特发性肺纤维化急性加重的研究进展

Research progress on acute exacerbation of idiopathic pulmonary fibrosis

特发性肺纤维化(IPF)患者病程中出现的突然的临床恶化称为特发性肺纤维化急性加重(AE-IPF)。IPF患者急性加重病情进展快、发病率逐年增高,其预后差,是IPF患者病死的主要原因。目前AE-IPF的病因及发病机制不明,临床诊断困难,且目前无特效的治疗方法,是临床医生面临的诊治难点之一。

Acute exacerbations of idiopathic pulmonary fibrosis（AE-IPF） is a sudden clinical deterioration in the disease course of IPF, whose incidence is increasing year by year. AE-IPF has rapid progression and poor prognosis,and becomes the main cause of death in patients with IPF. So far, the etiology and pathogenesis of AE-IPF is unclear.Difficulties in diagnosis and lack of effective treatments for AE-IPF are the problems faced by doctors.