Articles: pulmonary-fibrosis-etiology.
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Am. J. Respir. Crit. Care Med. · Oct 2003
Adenoviral gene transfer of connective tissue growth factor in the lung induces transient fibrosis.
Connective tissue growth factor (CTGF) is felt to be one of the key profibrotic factors and is a downstream effector molecule mediating the action of transforming growth factor (TGF)-beta1, a cytokine known to induce severe and progressive fibrosis. However, the in vivo fibrogenic effect of isolated CTGF expression is not well described. We used adenoviral gene transfer to transiently overexpress CTGF in rat lungs after intratracheal administration and compared it with transient overexpression of active TGF-beta1 delivered by a similar adenovirus vector. ⋯ These same genes were robustly and persistently stimulated by TGF-beta1 from Day 3 to Day 21. This data suggested that CTGF may act as a TGF-beta1 cofactor rather than a direct fibrogenic factor. We demonstrate that CTGF overexpression can initiate fibrogenic activity but likely requires the presence of additional factors, such as tissue inhibitor of metalloproteinase-1, to maintain a nonfibrolytic environment and to cause progression of fibrosis.
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J. Thorac. Cardiovasc. Surg. · Aug 2003
Comparative StudySurvival benefit of lung transplantation for patients with idiopathic pulmonary fibrosis.
Although lung transplantation is viewed as an acceptable option for patients with end-stage idiopathic pulmonary fibrosis, the survival benefit of this approach is still debated. This study examined whether there was a survival benefit of lung transplantation in a cohort of patients referred to our transplant center with a diagnosis of idiopathic pulmonary fibrosis according to American Thoracic Society criteria. ⋯ Lung transplantation is effective in improving the survival of selected patients affected by idiopathic pulmonary fibrosis.
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Pol. Merkur. Lekarski · Jul 2003
Review[Potential role of environmental and occupational pollutants in the etiological pathogenesis of idiopathic pulmonary fibrosis].
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and usually fatal lung disease of no identifiable etiology, which is characterised by fibroblast proliferation and extracellular matrix accumulation resulting in irreversible distortion of the architecture of the lung. Some studies suggest the role of various environmental exposure and occupational pollutants in the etiopathogenesis of IPF. ⋯ This article is a review of studies searching for potential etiological factors of IPF in the environment in the place of living and place of working. It seems, that conflicting results of these studies make impossible to determine the potential role of environmental and occupational pollutants in the pathogenesis of IPF.
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Microscopic polyangiitis (MPA) is one of the vasculitides that is included in the pulmonary renal syndromes. Pathologically, MPA has been defined as necrotizing vasculitis with few or no immune deposits, primarily affecting small vessels including arterioles, venules, or capillaries. Pulmonary interstitial fibrosis (PIF) as an accompanying manifestation in MPA has not been widely appreciated. ⋯ As determined by CT of the chest, PIF was detected in all of the patients and was often present years before a diagnosis of MPA was made. We conclude that PIF may occur as a pulmonary manifestation of MPA. Further appreciation of this finding may lead to more data with respect to the incidence of PIF in MPA, and to a better understanding of the mechanisms that are involved in the development of this finding.