New approaches to modulating idiopathic pulmonary fibrosis.

New approaches to modulating idiopathic pulmonary fibrosis.
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DOI:
10.1007/s11882-013-0377-5
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发表时间:
2013-12
影响因子:
5.5
通讯作者:
Gomer, Richard H.
Gomer, Richard H.
中科院分区:
医学2区
文献类型:
--
作者:
Gomer, Richard H.

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直到最近,特发性肺纤维化(IPF)一直是一种毁灭性的,通常是致命的疾病,没有有效的治疗方法。在了解疾病生物学方面的新进展包括越来越多的共识,即病变主要由源自常驻成纤维细胞的细胞组成。治疗方法的新发展包括对以前使用的几种治疗方案的建议。积极的一面是,口服药物吡非尼酮已在中国、日本、印度和欧盟批准用于IPF,但尚未在美国批准。控制IPF的其他可能性包括控制胃肠道反流和限制过量的盐摄入。多种潜在的IPF治疗方法正在临床试验中;例如,在1b期试验中,静脉注射正常人血清蛋白血清淀粉样蛋白P (SAP,也称为PTX2)的重组版本改善了IPF患者的肺功能。
Until recently, idiopathic pulmonary fibrosis (IPF) has been a devastating and generally fatal disease with no effective therapeutic. New developments in understanding the biology of the disease include a growing consensus that the lesions are mainly composed of cells that originated from resident fibroblasts. New developments in therapeutics include recommendations against several treatment regimes that have been previously used. On a positive note, the orally available drug pirfenidone has been approved for use in IPF in China, Japan, India, and the European Union, but not yet in the United States. Other possibilities for managing IPF include managing gastrointestinal reflux, and limiting excessive salt intake. A variety of potential therapeutics for IPF are in clinical trials; for instance in a Phase 1b trial, intravenous injections of a recombinant version of the normal human serum protein Serum Amyloid P (SAP, also known as PTX2) improved lung function in IPF patients.
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