特发性肺纤维化
医学
生物标志物
寻常性间质性肺炎
疾病
间质性肺病
免疫学
肺纤维化
肺
生物标志物发现
纤维化
生物信息学
病理
蛋白质组学
内科学
生物
基因
生物化学
作者
Fotios Drakopanagiotakis,Lukasz Wujak,Małgorzata Wygrecka,Philipp Markart
出处
期刊:Matrix Biology
[Elsevier]
日期:2018-08-01
卷期号:68-69: 404-421
被引量:86
标识
DOI:10.1016/j.matbio.2018.01.023
摘要
Idiopathic pulmonary fibrosis (IPF) is a chronic, debilitating, fibrotic lung disease leading to respiratory failure and ultimately to death. Being the prototype of interstitial lung diseases, IPF is characterized by marked heterogeneity regarding its clinical course. Despite significant progress in the understanding of its pathogenesis, we still cannot reliably predict the course of the disease and the response to treatment of an individual patient. Non-invasive biomarkers, in particular serum biomarkers, for the (early) diagnosis, differential diagnosis, prognosis and prediction of therapeutic response are urgently needed. Numerous molecules involved in alveolar epithelial cell injury, fibroproliferation and matrix remodeling as well as immune regulation have been proposed as potential biomarkers. Furthermore, genetic variants of TOLLIP, MUC5B, and other genes are associated with a differential response to treatment and with the development and/or the prognosis of IPF. Additionally, the bacterial signature in IPF lungs, as shown from microbiome analyses, as well as mitochondrial DNA seem to have promising roles as biomarkers. Moreover, combination of multiple biomarkers may identify comprehensive biomarker signatures in IPF patients. However, there is still a long way until these potential biomarkers complete or substitute for the clinical and functional parameters currently available for IPF.
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