特发性肺纤维化急性加重的专家共识解读

Advances in acute exacerbation of idiopathic pulmonary fibrosis

特发性肺纤维化是一种局限在肺脏的慢性、进行性、纤维化件间质性肺疾病,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,治疗措施也在进步。本文将对AE-IPF的国际专家共识进一步解读,以期提高临床医师对AE-IPF的认识并指导其临床诊疗。

Idiopathic pulmonary fibrosis is a chronic, progressive and fibrosis disease with a poor prog nosis. Some patients will experience acute exacerbation and rapid deterioration and die. The etiology nnd pathogenesis of acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) are still unclear. The definition and diagnostic criteria of AE-IPF are updated recently. This paper makes a brief i nt rod net i on to the international experts consensus on A E-IPF. so as to improve clinicians' un dersta nding of AEIPF and guide its clin icnl diagnosis and treat ment.