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[间质性肺疾病——历史发展、现状与未来展望]

[Interstitial lung diseases - historical development, current status, future prospects].

作者信息

Behr J, Costabel U

机构信息

Medizinische Klinik und Poliklinik I, Schwerpunkt Pneumologie, Klinikum der Universität München, München.

出版信息

Pneumologie. 2010 Sep;64(9):573-6. doi: 10.1055/s-0030-1255627. Epub 2010 Sep 8.

Abstract

Sarcoidosis and lung fibrosis were first described by histopathology in the 19th century. Since then a large number of different forms of diffuse parenchymal lung diseases has been identified. Although all these diseases manifest in the lung interstitium, there is a wide range of predominanthy inflammatory to purely fibrotic disease processes. Accordingly, anti-inflammatory treatment is successful in the former, whereas for the latter an effective medical therapy is lacking. Gene technology has recently led to results which have fundamentally changed our understanding of the pathophysiology of fibrosing lung disease. Early and differential diagnosis has much improved with the help of high-resolution computed tomography. Recent clinical trials in patients with idiopathic pulmonary fibrosis have shown at least some effectiveness for the antioxidant treatment approach using high-dosed N-acetylcysteine as well as for the use of pirfenidone. Importantly, these studies have proven that well-designed treatment trials are feasible in this patient population. This has kindled the hope that, based on a better understanding of the pathophysiology, new targeted therapies will prove to be successful in the future.

摘要

结节病和肺纤维化在19世纪首次通过组织病理学被描述。从那时起,已发现大量不同形式的弥漫性实质性肺疾病。尽管所有这些疾病都在肺间质中表现出来,但从主要是炎症性到纯粹纤维化的疾病过程范围很广。因此,抗炎治疗在前者中是成功的,而对于后者则缺乏有效的药物治疗。基因技术最近取得的成果从根本上改变了我们对纤维化肺病病理生理学的理解。借助高分辨率计算机断层扫描,早期和鉴别诊断有了很大改善。最近针对特发性肺纤维化患者的临床试验表明,使用高剂量N-乙酰半胱氨酸的抗氧化治疗方法以及使用吡非尼酮至少有一定效果。重要的是,这些研究证明精心设计的治疗试验在该患者群体中是可行的。这燃起了一种希望,即基于对病理生理学的更好理解,新的靶向治疗在未来将被证明是成功的。

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