Investigational approaches to therapies for idiopathic pulmonary fibrosis.
Investigational approaches to therapies for idiopathic pulmonary fibrosis.
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DOI：
10.1517/13543784.2010.484018
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发表时间：
2010-06
影响因子：
6.1
通讯作者：
Lupher ML Jr
中科院分区：
文献类型：
--
作者：
Gomer RH;Lupher ML Jr
在纤维化疾病中，疤痕组织开始取代正常组织，导致组织功能障碍。例如，在肺纤维化中，类似疤痕组织的病灶在肺部形成，阻碍患者呼吸的能力。这些疾病是发病率和死亡率的重要来源。美国有超过15万人患有某种形式的纤维化肺病，这些疾病的五年死亡率可高达80%。尽管有这么大的未满足的医疗需求，目前还没有fda批准的治疗方法。虽然我们对纤维化疾病的原因和生物学的理解仍然相对较差，但我们在确定主要细胞群和许多可以驱动这一过程的生化介质方面取得了令人印象深刻的进展。因此，基于这些发现正在开发新的治疗方法。本综述回顾了截至2009年底正在研究的实验性治疗特发性肺纤维化（IPF）的方法。读者将获得目前实验性治疗IPF的概述。随着吡非尼酮最近在日本被批准用于IPF，以及处于不同临床开发阶段的丰富的实验性疗法，新的治疗选择的未来看起来很光明。
In fibrosing diseases, scar tissue begins to replace normal tissue, causing tissue dysfunction. For instance, in lung fibrosis, foci of what resembles scar tissue form in the lungs, impeding the ability of patients to breath. These conditions represent a significant source of morbidity and mortality. More than 150,000 people in the US have some form of fibrotic lung disease, and the five-year mortality rate for these diseases can be as high as 80%. Despite this large unmet medical need, there are no FDA-approved therapies. Although our understanding of the causes and the biology of fibrosing diseases remains relatively poor, we have made impressive advances in identifying the major cell populations and many biochemical mediators that can drive this process. As a result, novel therapeutics are being developed based upon these discoveries. This review examines the experimental therapies currently under investigation as of late 2009 for a major class of lung fibrosis called idiopathic pulmonary fibrosis (IPF). The reader will gain an overview of current experimental therapies for IPF. With the recent approval of Pirfenidone in Japan for use in IPF, and a rich pipeline of experimental therapies in various stages of clinical development, the future looks bright for new treatment options.
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DOI：
10.1164/rccm.200906-0964oc
发表时间：
2010-03-15
影响因子：
24.7
作者：
Daniels, Craig E.;Lasky, Joseph A.;Schroeder, Darrell R.
通讯作者：
Schroeder, Darrell R.
影响因子：
5.8
作者：
Failla M;Genovese T;Mazzon E;Gili E;Muià C;Sortino M;Crimi N;Caputi AP;Cuzzocrea S;Vancheri C
通讯作者：
Vancheri C
DOI：
10.1073/pnas.94.12.6307
发表时间：
1997-06-10
影响因子：
11.1
作者：
Chesney, J;Bacher, M;Bucala, R
通讯作者：
Bucala, R
影响因子：
6.5
作者：
Frazier, K;Williams, S;Grotendorst, GR
通讯作者：
Grotendorst, GR
影响因子：
5
作者：
Hetzel, M;Bachem, M;Faehling, M
通讯作者：
Faehling, M