Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Proposal.
Acute Exacerbation of Idiopathic Pulmonary Fibrosis: A Proposal.
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DOI：
10.1007/s13665-013-0065-x
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发表时间：
2013-12
期刊：
影响因子：
--
通讯作者：
Collard, Harold R
中科院分区：
文献类型：
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作者：
Johannson, Kerri;Collard, Harold R
特发性肺纤维化（IPF）的急性加重每年发生在大约10%的患者中，并且是该疾病发病率和死亡率的主要原因。虽然目前定义为特发性急性加重，但IPF急性加重实际上可能有多种原因，特别是感染和误吸。具有临床意义的事件的病理生物学中心是IPF肺弥漫性损伤，在组织病理学上表现为弥漫性肺泡损伤，在生物学上表现为加速的肺泡上皮细胞损伤或修复。基于这些最近的观察结果，我们提出了一种新的IPF急性加重模式，该模式消除了特发性要求，并专注于所涉及的病理生理机制。
Acute exacerbation of idiopathic pulmonary fibrosis (IPF) occurs in roughly 10% of patients annually, and is a leading cause of morbidity and mortality in this disease. While currently defined as idiopathic acute worsenings, acute exacerbations of IPF may in fact have a variety of causes, in particular infection and aspiration. Central to the pathobiology of clinically meaningful events is a diffuse injury to the IPF lung manifest histopathologically as diffuse alveolar damage, and biologically as accelerated alveolar epithelial cell injury or repair. Based on these recent observations, we propose a new paradigm for acute exacerbation of IPF that removes the idiopathic requirement and focuses on the pathophysiological mechanism involved.