特发性肺纤维化的发病机制和临床治疗  

Pathogenesis and clinical therapy of idiopathic pulmonary fibrosis

作　　者：王恺京[1] 陆英[1] 赵倩[1] 邓生琼[1] 刘翠翠[1,2] 顾颖莹[1,3] 王光学[1] 林忻[1] 俞作仁[1] 

机构地区：[1]同济大学附属东方医院转化医学研究中心,上海200120 [2]大连医科大学,辽宁大连116044 [3]温州医科大学,浙江温州325000

出　　处：《中国医药导报》2015年第12期32-35,39,共5页China Medical Herald

基　　金：国家自然科学基金资助项目(编号81370175)

摘　　要：特发性肺纤维化（IPF）是一种进行性且不可逆肺间质疾病，以弥漫性肺泡炎和肺泡结构紊乱并导致肺间质纤维化为主要特征。 IPF发病率各8/10万～15/10万，占间质性肺病的65%，因为其发病机制不明，治疗手段有限，生存期短，严重危害生命健康。传统观念认为IPF是慢性炎症积累性疾病，但最近研究表明IPF是一种上皮细胞纤维化疾病。激素治疗能够减轻患者症状，目前尚未发现逆转IPF肺纤维化进程的有效手段，晚期IPF患者只能依靠肺移植作为挽救生命的措施。随着医学研究对IPF发病机制的进一步认识及基因治疗等手段的发展，IPF临床治疗措施更加多样化。Idiopathic pulmonary fibrosis （IPF） is a chronic, progressive and lethal fibrotic lung disease, characterized by diffuse alveolitis, profound changes in epithelial cell phenotype and fibroblast proliferation. The incidence of IPF is around 8/10 million-15/10 million, accounting 65% interstitial lung disease. Because of its unclear pathogenesis, the treatments for IPF are limited and causing high rate of mortality. Traditionally, IPF was considered a chronic inflammatory disease, but emerging evidences have considered IPF to be an epithelial-fibroblastic disease. Corticosteroids can relieve IPF patients＇ symptoms, but it is ineffective in reversing the lung damage. Lung transplantation is the last treat-ment for IPF patients. More effective therapeutic ways are becoming avallable for IPF patients following the research progress on pathogenesis of IPF and development of gene therapy.

关 键 词：特发性肺纤维化 研究进展 治疗手段 

分 类 号：R563.9[医药卫生—呼吸系统]