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抗中性质细胞质抗体 阴性血管神经病变
Prateek Harsh1, Shalesh Rohatgi, Prajwal Rao
1Department of Neurology, Dr. D. Y. Patil Medical College, Hospital and Research Center, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra, India.
Annals of African medicine
|September 15, 2025
概括
与抗中性粒细胞体抗体 (ANCA) 相关的血管炎可以在没有可检测的ANCA的情况下发生. 这一案例凸显了在患有神经病变和多系统参与的患者中怀疑ANCA相关血管炎的重要性,即使ANCA测试是负面的.
科学领域:
- 腎臟病學 (nephrology) 是一種醫學專業.
- 神经学 神经学
- 类风湿病学 类风湿病学
背景情况:
- 抗中性细胞体抗体 (ANCA) 相关血管炎 (AAA) 是一种小血管系统性血管炎.
- 神经病变是一种潜在的表现,通常与脏,肺部和心脏相关.
- 在80%-90%的病例中检测到ANCA,需要高临床怀疑,如果是负面的.
研究的目的:
- 报告一种与神经病变和多系统参与有关的血清阴性ANCA相关血管炎病例.
- 强调ANCA无法检测到的ANCA相关血管炎的诊断挑战和管理策略.
主要方法:
- 一个49岁的男性患有2型糖尿病和慢性病的病例报告.
- 临床表现包括渐进的感觉运动神经病变和急性间歇性肺部疾病.
- 诊断工作包括神经传导研究,胸部CT和外围神经活检;ANCA和ANA是负的.
主要成果:
- 患者表现出严重的泛性轴突感官运动神经病变和间歇性肺部疾病.
- 外科神经活检显示炎症性轴心病,暗示血管炎.
- 用环胺和修复剂治疗导致两年内显著的神经恢复.
结论:
- 在多系统参与的轴突神经病变的情况下,即使有负ANCA,也应该怀疑ANCA相关的血管炎.
- 血清阴性ANCA相关血管炎占病例的10%-20%.
- 术外神经活检对于在血清学标志物缺失时诊断血管神经病变至关重要.
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