特发性肺纤维化的发病机制和临床治疗

摘要

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 inflamma-tory 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.%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。