Acute exacerbations of idiopathic pulmonary fibrosis
Acute exacerbations of idiopathic pulmonary fibrosis
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DOI：
10.1164/rccm.200703-463pp
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发表时间：
2007-10-01
影响因子：
24.7
通讯作者：
Martinez, Fernando J.
中科院分区：
文献类型：
--
作者：
Collard, Harold R.;Moore, Bethany B.;Martinez, Fernando J.
特发性肺纤维化（IPF）的自然史特征为肺功能随时间稳定、可预测的下降。最近的证据表明，一些患者可能会经历一个更陡峭的过程，相对稳定的时期，然后是呼吸状态的急性恶化。许多急性恶化的病因不明，被称为IPF急性加重。这一观点是总结IPF急性加重知识现状的国际努力的结果。IPF急性加重定义为基础IIPF患者发生急性、临床显著性恶化，原因不明。建议的诊断标准包括主观恶化超过30天或更少，新的双侧影像学混浊，以及没有感染或其他可识别的病因。感染，细胞生物学紊乱，凝血和遗传学的潜在病理生物学作用进行了讨论，并提出了未来的研究方向。
The natural history of idiopathic 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 been termed acute exacerbations of IPF. This perspective is the result of an international effort to summarize the current state of knowledge regarding acute exacerbations of IPF. Acute exacerbations of IPF are defined as acute, clinically significant deteriorations of unidentifiable cause in patients with underlying IIPF. Proposed diagnostic criteria include subjective worsening over 30 days or less, new bilateral radiographic opacities, and the absence of infection or another identifiable etiology. The potential pathobiological roles of infection, disordered cell biology, coagulation, and genetics are discussed, and future research directions are proposed.