Idiopathic Pulmonary Fibrosis (IPF): An Overview.

Idiopathic Pulmonary Fibrosis (IPF): An Overview.
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特发性肺纤维化(IPF):概述。

DOI:
10.3390/jcm7080201
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发表时间:
2018-08-06
影响因子:
3.9
通讯作者:
Chaudhuri N
Chaudhuri N
中科院分区:
医学2区
文献类型:
--
作者:
Barratt SL;Creamer A;Hayton C;Chaudhuri N

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特发性肺纤维化(IPF)是一种间质性肺病,其特征为肺部慢性进行性瘢痕形成和普通型间质性肺炎的病理学标志。目前的范例表明肺泡上皮细胞损伤是一个关键的启动因素。在全球范围内,该疾病的发病率正在上升,伴随着高发病率、高死亡率和高经济医疗负担。诊断依赖于多学科团队的方法,排除了间质性肺病的其他原因。近年来,已开发出两种新型抗纤维化疗法吡非尼酮和尼达尼布,为许多IPF患者提供了治疗选择,并在早期临床试验中使用了其他几种药物。目前的努力旨在确定关键的生物标志物,这些生物标志物可能会指导更定制的以患者为中心的医疗保健,以改善这些患者未来的结局。
Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease characterised by chronic, progressive scarring of the lungs and the pathological hallmark of usual interstitial pneumonia. Current paradigms suggest alveolar epithelial cell damage is a key initiating factor. Globally, incidence of the disease is rising, with associated high morbidity, mortality, and economic healthcare burden. Diagnosis relies on a multidisciplinary team approach with exclusion of other causes of interstitial lung disease. Over recent years, two novel antifibrotic therapies, pirfenidone and nintedanib, have been developed, providing treatment options for many patients with IPF, with several other agents in early clinical trials. Current efforts are directed at identifying key biomarkers that may direct more customized patient-centred healthcare to improve outcomes for these patients in the future.
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