.2009.01676.x. Epub 2009 Dec 27.


This study describes the clinical characteristics of patients with CPFE and compares these with patients with COPD. It should therefore be noteworthy that the prevalence of lung cancer might be high in CPFE patients.

Background and objective: Patients with combined pulmonary fibrosis and emphysema (CPFE) are sometimes seen, and we speculate that these patients have some different clinical characteristics from COPD patients. This study clarifies the clinical characteristics of CPFE patients.

Methods: This was a retrospective study of 47 stable patients with concurrent emphysema and diffuse parenchymal lung disease with fibrosis, based on the findings of chest CT (CPFE patients). The clinical characteristics of CPFE patients were compared with those of emphysema-dominant COPD patients without parenchymal lung disease (COPD patients).

Results: Forty-six of the 47 CPFE patients were male. Paraseptal emphysema was particularly common in the CPFE group. Honeycombing, ground-glass opacities and reticular opacities were present in 75.6%, 62.2% and 84.4% of CPFE patients, respectively. Twenty-two of the 47 CPFE patients (46.8%) had lung cancer. Pulmonary function tests showed that the CPFE group had milder airflow limitation and lower diffusing capacity than the COPD group. Desaturation during 6-min walking test in CPFE patients tended to be more severe than in COPD patients, if the level of FEV1 or 6MWD was equal.

Conclusions: CPFE patients had some different clinical characteristics in comparison with COPD patients and may also have a high prevalence of lung cancer.

Publication types

  • Comparative Study

MeSH terms

  • Aged
  • Biopsy
  • Comorbidity
  • Female
  • Humans
  • Lung / diagnostic imaging
  • Lung / pathology
  • Lung Neoplasms / epidemiology
  • Male
  • Prevalence
  • Pulmonary Disease, Chronic Obstructive / pathology
  • Pulmonary Disease, Chronic Obstructive / physiopathology
  • Pulmonary Emphysema / epidemiology
  • Pulmonary Emphysema / pathology*
  • Pulmonary Emphysema / physiopathology*
  • Pulmonary Fibrosis / epidemiology
  • Pulmonary Fibrosis / pathology*
  • Pulmonary Fibrosis / physiopathology*
  • Respiratory Function Tests
  • Retrospective Studies
  • Tomography, X-Ray Computed