吡非尼酮对结缔组织病相关间质性肺疾病的治疗进展

Research Progress of Pirfenidone for connective tissue disease-associated interstitial lung diseases

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。

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.