Acute exacerbation of idiopathic pulmonary fibrosis
Acute exacerbation of idiopathic pulmonary fibrosis
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DOI：
10.1378/chest.07-0299
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发表时间：
2007-11-01
期刊：
影响因子：
9.6
通讯作者：
Martinez, Fernando
中科院分区：
文献类型：
--
作者：
Hyzy, Robert;Huang, Steven;Martinez, Fernando
背景：特发性肺纤维化（IPF）患者的临床病程通常以肺功能随时间的下降为特征。越来越多的患者被认为具有急性且通常是致命的临床恶化，称为IPF急性加重（AE-IPF）。方法：回顾目前有关AE-IPF的文献。结果：急性加重定义为急性发作呼吸困难（< 1个月），缺氧恶化，在没有心力衰竭或感染的情况下出现进行性浸润。胸部CT扫描可见新的磨玻璃浸润，弥漫性肺泡损伤叠加在组织病理学上明显的常规间质性肺炎背景上。发病率尚不清楚，而且由于难以排除感染的原因，以及由于报告系列中只包括活检患者或只包括死亡，或不包括晚期疾病患者而存在的报告偏差，发病率受到阻碍。预后差，但可能受到诊断不准确的影响。抗炎治疗，如皮质类固醇，或抗凝治疗是未经证实的，尚未得到充分的研究。结论：IPF的AE是一种并发症，需要进一步仔细研究以阐明其与IPF患者临床病程的关系。
Background: The clinical course of patients with idiopathic pulmonary Fibrosis (IPF) is generally marked by a decline in pulmonary function over time. Increasingly, patients have been recognized as having an acute, and often fatal, clinical deterioration, termed an acute exacerbation of IPF (AE-IPF).Methods: Review of the current literature pertaining to AE-IPF.Results: Acute exacerbations are defined by an acute onset of dyspnea (< 1 month) with worsening hypoxia and progressive infiltrates seen in the absence of heart failure or infection. New ground-glass infiltrates are seen on chest CT scans with diffuse alveolar damage superimposed on a background of usual interstitial pneumonia that is evident on histopathology. The incidence is unknown and is impeded by difficulties in eliminating infection as a cause, as well as by reporting biases contained in reported series introduced by including only biopsied patients or only deaths, or by excluding patients with advanced disease. Prognosis is poor but may be influenced by diagnostic inaccuracy. Treatment with antiinflammatory therapies, such as corticosteroids, or with anticoagulation are unproven and have not as yet been fully studied.Conclusions: AE of IPF is a complication that demands additional careful study to clarify its relationship to the clinical course of patients with IPF.