中文摘要：

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。

英文摘要：

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.