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混合结合组织疾病中的抗SMN自身抗体与严重的全身性硬化症表型有关
Hajar El Kamouni1,2, Darya S Jalaledin3, Alexandra Albert4
1Division of Rheumatology, Centre hospitalier de l'Université de Montréal, Montreal, Quebec, Canada.
RMD open
|October 5, 2023
概括
对生存运动神经元 (SMN) 复合体的自身抗体存在于59%的混合结合组织疾病 (MCTD) 患者中. 高位抗SMN自身抗体与严重的全身性硬化症表型有关.
科学领域:
- 免疫学 免疫学 免疫学
- 类风湿病学 类风湿病学
- 这是一种自身免疫力.
背景情况:
- 运动神经元 (SMN) 复合体的生存对于小核核糖核蛋白 (RNP) 组装至关重要.
- 对SMN复合体的自身抗体 (aAbs) 是抗U1RNP+肌炎的新兴生物标志物.
- 了解抗SMN aAbs在混合结合组织疾病 (MCTD) 的临床意义很重要.
研究的目的:
- 在混合结缔组织疾病 (MCTD) 队列中调查抗SMN自身抗体的流行率.
- 为了比较MCTD患者的临床和表型特征,有和没有抗SMN自身抗体.
- 确定抗SMN自身抗体标位与疾病严重程度之间的关联.
主要方法:
- 混合结合组织疾病 (MCTD) 队列的回顾性分析.
- 使用可定位激光珠免疫试验检测抗SMN自身抗体,用于低和高标位的MFI值.
- 使用费舍尔精确测试和后勤回归的临床特征的统计比较.
主要成果:
- 在59%的MCTD队列中 (39名66名患者中) 检测到抗SMN自身抗体.
- 患有抗SMN自身抗体的患者表现出更高的指尖痕 (23%) 和心肌炎 (16%),以及较低的胃肠道干扰 (4%) 的频率.
- 高位数的抗SMN自身抗体与严重的全身性硬化症表型有显著的相关性,包括痕,下肠道干扰,肌炎和心肌炎 (调整后OR 7.79).
结论:
- 反SMN自身抗体在59%的混合结合组织疾病 (MCTD) 患者中普遍存在.
- 抗SMN自身抗体的存在,特别是高位数的存在,与严重的全身性硬化症表型有关.
- 这些发现突出了抗SMN自身抗体作为潜在的生物标志物,用于识别严重的系统性参与MCTD.
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