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- W2323146593 abstract "In Brief Idiopathic pulmonary fibrosis (IPF), with the histologic correlate of usual interstitial pneumonia, has an almost uniformly poor prognosis when compared with other idiopathic interstitial pneumonias. To date, therapeutic attempts, including corticosteroids and immunosuppressive agents, have focused on targeting the inflammatory component of the disease with limited success; only 10% of patients derive significant physiological benefit with this approach. Systematic evaluation of different therapeutic regimens has previously been hampered by a nonstandardized approach to trial design, diagnostic criteria, and treatment protocols. The pathogenesis of IPF has been classically described as a predominantly inflammatory process, in response to an environmental trigger in a genetically susceptible individual, with subsequent fibrogenesis. The relative lack of efficacy of anti-inflammatory therapy has prompted an evolution in our understanding of pathogenesis, with focus on epithelial injury, dysregulation of wound healing, and the fibroblastic focus. Recent therapeutic approaches include antifibrogenic agents such as interferon-γ, pirfenidone, and bosentan, and antioxidant therapies such as N-acetylcysteine, whereas many others are at preliminary stages of laboratory and clinical evaluation. In light of recent reclassification of the idiopathic interstitial pneumonias, this review explores the evolving theories of pathogenesis of IPF, the evidence supporting traditional anti-inflammatory and recent antifibrotic and antioxidant therapies, the idiosyncrasies of past study design, and the need for standardized treatment guidelines. At present, the course of IPF remains relentlessly progressive. The development of new therapeutic approaches, recruitment into well-constructed large multicenter trials, and ongoing laboratory and clinical research brings renewed enthusiasm to this devastating disease. Idiopathic pulmonary fibrosis carries a poor prognosis despite current anti-inflammatory and immunosuppressive therapies. This review explores the evolving theories of pathogenesis, the evidence supporting traditional anti-inflammatory and recent antifibrotic and antioxidant therapies, the idiosyncrasies of past study design, and the need for standardized treatment guidelines." @default.
- W2323146593 created "2016-06-24" @default.
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- W2323146593 date "2006-01-01" @default.
- W2323146593 modified "2023-09-28" @default.
- W2323146593 title "Treatment of Idiopathic Pulmonary Fibrosis" @default.
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- W2323146593 doi "https://doi.org/10.1097/01.cpm.0000198144.90287.1f" @default.
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