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吡非尼酮:对特发性肺纤维化有显著治疗效果。

Pirfenidone: significant treatment effects in idiopathic pulmonary fibrosis.

作者信息

Hilberg Ole, Simonsen Ulf, du Bois Roland, Bendstrup Elisabeth

机构信息

Aarhus University Hospital, Respiratory Diseases and Allergology, Aarhus, Denmark.

出版信息

Clin Respir J. 2012 Jul;6(3):131-43. doi: 10.1111/j.1752-699X.2012.00302.x.

DOI:10.1111/j.1752-699X.2012.00302.x
PMID:22697264
Abstract

Pirfenidone has been shown in three recently published trials to slow down the progression of the devastating interstitial lung disease, idiopathic pulmonary fibrosis (IPF). The precise mechanisms that initiate and perpetuate the histopathological process leading to lung fibrosis in IPF are still uncertain, but increased concentrations of reactive oxidative species and fibrogenetic factors have been observed in the pulmonary tissue of patients. Although the exact mechanisms of its action are unknown, pirfenidone is a small molecule with antifibrotic and some hydroxyl scavenger properties that has recently been approved in Europe and elsewhere for the treatment of IPF. Along with the new ATS/ERS/JRS/ALAT 2011 statement for 'Evidence Based Guidelines for Diagnosis and Management', there is now a more profound basis for offering IPF patients an evidence-based evaluation and treatment. This review summarizes the background to the recommended use of pirfenidone for the treatment of IPF.

摘要

在最近发表的三项试验中,已证实吡非尼酮可减缓严重的间质性肺病——特发性肺纤维化(IPF)的进展。引发并维持导致IPF肺纤维化的组织病理学过程的确切机制仍不确定,但在患者的肺组织中已观察到活性氧化物质和纤维生成因子浓度升高。尽管其作用的确切机制尚不清楚,但吡非尼酮是一种具有抗纤维化和一些羟基清除剂特性的小分子,最近已在欧洲和其他地区获批用于治疗IPF。随着新的美国胸科学会/欧洲呼吸学会/日本呼吸学会/拉丁美洲胸科协会2011年“基于证据的诊断和管理指南”声明的发布,现在有了更坚实的基础为IPF患者提供基于证据的评估和治疗。本综述总结了推荐使用吡非尼酮治疗IPF的背景。

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