Ruhrlandklinik Essen, Universitätsklinik Tüschener Weg 40, Essen, Germany.
Eur Respir Rev. 2011 Sep 1;20(121):201-7. doi: 10.1183/09059180.00002011.
Currently, there are no approved pharmacological treatments for the management of patients with idiopathic pulmonary fibrosis (IPF) in the USA or Europe. Pirfenidone is an orally bio-available small molecule that exhibits antifibrotic and anti-inflammatory properties in a variety of in vitro and animal models. Pirfenidone has been evaluated in four randomised, double-blind, placebo-controlled clinical trials conducted in Japan, North America and Europe. The totality of the data from these trials indicates that pirfenidone is able to reduce the rate of decline in lung function, measured as change in per cent predicted forced vital capacity (FVC) or vital capacity. There was also an effect on secondary end-points of progression free survival, categorical change in per cent predicted FVC, and the 6-min walk test. A recent meta-analysis of the three phase III studies in IPF demonstrated that pirfenidone significantly reduced the risk of disease progression by 30%. The efficacy of pirfenidone is associated with an acceptable tolerability and safety profile.
目前，在美国和欧洲，尚无批准的药物疗法可用于治疗特发性肺纤维化（IPF）患者。吡非尼酮是一种口服生物利用的小分子，在各种体外和动物模型中具有抗纤维化和抗炎特性。吡非尼酮已在日本、北美和欧洲进行的四项随机、双盲、安慰剂对照临床试验中进行了评估。这些试验的全部数据表明，吡非尼酮能够降低肺功能下降的速度，用预计用力肺活量（FVC）或肺活量的百分比变化来衡量。无进展生存率、预计 FVC 百分比的分类变化和 6 分钟步行测试等次要终点也有影响。最近对 IPF 的三项 III 期研究的荟萃分析表明，吡非尼酮可显著降低 30%的疾病进展风险。吡非尼酮的疗效与可接受的耐受性和安全性相关。
