特发性肺纤维化急性加重的研究进展

Current progression in acute deteriorations of idiopathic pulmonary fibrosis

特发性肺纤维化（IPF）是一种慢性间质性肺疾病，通常其肺功能逐渐下降。最近发现部分患者在病程中可能出现难以预料、爆发性并且是致命性的快速恶化，患者突然出现临床症状加重，这种没有原因可解释的现象称为IPF急性加重或IPF的加速期。本文就近年来IPF急性加重的定义、病理生理、发病率、发病机制、诊断和治疗等方面的研究进展进行综述。在上述问题中，一些得到证实，但仍有一些问题需进一步研究证实。

The natural history of idipathic pulmonary fibrosis （IPF） has been characterized as a steady,predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have termed acute deteriorations of IPF. This article reviews the current progresses in the definition, pathophysiology, etiology, diagnosis,treatment and prophylaxis of acute deteriorations of IPF. For above questions, some have been proved. But still other problems remain disputable.