特发性肺纤维化急性加重的研究进展

Advances in acute exacerbation of idiopathic pulmonary fibrosis

【摘要】特发性肺纤维化是一种慢性进展性疤痕性疾病，预后差。部分患者可以出现急性加重，导致病情迅速恶化死亡。特发性肺纤维化急性加重（AE-IPF）发生的原因及发病机制未明，对于AE-IPF的定义和诊断标准也不断地更新，治疗措施有限。本文将对AE-IPF的发病机制、定义、临床特征、诊断标准和治疗手段的研究进展作一综述。

Idiopathic pulmonary fibrosis is a chronic, progressive and scaring disease with a poor prognosis. Some patients will experience acute exacerbation and rapid deterioration and die. The etiology and pathogenesis of acute exacerbation of idiopathic pulmonary fibrosis （AE-IPF） are still unclear. The definition and diagnostic criteria of AE-IPF were updated recently. There is no special effective treatment for AE-IPF till now. This article reviews the research advances in pathogenesis, definition, clinical manifestation, diagnostic criteria and treatment of AE IPF.