与系统性硬化症相关的间歇性肺部疾病
Valentine Mismetti1, Salim Si-Mohamed2,3, Vincent Cottin1,4
1Department of Respiratory Medicine, National Coordinating Reference Centre for Rare Pulmonary Diseases, ERN-LUNG, Louis Pradel Hospital, Hospices Civils de Lyon, Lyon, France.
概括
系统性硬化症 (SSc) 是一种罕见的自身免疫性疾病. 本综述涵盖了诊断和管理SSc相关间歇性肺病 (SSc-ILD) 的最新证据,这是死亡的主要原因.
科学领域:
- 类风湿病学 类风湿病学
- 肺部病理学 肺部病理学
- 免疫学 免疫学 免疫学
背景情况:
- 系统性硬化症 (SSc) 是一种罕见的自身免疫性疾病,其特征是血管病变,纤维化和免疫媒介炎症.
- 间歇性肺病 (ILD) 在SSc的患病率在25-95%之间,是导致死亡的主要原因.
- 肺部表现显著影响SSc的发病率和死亡率.
研究的目的:
- 为诊断和管理SSc-ILD提供最新证据的概述.
- 总结当前的治疗选择和SSc-ILD管理的新兴专家共识.
- 为SSc-ILD患者提供基于证据的实践指导临床医生.
主要方法:
- 审查最近的随机对照试验和专家共识.
- 对SSc-ILD的诊断方法的分析.
- 对治疗干预措施的评估,包括mycophenolate,rituximab,tocilizumab和 nintedanib.
主要成果:
- 系统性硬化症的SSc-ILD是系统性硬化症结果的关键决定因素.
- 几种药物在治疗SSc-ILD方面表现出有效性.
- 目前正在进行的研究旨在完善管理战略和指导方针.
结论:
- 优化SSc-ILD的治疗管理至关重要.
- 基于证据的临床实践指南正在为SSc-ILD.出现.
- 持续的研究是必要的,以解决SSc-ILD护理的剩余不确定性.
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