Current and emerging drugs for idiopathic pulmonary fibrosis

被引:21
作者
Gharaee-Kermani, Mehrnaz [1 ]
Hu, Biao [1 ]
Thannickal, Victor J. [1 ]
Phan, Sern. H. [1 ]
Gyetko, Margaret R. [1 ]
机构
[1] Univ Michigan, Sch Med, Dept Internal Med, Div Pulm & Crit Care Med, Ann Arbor, MI 48109 USA
基金
美国国家卫生研究院;
关键词
angiogenesis; antifibrotic agents; anti-inflammatory agents; fibrocytes; idiopathic pulmonary fibrosis; IFN-gamma; inflammation; myofibroblasts; N-acetylcysteine; pathogenesis; pirfenidone; repair;
D O I
10.1517/14728214.12.4.627
中图分类号
R9 [药学];
学科分类号
1007 ;
摘要
Idiopathic pulmonary fibrosis is a chronic, progressive and often fatal form of interstitial lung disease. It is characterized by injury with loss of lung epithelial cells and abnormal tissue repair, resulting in abnormal accumulation of fibroblasts and myofibroblasts, deposition of extracellular matrix and distortion of lung architecture, leading to respiratory failure. This lethal lung disorder continues to pose major clinical challenges as an effective therapeutic regimen has yet to be developed. In this report, therapeutic strategies are reviewed, including the use of antifibrotic agents, inhibition of cytokines, leukotrienes and cytokines receptors, and molecular targeting of specific signaling pathways during fibrotic processes and angiogenesis. This article examines the body of evidence supporting present therapies and reviews the newer agents being tested in patients with idiopathic pulmonary fibrosis.
引用
收藏
页码:627 / 646
页数:20
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