title: 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: Department of Internal Medicine, University of Rome Tor Vergata, Rome, Italy.
authors: Paola
authors: P
authors: Rogliani
authors: Marco
authors: M
authors: Mura
authors: Paolo
authors: P
authors: Mattia
authors: Amedeo
authors: A
authors: Ferlosio
authors: Gianfranco
authors: G
authors: Farinelli
authors: Salvatore
authors: S
authors: Mariotta
authors: Paolo
authors: P
authors: Graziano
authors: Gabriella
authors: G
authors: Pezzuto
authors: Alberto
authors: A
authors: Ricci
authors: Cesare
authors: C
authors: Saltini
authors: Augusto
authors: A
authors: Orlandi
mesh_terms: D000368:Aged; D002245:Carbon Dioxide / Q000097:blood; D005260:Female; D006801:Humans; D008297:Male; D020782:Matrix Metalloproteinases / Q000378:metabolism; D008875:Middle Aged; D010100:Oxygen / Q000097:blood; D010313:Partial Pressure; D011656:Pulmonary Emphysema / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D011658:Pulmonary Fibrosis / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D012129:Respiratory Function Tests; D019714:Tissue Inhibitor of Metalloproteinases / Q000378:metabolism; D014057:Tomography, X-Ray Computed; D014109:Total Lung Capacity
chemical_list: D019714:Tissue Inhibitor of Metalloproteinases; D002245:Carbon Dioxide; D020782:Matrix Metalloproteinases; D010100:Oxygen