Texas A&M University, Department of Biology, College Station, TX 77843, USA.
Expert Opin Investig Drugs. 2010 Jun;19(6):737-45. doi: 10.1517/13543784.2010.484018.
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 breathe. These conditions represent a significant source of morbidity and mortality. More than 150,000 people in the USA have some form of fibrotic lung disease, and the 5-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.
在纤维性疾病中，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，类似于疤痕组织的病灶在肺部形成，阻碍了患者的呼吸能力。这些情况是发病率和死亡率的重要来源。在美国，超过 15 万人患有某种形式的纤维化肺疾病，这些疾病的 5 年死亡率可能高达 80%。尽管存在如此巨大的未满足的医疗需求，但仍没有获得 FDA 批准的治疗方法。尽管我们对纤维性疾病的病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体和许多可以驱动这一过程的生化介质方面取得了令人瞩目的进展。因此，正在根据这些发现开发新的治疗方法。
本综述检查了截至 2009 年底针对一种称为特发性肺纤维化 (IPF) 的主要肺纤维化类别的实验性治疗方法。
读者将获得 IPF 的当前实验性治疗方法的概述。
随着吡非尼酮在日本被批准用于 IPF 的使用，以及各种临床开发阶段的丰富实验性治疗方法，新的治疗选择前景光明。
