HRCT and histopathological evaluation of fibrosis and tissue destruction in IPF associated with pulmonary emphysema

Respir Med. 2008 Dec;102(12):1753-61. doi: 10.1016/j.rmed.2008.07.010. Epub 2008 Aug 23.


Idiopathic pulmonary fibrosis has been associated with emphysema in cigarette smokers as a new clinical entity: combined pulmonary fibrosis and emphysema (CPFE). In order to compare histomorphometrical, roentgenological and immunohistochemical aspects of usual interstitial pneumonia (UIP) with and without associated pulmonary emphysema, 17 patients with biopsy-proven UIP were evaluated. Morphometrical evaluation of lung parenchyma destruction was used to divide patients in two subgroups: emphysema/UIP (n=9) and UIP alone (n=8); four patients with biopsy-proven emphysema without fibrosis were also evaluated. At HRTC scan, emphysematous lesions were prevalent in the upper fields of both emphysema/UIP and emphysema groups and the distribution of fibrotic lesions was similar in emphysema/UIP compared to UIP alone. The semiquantitative histopathological fibrotic score was also similar in emphysema/UIP and UIP alone. In addition, the expression of tumor necrosis factor (TNF)-alpha, matrix metalloproteinase (MMP)-2, MMP-9, MMP-7 and membrane type 1-metalloproteinase (MT1-MMP) by fibroblasts of myofibroblastic foci was similar in emphysema/UIP and UIP alone patients. In contrast, fibroblasts in areas of parenchymal destruction of emphysema/UIP expressed MMP-2, MMP-9, MMP-7 and MT1-MMP at variable but significantly higher levels when compared to emphysema subjects, in the presence of similar levels of TIMP-1, TIMP-2 and TNF-alpha. Fibrotic and emphysematous lesions in emphysema/UIP patients appear to follow the roentgenological and histopathological patterns expected for either UIP or emphysema. Interstitial fibroblast activation is more pronounced in the areas of lung destruction in emphysema/UIP compared to those with emphysema alone, as for exaggerated tissue remodeling.

MeSH terms

  • Aged
  • Carbon Dioxide / blood
  • Female
  • Humans
  • Male
  • Matrix Metalloproteinases / metabolism
  • Middle Aged
  • Oxygen / blood
  • Partial Pressure
  • Pulmonary Emphysema / complications
  • Pulmonary Emphysema / diagnostic imaging*
  • Pulmonary Emphysema / pathology
  • Pulmonary Emphysema / physiopathology
  • Pulmonary Fibrosis / complications
  • Pulmonary Fibrosis / diagnostic imaging*
  • Pulmonary Fibrosis / pathology
  • Pulmonary Fibrosis / physiopathology
  • Respiratory Function Tests
  • Tissue Inhibitor of Metalloproteinases / metabolism
  • Tomography, X-Ray Computed
  • Total Lung Capacity


  • Tissue Inhibitor of Metalloproteinases
  • Carbon Dioxide
  • Matrix Metalloproteinases
  • Oxygen