Therapeutic advances in idiopathic pulmonary fibrosis
Therapeutic advances in idiopathic pulmonary fibrosis
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DOI：
10.7861/clinmedicine.16-1-42
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发表时间：
2016-02-01
影响因子：
4.4
通讯作者：
Hoyles, Rachel K.
中科院分区：
文献类型：
--
作者：
Fraser, Emily;Hoyles, Rachel K.
特发性肺纤维化（IPF）的特征是肺部瘢痕组织的进行性积累，平均预期寿命为2-4年。直到最近，治疗选择有限，主要集中在无效的抗炎治疗、姑息治疗、移植或试验招募。最近该领域的重大进展导致了两种新型抗纤维化药物，吡非尼酮和尼达尼布，它们已被证明可显着减缓IPF的疾病进展。本文概述了IPF的治疗方法、专科中心的作用和专科间质性肺疾病的多学科评价，并探讨了使用这些抗纤维化药物的试验证据和实际考虑。
Idiopathic pulmonary fibrosis (IPF) is characterised by progressive accumulation of scar tissue in the lung and is associated with a median life expectancy of 2-4 years. Until recently, treatment options were limited, focusing on ineffective anti-inflammatory therapy, palliation, transplant or trial recruitment. Significant recent advances in the field have led to two novel anti-fibrotic agents, pirfenidone and nintedanib, which have been shown to significantly slow disease progression in IPF. This article outlines the approach to management of IPF, the role of specialist centres and specialist interstitial lung disease multidisciplinary review, and explores both the trial evidence and practical considerations in the use of these anti-fibrotic agents.