Hyzy Robert, Huang Steven, Myers Jeffrey, Flaherty Kevin, Martinez Fernando
Division of Pulmonary Medicine, University of Michigan Medical Center, Ann Arbor, MI 48109, USA.
Chest. 2007 Nov;132(5):1652-8. doi: 10.1378/chest.07-0299.
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).
Review of the current literature pertaining to AE-IPF.
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.
AE of IPF is a complication that demands additional careful study to clarify its relationship to the clinical course of patients with IPF.
特发性肺纤维化（IPF）患者的临床病程通常以肺功能随时间下降为特征。越来越多的患者被认为会出现急性且往往致命的临床恶化，即所谓的IPF急性加重（AE-IPF）。
回顾当前与AE-IPF相关的文献。
急性加重的定义为在无心力衰竭或感染的情况下，急性起病的呼吸困难（<1个月）、低氧血症恶化以及进行性浸润。胸部CT扫描可见新的磨玻璃样浸润影，组织病理学显示在普通间质性肺炎背景上叠加有弥漫性肺泡损伤。其发病率未知，由于难以排除感染作为病因，以及报告系列中存在的报告偏倚（仅纳入活检患者或仅纳入死亡病例，或排除晚期疾病患者）而受到阻碍。预后较差，但可能受诊断不准确的影响。使用抗炎疗法（如皮质类固醇）或抗凝治疗未经证实，尚未得到充分研究。
IPF急性加重是一种并发症，需要进一步仔细研究以阐明其与IPF患者临床病程的关系。
