异常性肺纤维化
Luca Richeldi1, Harold R Collard2, Mark G Jones3
1Unità Operativa Complessa di Pneumologia, Università Cattolica del Sacro Cuore, Fondazione Policlinico A. Gemelli, Rome, Italy; National Institute for Health Research Southampton Respiratory Biomedical Research Unit and Clinical and Experimental Sciences, University of Southampton, Southampton, UK.
Lancet (London, England)
|April 3, 2017
概括
异常性肺纤维化 (IPF) 是一种渐进的肺部疾病. 最近的进展改变了IPF的理解和管理,两种新疗法为纤维化肺部疾病提供了希望.
科学领域:
- 肺病学
- 纤维化肺部疾病
- 细胞外基质生物学
背景情况:
- 异常性肺纤维化 (IPF) 是一种慢性,渐进的纤维化肺病,其特征是肺气泡结构的破坏.
- 这导致肺部不适应,气体交换受损,呼吸衰竭和死亡率.
- 最近在了解IPF病变方面取得了重大进展.
研究的目的:
- 总结一下当前关于异常性肺纤维化的认识.
- 审查IPF的表现,病理生理和诊断.
- 概述IPF患者可用的治疗方案.
主要方法:
- 对最新的科学文献和临床试验数据的审查.
- 关于IPF病变和治疗策略的综合信息.
- 诊断标准和临床表现的总结
主要成果:
- 人们对IPF病变的理解迅速发展.
- 全球已批准两种疾病修饰疗法.
- IPF研究提供了其他渐进性纤维性肺病的见解.
结论:
- 最近的突破已经改变了IPF的管理.
- 新的治疗方法为IPF患者提供了希望.
- 在IPF的进步作为治疗其他纤维性肺部疾病的模型.
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