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特发性肺纤维化发病机制的研究进展
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Abstract:
特发性肺纤维化(IPF)是一种不明原因导致的慢性、进行性、间质性肺部疾病，以弥漫性肺泡炎和肺泡结构紊乱并最终导致肺间质纤维化为主要特征。IPF预后普遍较差，诊断后的中位生存时间仅为3~5年，约5%~20%的患者病程中可出现急性加重，急性加重后中位生存时间相较之前将进一步减少。目前现有的治疗手段无法彻底治愈IPF，治疗目的主要为延缓疾病进展。本文旨在对现有IPF的致病机制进行回顾与总结，以期为寻找合适的治疗靶点及新的治疗途径提供新的思路。
Idiopathic pulmonary fibrosis (IPF) is an unexplained, chronic, progressive, interstitial lung disease characterized by diffuse alveolitis and alveolar structural disturbances that eventually lead to interstitial fibrosis. The prognosis of IPF is generally poor, with a median survival time of 3~5 years after diagnosis, with acute exacerbations occurring in about 5%~20% of patients. After acute exacerbation, the median survival time will be further reduced compared with the previous one. Currently available treatments do not provide a complete cure for IPF, and the main goal of treatment is to slow down the progression of the disease. The aim of this paper is to review and summarize the existing pathogenic mechanisms of IPF, with a view to providing new ideas for finding suitable therapeutic targets and new therapeutic pathways.
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