特发性肺纤维化发病机制及药物研究新进展  

Pathogenesis and Drug Research Progress of Idiopathic Pulmonary Fibrosis

作　　者：刘建[1] 王玥[1] 王玉光[1] LIU Jian;WANG Yue;WANG Yuguang(Department of Respiratory Sciences,Beijing Hospital of Traditional Chinese Medicine,Capital Medical University,Beijing 100010,China)

机构地区：[1]首都医科大学附属北京中医医院呼吸科,北京100010

出　　处：《医学综述》2020年第11期2111-2116,共6页Medical Recapitulate

基　　金：北京市医院管理局“登峰”人才培养计划(DFL20150902)。

摘　　要：特发性肺纤维化(IPF)是一种原因不明、发病机制不清、预后不良的慢性纤维化间质性肺炎。IPF发病机制为持续性肺上皮微损伤和激活、异常组织损伤修复导致的成纤维细胞活化和细胞外基质过度产生。目前IPF仍缺乏的有效治疗方法,吡非尼酮与尼达尼布仅可减缓纤维化进程,而抗纤维化药物的使用仍存在许多问题,包括初始药物选择、药物疗程及联合使用风险等。随着对IPF发病机制和病理过程的深入研究,诞生了许多新的治疗方法,包括减少细胞外基质沉积、抑制酪氨酸激酶、调节免疫等,并且开展了多种用于研发个性化治疗IPF新型药剂的临床药物试验。Idiopathic pulmonary fibrosis(IPF)is a specific form of chronic fibrotic interstitial pneumonia of unknown cause,characterized by unclear pathogenesis and an unfavorable prognosis.The pathogenesis of IPF is persistent pulmonary epithelial microinjury and activation,and abnormal tissue damage repair leading to fibroblast activation and extracellular matrix overproduction.There is still a lack of effective treatment for IPF.Pifenidone and Nintedanib have shown efficacy in slowing fibrosis process,but there are still many problems in the use of anti-fibrosis drugs,including initial drug selection,course of treatment,and risk of combined use,etc.Many new treatments were born along with the further study of the pathogenesis and pathophysiology of IPF,for instance,reducing extracellular matrix deposition,inhibiting tyrosine kinase,regulating immunity,etc.A range of clinical trials have been carried out to develop new agents for personalized treatment of IPF.

关 键 词：特发性肺纤维化 吡非尼酮 尼达尼布 酪氨酸激酶拮抗剂 

分 类 号：R563.9[医药卫生—呼吸系统]