Margaritopoulos George A, Vasarmidi Eirini, Antoniou Katerina M
Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK.
Department of Thoracic Medicine and Laboratory of Molecular and Cellular Pneumonology, Interstitial Lung Disease Unit, University Hospital of Heraklion, Heraklion, Greece.
Core Evid. 2016 Jul 1;11:11-22. doi: 10.2147/CE.S76549. eCollection 2016.
The landscape of idiopathic pulmonary fibrosis (IPF) has changed. The significant progress regarding our knowledge on the pathogenesis of the disease together with the experience achieved after a series of negative trials has led to the development of two drugs for the treatment of IPF. Both pirfenidone and nintedanib can slow significantly the rate of disease progression. They are safe with side effects that can be either prevented by close collaboration between health care professionals and patients or treated successfully when they occur, rarely leading to treatment discontinuation. However, there are still few unanswered questions regarding the application of the beneficial results of pharmaceutical trials in the general population of IPF patients. Long-term "real-life" studies are being undertaken to answer these questions. In this article, we focus on the advances that have led to the development of the antifibrotic agents with particular focus on pirfenidone.
特发性肺纤维化（IPF）的局面已发生变化。我们对该疾病发病机制的认识取得了重大进展，同时一系列阴性试验所积累的经验促使了两种治疗IPF的药物得以研发。吡非尼酮和尼达尼布均可显著减缓疾病进展速度。它们安全性良好，其副作用可通过医护人员与患者的密切合作加以预防，或在副作用出现时成功治疗，很少导致治疗中断。然而，关于药物试验的有益结果在IPF患者普通人群中的应用，仍存在一些未解决的问题。正在开展长期的“实际应用”研究以回答这些问题。在本文中，我们重点关注促成抗纤维化药物研发的进展，尤其关注吡非尼酮。
