Department of Medicine, University of California San Francisco, San Francisco, California, USA.
Am J Respir Crit Care Med. 2011 Feb 15;183(4):431-40. doi: 10.1164/rccm.201006-0894CI. Epub 2010 Oct 8.
Idiopathic pulmonary fibrosis (IPF) is a progressive, life-threatening, interstitial lung disease of unknown etiology. The median survival of patients with IPF is only 2 to 3 years, yet some patients live much longer. Respiratory failure resulting from disease progression is the most frequent cause of death. To date we have limited information as to predictors of mortality in patients with IPF, and research in this area has failed to yield prediction models that can be reliably used in clinical practice to predict individual risk of mortality. The goal of this concise clinical review is to examine and summarize the current data on the clinical course, individual predictors of survival, and proposed clinical prediction models in IPF. Finally, we will discuss challenges and future directions related to predicting survival in IPF.
特发性肺纤维化（IPF）是一种病因不明的进行性、致命性间质性肺疾病。IPF 患者的中位生存时间仅为 2 至 3 年，但有些患者的生存时间要长得多。疾病进展导致的呼吸衰竭是最常见的死亡原因。迄今为止，我们对 IPF 患者死亡预测因素的了解有限，该领域的研究未能产生可在临床实践中可靠用于预测个体死亡风险的预测模型。本简明临床综述的目的是检查和总结 IPF 中目前关于临床病程、生存个体预测因素和拟议临床预测模型的资料。最后，我们将讨论与预测 IPF 生存相关的挑战和未来方向。
