Pharmacological Treatment of Idiopathic Pulmonary Fibrosis: Current Approaches, Unsolved Issues, and Future Perspectives.
Pharmacological Treatment of Idiopathic Pulmonary Fibrosis: Current Approaches, Unsolved Issues, and Future Perspectives.
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DOI：
10.1155/2015/329481
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发表时间：
2015
影响因子：
--
通讯作者：
Spagnolo P
中科院分区：
文献类型：
--
作者：
Kreuter M;Bonella F;Wijsenbeek M;Maher TM;Spagnolo P
特发性肺纤维化（IPF）是一种破坏性疾病，5年生存率约为20%。该病主要发生在老年患者中。IPF是一种高度异质性的疾病，其临床病程从长期稳定到快速恶化不等。在过去的十年中，随着对疾病机制的理解沿着得到了更精确的疾病定义，许多高质量的临床试验得以设计和完成。然而，直到最近，IPF基本上是一种无法治愈的疾病。最后，吡非尼酮和尼达尼布（两种具有抗纤维化特性的化合物）已被一致证明可有效降低IPF患者的功能下降和疾病进展。这对患者和医生来说都是一个重大突破，但还有很长的路要走。事实上，吡非尼酮和尼达尼布均不能治愈IPF，大多数患者尽管接受治疗仍继续进展。因此，IPF患者的综合护理，包括管理合并症/并发症和身体虚弱，及时转诊接受姑息治疗，或者在少数高度选择的患者中进行肺移植，仍然至关重要。目前正在测试几种具有高潜力的药物，还有更多药物准备在临床试验中进行评估。
Idiopathic pulmonary fibrosis (IPF) is a devastating condition with a 5-year survival of approximately 20%. The disease primarily occurs in elderly patients. IPF is a highly heterogeneous disorder with a clinical course that varies from prolonged periods of stability to episodes of rapid deterioration. In the last decade, improved understanding of disease mechanisms along with a more precise disease definition has allowed the design and completion of a number of high-quality clinical trials. Yet, until recently, IPF was essentially an untreatable disease. Finally, pirfenidone and nintedanib, two compounds with antifibrotic properties, have consistently proven effective in reducing functional decline and disease progression in IPF. This is a major breakthrough for patients and physicians alike, but there is still a long way to go. In fact, neither pirfenidone nor nintedanib is a cure for IPF, and most patients continue to progress despite treatment. As such, comprehensive care of patients with IPF, including management of comorbidities/complications and physical debility and timely referral for palliative care or, in a small number of highly selected patients, lung transplantation, remains essential. Several agents with high potential are currently being tested and many more are ready to be evaluated in clinical trials.