特发性肺纤维化急性加重的研究进展

摘要

Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) is a sudden, unpredictable clinical deterioration, which emerges in the course of idiopathic pulmonary fibrosis. It is a serious disease with high mortality and not uncommon in clinical practice. So far, the etiology and pathogenesis of AE-IPF is unclear. The existing diagnostic criteria of AE-IPF are controversial and there is still lack of effective Methods for treatment of AE-IPF.%特发性肺纤维化(idiopathic pulmonary fibrosis,IPF)患者病程中出现的突然的、不可预测的临床急剧恶化称为特发性肺纤维化急性加重(acute exacerbations of idiopathic pulmonary fibrosis以下称为AE-IPF).其病情凶险,病死率高,且临床并非少见.目前AE-IPF病因、机制未明,现有的诊断标准尚有争议,缺乏有效的治疗措施,是临床医生面临的诊治难点之一.