Khalil Nasreen, O'Connor Robert
Respiratory Division, Vancouver Coastal Health Research Institute, Vancouver, BC.
CMAJ. 2004 Jul 20;171(2):153-60. doi: 10.1503/cmaj.1030055.
Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal pulmonary fibrotic lung disease. The diagnostic histological changes are called usual interstitial pneumonia and are characterized by histological temporal heterogeneity, whereby normal lung tissue is interspersed with interstitial fibrosis, honeycomb cysts and fibroblast foci. Pulmonary functions show restricted volumes and capacities, preserved flows and evidence of decreased gas exchange. High-resolution computed axial tomography demonstrates evidence of fibrosis and lung remodelling such as honeycomb cysts and traction bronchiectasis. There is no known effective treatment for IPF, but lung transplantation improves survival.
特发性肺纤维化(IPF)是一种进行性致死性肺纤维化疾病。诊断性组织学改变称为普通型间质性肺炎,其特征为组织学时间异质性,即正常肺组织中散布着间质纤维化、蜂窝状囊肿和成纤维细胞灶。肺功能显示肺容积和肺活量受限,气流正常,但气体交换减少。高分辨率计算机断层扫描显示有纤维化和肺重塑的证据,如蜂窝状囊肿和牵拉性支气管扩张。目前尚无已知的有效治疗IPF的方法,但肺移植可提高生存率。