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

Abstract

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.

• Authors: Paola Rogliani, Marco Mura, Paolo Mattia, Amedeo Ferlosio, Gianfranco Farinelli, Salvatore Mariotta, Paolo Graziano, Gabriella Pezzuto, Alberto Ricci, Cesare Saltini, Augusto Orlandi
• Journal: Respiratory medicine (Respir Med) Vol. 102 Issue 12 Pg. 1753-61 (Dec 2008) ISSN: 1532-3064 [Electronic] England
• PMID: 18723334 (Publication Type: Journal Article)

Chemical References

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

Topics

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