Division of Respiratory Medicine, Infection and Oncology, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan.
Expert Rev Respir Med. 2010 Jun;4(3):301-10. doi: 10.1586/ers.10.32.
Idiopathic pulmonary fibrosis (IPF) is a devastating disease with rare incidence but high mortality, and the pathogenesis of which is still poorly understood. Available treatment options have been empirically applied but evidence-based benefits have not yet been confirmed. Pirfenidone is an antifibrotic agent that is potentially effective for IPF treatment. Preclinical studies have been reported using experimental animal models, which revealed inhibitory effects pn profibrotic and proinflammatory cytokines. Several clinical studies provided promising and reproducible effects for inhibition of IPF disease progression in different nations. The efficacy is demonstrated in patients with mild-to-moderate impairment of pulmonary functions, but not confirmed for patients with severe impairment. Major adverse events are photosensitivity and anorexia, but the treatment was generally safe and well tolerated. In this article, the usefulness and limitations of pirfenidone in IPF treatment are discussed to determine its potential for the management of IPF progression.
特发性肺纤维化（IPF）是一种罕见但死亡率高的破坏性疾病，其发病机制仍不清楚。现有的治疗选择是经验性应用的，但尚未证实有基于证据的益处。吡非尼酮是一种抗纤维化药物，对 IPF 的治疗可能有效。已经有使用实验动物模型的临床前研究报告，这些研究显示对致纤维化和促炎细胞因子有抑制作用。一些临床研究在不同国家提供了抑制 IPF 疾病进展的有前景和可重复的效果。该疗效在肺功能轻度至中度受损的患者中得到证实，但在严重受损的患者中尚未得到证实。主要的不良事件是光过敏和食欲不振，但治疗总体上是安全且耐受良好的。本文讨论了吡非尼酮在 IPF 治疗中的有用性和局限性，以确定其在管理 IPF 进展方面的潜力。
