在结合性组织疾病与关联的间歇性肺部疾病中的新型治疗方法
Erica Mulcaire-Jones1, Janelle Vu Pugashetti2, Justin M Oldham2
1Division of Rheumatology, Internal Medicine, University of Michigan, Ann Arbor, Michigan.
与结缔组织疾病相关的间歇性肺病 (CTD-ILD) 可以进展并增加死亡率. 目前的治疗包括免疫抑制,抗纤维素药物和对进展病例的移植.
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:
- 连接组织疾病 (CTD) 是一种影响多个器官的自身免疫性疾病,经常通过间歇性肺病 (ILD) 影响肺部.
- 与CTD相关的ILD (CTD-ILD) 呈现出多种不同的临床过程,与与较高死亡率相关的渐进模式.
- 有限的高质量的临床试验数据存在CTD-ILD治疗,大多数证据来自系统性硬化症相关的ILD.
研究的目的:
- 审查连接组织疾病患者间歇性肺病的当前治疗选择.
- 总结关于特定CTD的ILD治疗策略的数据,包括全身性硬化症,类风湿性关节炎,肌炎和原发性Sjögren综合征.
主要方法:
- 文献综述侧重于CTD-ILD的治疗数据.
- 对免疫抑制,抗纤维素药物和移植选择的现有证据的分析.
主要成果:
- 免疫抑制是CTD-ILD的主要治疗方法.
- 抗纤维素药物,干细胞移植和肺移植被认为是疾病的进展.
- 对于系统性硬化症相关的ILD,治疗数据最为稳健.
结论:
- 有效管理CTD-ILD需要了解疾病的过程和可用的治疗选择.
- 需要进一步的研究,以建立各种CTD-ILD亚型的基于证据的治疗指南.
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