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.
出处
《中国医药导报》
CAS
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)
关键词
特发性肺纤维化
研究进展
治疗手段
Idiopathic pulmonary fibrosis
Research progress
Treatment methods
