特发性肺纤维化急性加重的最新研究进展

Recent advances in acute exacerbation of idiopathic pulmonary fibrosis

特发性肺纤维化（idiopathic pulmonary fibrosis,IPF）是一种病因不明的慢性进展性纤维化型间质性肺炎,组织学表现为普通型间质性肺炎.IPF通常是缓慢进展,但部分IPF患者可以出现急剧、原因不明的临床明显恶化,称之为IPF急性加重.IPF急性加重难以预测、病死率高、缺乏有效的治疗措施.本文就近年来IPF急性加重的发病率、危险因素、病理生理、发病机制、诊断和治疗等方面的研究进展进行综述.

Idiopathic pulmonary fibrosis （IPF） is defined as a specific form of chronic,progressive fibrosing interstitial pneumonia of unknown cause,characterized by a histological pattern of usual interstitial pneumonia.The clinical course of IPF is usually chronic,but some patients may experience episodes of acute respiratory worsening.These episodes of idiopathic acute deterioration have been termed as acute exacerbations of IPF （AE-IPF）.AE-IPF may be less predictable,associated with a high mortality rate and no effective therapy.This article reviews the incidence,risk factors,pathophysiology,pathogenesis,diagnosis and treatment progress in AE-IPF.