吡非尼酮对结缔组织病相关间质性肺疾病的治疗进展

doi:10.12092/j.issn.1009-2501.2020.05.016

摘要/Abstract

摘要： 吡非尼酮(pirfenidone, PFD)是新型的抗纤维化药物,在多个临床试验中证实能够延缓特发性肺纤维化(idiopathic pulmonary fibrosis, IPF)患者肺功能下降速度,是治疗IPF的一线药物。与IPF发病机制相似的结缔组织病相关间质性肺疾病(connective tissue disease-associated interstitial lung diseases, CTD-ILD)的临床发生率较高,临床症状表现不明显,肺间质病变可能是唯一或最初的表现,临床表现异质性大,误诊率较高,同时也是导致患者死亡的重要原因之一,目前尚缺乏统一有效的指南或共识。有研究表明抗纤维化新药吡非尼酮在CTD-ILD的治疗中表现出一定潜力,本文对吡非尼酮治疗CTD-ILD的相关研究进展进行综述。

关键词: 关键词吡非尼酮, 结缔组织病, 间质性肺疾病

Abstract: Pirfenidone is an anti-fibrotic drug that has been shown to reduce the decline of lung function in patients with idiopathic pulmonary fibrosis (IPF) in multiple clinical trials, and has become first-line drugs for the treatment of IPF. Similar to the pathogenesis of IPF , clinical incidence of connective tissue disease-associated interstitial lung diseases (CTD-ILD) is high, and the clinical performance is not obvious. ILD may be the only or the original manifestation. Thus the clinical heterogeneity and the misdiagnosis rate are relatively high. Also, CTD-ILD is one of the important reasons that resulted in the death of patients. Currently, the clinical diagnosis and treatment of CTD-ILD still lack effective guide or unified agreement. Studies have shown that anti fibrosis drug pirfenidone shows some potential in the treatment of CTD-ILD. Here we summarize the research advances of pirfenidone in the treatment of CTD-ILD.

Key words: pirfenidone, conective tissue disease, interstitial lung disease

中图分类号:

R563
R974

王潇, 岳红梅, 王若利, 孙金英, 李乐, 刘睿超. 吡非尼酮对结缔组织病相关间质性肺疾病的治疗进展[J]. 中国临床药理学与治疗学, 2020, 25(5): 584-590.

WANG Xiao, YUE Hongmei, WANG Ruoli, SUN Jinying, LI Le, LIU Ruichao. Research Progress of Pirfenidone for connective tissue disease-associated interstitial lung diseases[J]. Chinese Journal of Clinical Pharmacology and Therapeutics, 2020, 25(5): 584-590.

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