与Sjögren病相关的异常性炎症性肌肉病变:一个独特的临床实体的特征
Franz Felix Konen1, Yunus Emre Güzeloglu1, Tabea Seeliger1
1Department of Neurology, Hannover Medical School, Hannover, Germany.
Frontiers in immunology
|September 25, 2025
概括
异常性炎症性肌肉病变 (IIM) 与Sjögren一起发生
科学领域:
- 类风湿病学 类风湿病学
- 免疫学 免疫学 免疫学
- 神经学 神经学
背景情况:
- 异形性炎症性肌肉病变 (IIM) 和斯约格伦病 (SjD) 可以同时发生,但关于它们的联合表现和治疗结果的数据有限.
- 了解IIM和SjD之间的相互作用对于优化患者护理和治疗策略至关重要.
研究的目的:
- 调查IIM和SjD同时存在的患者的临床特征,治疗反应和结果.
- 将这些特征与IIM患者没有SjD的对照组进行比较.
主要方法:
- 对23名生物检查确诊的IIM和SjD患者的回顾性分析,与24名年龄和性别匹配的IIM对照进行了比较.
- 评估临床,电生理学和免疫学数据,以及使用经过验证的指数 (ESSDAI,ACR/EULAR肌炎反应标准) 的治疗方法和结果.
主要成果:
- 与对照组相比,IIM-SjD患者表现出不对称的肌肉虚弱率 (78%) 和非肌肉表现的比例 (52%) 较高.
- 患有IIM-SjD的患者接受了更多的免疫抑制疗法 (中位数为3),包括频繁使用抗CD20 (30%).
- 经过80个月的中位数随访后,IIM-SjD患者表现出更好的结果,由较低的ESSDAI和较高的ACR/EULAR反应得分表明.
- 纳入体肌炎 (IBM) 被确定为随访时ESSDAI分数较高的预测因素.
结论:
- IIM和SjD的共存与明显的临床特征有关,包括不对称的肌肉虚弱和肌肉外参与.
- 积极的免疫抑制,特别是抗CD20疗法,似乎有利于管理IIM-SjD,特别是在多或皮肤肌炎亚型.
- 包容体肌肉炎是一个挑战,与IIM-SjD的背景下更高的疾病活性相关.
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