在Aquaporin-4-IgG-阳性NMOSD中独立于复发活动的进展:长达十年的队列研究
You-Ri Kang1,2, Ki Hoon Kim1,3, Jae-Won Hyun1
1Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
Neurology(R) neuroimmunology & neuroinflammation
|December 23, 2025
概括
在神经髓炎光学谱障碍 (NMOSD) 中,复发活动独立的进展 (PIRA) 是罕见的,复发是导致残疾的主要原因. 持续关注复发预防对于管理NMOSD至关重要.
科学领域:
- 神经免疫学 神经免疫学
- 神经学 神经学
- 临床研究 临床研究
背景情况:
- 神经脊髓炎光学谱系障碍 (NMOSD) 的残疾传统上与复发有关.
- 新兴证据表明潜在的亚临床进展,但其临床意义和关于进展独立于复发活动 (PIRA) 的定量数据是有限的.
研究的目的:
- 调查PIRA在大量AQP4-IgG阳性NMOSD患者队列中的频率和临床相关性.
- 在长期随访期间评估残疾恶化是否独立于复发活动.
主要方法:
- 对281名AQP4-IgG阳性NMOSD患者和≥2年的随访期进行了回顾性分析.
- 定义PIRA为持续扩展残疾状况尺度 (EDSS) 恶化而没有复发,在≥6个月后得到确认.
- 确定的复发相关恶化 (RAW) 当EDSS恶化 ≥6个月后复发.
主要成果:
- 在平均11.3年的随访期间,PIRA仅在2.5%的患者 (7人) 中被发现.
- 没有PIRA患者在没有治疗升级的情况下出现进一步恶化.
- 与复发相关的恶化 (RAW) 在经历复发的患者中发生了36.1%.
结论:
- 独立于复发活动的进展 (PIRA) 是NMOSD的一个罕见事件,即使随访时间长.
- 复发仍然是NMOSD中残疾的主要驱动因素.
- 继续强调复发预防策略是支持管理NMOSD的.
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