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特发性肺纤维化
Idiopathic Pulmonary Fibrosis


David J. Lederer ... 呼吸系统疾病 • 2018.05.10
相关阅读
• 在理解和治疗重度纤维化疾病方面的进展

间质性肺疾病是一组疾病,其特征是肺泡壁内出现并非因感染或癌症引起的细胞增殖、间质炎症和纤维化或此类表现的组合1。在大多数病例中,间质纤维化是最突出的表型。大多数间质纤维化患者最终得到的诊断是慢性超敏性肺炎(由于接触霉菌或禽类)、肺结节病(一种基础性自身免疫病),如果无法确定病因,则诊断为特发性间质性肺炎(参见术语表)(图1)。最常见的特发性间质性肺炎是特发性肺纤维化(IPF),这是一种原因不明的慢性、进行性的纤维性间质性肺疾病,通常具有特征性的影像学和组织学表现(下文详述),主要发生于老年人2。因为IPF死亡率高,经常被误诊并错误地使用免疫抑制疗法治疗,且现已有可减缓疾病进展的疗法,所以IPF在临床上受到很大的关注。





作者信息

David J. Lederer, M.D., and Fernando J. Martinez, M.D.
From the Departments of Medicine and Epidemiology, Columbia University Irving Medical Center (D.J.L.), and the Department of Medicine, Weill Cornell Medical Center (F.J.M.) — both in New York. Address reprint requests to Dr. Martinez at the Division of Pulmonary and Critical Care Medicine, Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medical College, New York–Presbyterian Hospital/Weill Cornell Medical Center, New York, NY 10021, or at fjm2003@med.cornell.edu.

 

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