活检证明的神经大动脉血管炎和微血管炎的独特临床特征
Pannathat Soontrapa1,2, Marcus V Pinto1, Kamal Shouman1
1Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
Brain : a journal of neurology
|December 23, 2024
概括
神经大动脉管血管炎通常具有急性发作和远端多神经病变,通常与自身免疫性疾病有关. 神经微血管炎呈现更慢性,影响近道神经和自主功能,经常作为非系统性血管炎.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
- 病理学 病理学 病理学
背景情况:
- 血管性神经病因神经血管的炎症性破坏而导致,导致缺血症.
- 神经血管炎根据血管大小进行分类:大动脉 (≥75μm) 和微血管炎 (<75μm).
研究的目的:
- 描述和比较神经大动脉血管炎和神经微血管炎的临床特征.
主要方法:
- 对278名神经活检诊断或暗示血管炎的患者进行了回顾性队列研究 (2001-2020年).
- 收集和分析临床和病理学数据.
主要成果:
- 大动脉血管炎的急性发病率较高 (50.4%与26.8%相比),远部不对称性多神经病 (48.0%).
- 微血管炎有慢性发病 (57.5%与33.6%相比),更长的诊断/平原时间,根部复合神经病变 (43.8%),更多的自主参与 (24.2%) 和与非系统性血管炎的关联 (71%).
- 系统性自身免疫性疾病在大动脉管血管炎中更为常见 (70.4%对22.9%).
结论:
- 神经大动脉血管炎和微血管炎表现出不同的,但重叠的临床表现.
- 大动脉血管炎通常呈现为急性,具有远端多神经病变和全身自身免疫关联.
- 微血管炎通常呈现为慢性,包括近道神经,自主功能障碍和非系统性血管炎.
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