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肌炎相关的间歇性肺部疾病:三级中心的经验

Bianca Paulo Correia^1,2, Raquel Campanilho-Marques^1,2, Eduardo Dourado^2,3,4

¹Rheumatology Department, Unidade Local de Saúde Santa Maria (ULSSM), 1649-028 Lisbon, Portugal.

Journal of clinical medicine

|October 26, 2024

概括

在异形性炎症性肌肉病症 (IIM) 中,间歇性肺病 (ILD) 可以通过静脉注射免疫球蛋白 (IVIg) 和阿扎西奥普林 (AZA) 等治疗来稳定或改善. 这项研究显示,在IIM-ILD患者的特定免疫抑制疗法中,肺功能结果呈阳性.

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:

间歇性肺病 (ILD) 是异常性炎症性肌肉病 (IIM) 的常见和严重并发症.
IIM-ILD与增加的死亡率和更差的预后有关.
描述肺部参与和治疗反应对于管理IIM患者至关重要.

研究的目的:

调查IIM患者队列中肺部参与的患病率和特征.
评估自身抗体形状与IIM中的ILD之间的关联.
在IIM-ILD中评估不同免疫抑制治疗方案对肺功能的疗效.

主要方法:

从2016年6月到2024年3月,对198名IIM患者的回顾性分析.
高分辨率计算机断层扫描 (HRCT) 扫描和肺功能测试 (PFTs) 的审查.
肺部感染与自身抗体概况和治疗反应的相关性.

主要成果:

34.8%的IIM患者患有ILD,其中抗合成酶综合征 (ASyS) 和皮肤肌炎是最常见的.
在IIM-ILD患者中,抗Jo1和抗MDA-5抗体更为普遍;抗Mi2较少.
IIM-ILD患者的强迫生命能力 (FVC) 和肺部对一氧化碳的扩散能力 (DLCO) 降低,这些治疗包括IVIg和阿扎西奥普林 (AZA).

结论:

免疫抑制疗法,特别是IVIg和AZA,可以稳定或改善IIM-ILD中的肺功能.
组合疗法在增强肺功能方面显示出有希望的结果.
大多数接受治疗的IIM-ILD患者的HRCT模式保持稳定,这表明治疗有效性.

关键词:

异形性炎症性肌肉病变 (Idiopathic Inflammatory Myopathies) 是一种异形性炎症性肌肉病变 (Idiopathic Inflammatory Myopathies) 的一种疾病.免疫抑制疗法是一种免疫抑制疗法.间歇性肺病间歇性肺病

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