肺纤维化的转化研究。
Translational research in pulmonary fibrosis.
机构信息
Interstitial Lung Disease Program, Center for Advanced Heart & Lung Disease, Department of Medicine, Baylor University Medical Center at Dallas, Dallas, Texas; Department of Internal Medicine, Texas A&M University College of Medicine.
Department of Medicine, University of Colorado School of Medicine, Aurora, Colorado.
出版信息
Transl Res. 2019 Jul;209:1-13. doi: 10.1016/j.trsl.2019.02.001. Epub 2019 Feb 5.
Abstract
Pulmonary fibrosis refers to the development of diffuse parenchymal abnormalities in the lung that cause dyspnea, cough, hypoxemia, and impair gas exchange, ultimately leading to respiratory failure. Though pulmonary fibrosis can be caused by a variety of underlying etiologies, ranging from genetic defects to autoimmune diseases to environmental exposures, once fibrosis develops it is irreversible and most often progressive, such that fibrosis of the lung is one of the leading indications for lung transplantation. This review aims to provide a concise summary of the recent advances in our understanding of the genetics and genomics of pulmonary fibrosis, idiopathic pulmonary fibrosis in particular, and how these recent discoveries may be changing the clinical approach to diagnosing and treating patients with fibrotic interstitial lung disease.
摘要
肺纤维化是指肺部弥漫性实质异常导致呼吸困难、咳嗽、低氧血症和气体交换受损，最终导致呼吸衰竭。尽管肺纤维化可能由多种潜在病因引起，包括遗传缺陷、自身免疫性疾病和环境暴露等，但一旦纤维化形成，它就是不可逆转的，而且通常是进行性的，因此肺纤维化是肺移植的主要指征之一。本文旨在简要总结肺纤维化（特别是特发性肺纤维化）遗传学和基因组学研究的最新进展，以及这些新发现如何改变我们诊断和治疗纤维性间质性肺疾病患者的临床方法。
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本文引用的文献
1
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Nat Commun. 2019 Apr 5;10(1):1561. doi: 10.1038/s41467-019-09381-w.
2
Muc5b overexpression causes mucociliary dysfunction and enhances lung fibrosis in mice.Muc5b 过表达导致小鼠黏液纤毛功能障碍和肺纤维化增强。
Nat Commun. 2018 Dec 18;9(1):5363. doi: 10.1038/s41467-018-07768-9.
3
MUC5B Promoter Variant and Rheumatoid Arthritis with Interstitial Lung Disease.MUC5B 启动子变异与伴有间质性肺病的类风湿关节炎。
N Engl J Med. 2018 Dec 6;379(23):2209-2219. doi: 10.1056/NEJMoa1801562. Epub 2018 Oct 20.
4
Metal-Induced Pulmonary Fibrosis.金属诱导性肺纤维化。
Curr Environ Health Rep. 2018 Dec;5(4):486-498. doi: 10.1007/s40572-018-0219-7.
5
Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.特发性肺纤维化诊断。美国胸科学会/欧洲呼吸学会/日本呼吸学会/拉丁美洲胸科学会临床实践指南。
Am J Respir Crit Care Med. 2018 Sep 1;198(5):e44-e68. doi: 10.1164/rccm.201807-1255ST.
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