Articles: pulmonary-fibrosis-etiology.
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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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Idiopathic pulmonary fibrosis is a progressive and usually fatal lung disease characterized by fibroblast proliferation and extracellular matrix remodeling, which result in irreversible distortion of the lung's architecture. Although the pathogenetic mechanisms remain to be determined, the prevailing hypothesis holds that fibrosis is preceded and provoked by a chronic inflammatory process that injures the lung and modulates lung fibrogenesis, leading to the end-stage fibrotic scar. However, there is little evidence that inflammation is prominent in early disease, and it is unclear whether inflammation is relevant to the development of the fibrotic process. ⋯ Clinical measurements of inflammation fail to correlate with stage or outcome, and potent anti-inflammatory therapy does not improve outcome. This review presents a growing body of evidence suggesting that idiopathic pulmonary fibrosis involves abnormal wound healing in response to multiple, microscopic sites of ongoing alveolar epithelial injury and activation associated with the formation of patchy fibroblast-myofibroblast foci, which evolve to fibrosis. Progress in understanding the fibrogenic mechanisms in the lung is likely to yield more effective therapies.
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Pulmonary fibrosis is a frequent and serious complication of scleroderma whose pathophysiology remains poorly understood. The alveolar structures are infiltrated by activated chronic inflammatory cells, alveolar macrophages and polymorphonuclear neutrophils in particular and these could play a determining role. We have studied the state of activation of alveolar macrophages and monocytes circulating in these patients who presented with scleroderma and interstitial pulmonary involvement and also in healthy subjects. ⋯ The neutrophil alveolitis is accompanied by a breakdown in the equilibrium of elastase-antielastase which could participate in the development of alveolar lesions leading to fibrosis. In addition to the activation of macrophages, there is an activation of monocytes marked by the increase in secretion of interleukin-6 and interleukin-8 in vitro during the progression of the disease of scleroderma. Thus, alveolar inflammation is integrated with the overall systemic inflammation whose causes remain unknown.