抗中性粒细胞胞浆抗体相关性血管炎神经损害的病理和临床特点  

Neuropathy in Antineutrophil Cytoplasmic Antibody Associated Vasculitis:from Pathology to Clinical

作　　者：林森[1] 张文[2] 

机构地区：[1]枣庄市中医医院风湿科,枣庄山东300120 [2]中国医学科学院北京协和医学院北京协和医院风湿免疫科,北京100032

出　　处：《中华临床免疫和变态反应杂志》2013年第3期282-286,共5页Chinese Journal of Allergy & Clinical Immunology

基　　金：北京自然科学基金(7132206);中华医学会临床医学科研专项基金(12040680368)

摘　　要：抗中性粒细胞胞浆抗体(antineutrophil cytoplasmic antibodies,ANCA)相关性血管炎是一类以小动、静脉和毛细血管受累为主的全身多系统、多脏器损害的疾病。神经系统损害是其常见的临床表现,部分患者甚至以神经系统表现为首发症状。累及周围神经时,患者多表现为受累神经或肢体剧烈的灼烧样疼痛伴感觉迟钝,痛温觉、深感觉缺失,感觉异常,此外还可出现肌无力、肌萎缩和感觉性共济失调;累及中枢神经时,常见症状为头痛。ANCA相关性血管炎周围神经病变多由于神经表面小动脉炎性反应、闭塞导致神经缺血性损伤,进一步引起束状神经纤维变性和髓鞘纤维减少造成;中枢神经病变常由颅内小血管炎和坏死性肉芽肿炎性病变引起。腓肠神经活组织检查是诊断ANCA相关性血管炎周围神经病的有效手段。对于神经受累的ANCA相关性血管炎患者,应积极控制原发病,并辅助以扩血管和营养神经等对症治疗,以期改善预后、提高生活质量。Anti-neutrophil cytoplasmic antibodies （ is a category of multi-system, multi-organ antineutrophil cytoplasmic antibodies, ANCA） associated vasculitis damaging disease based on small involvement. Its common clinical manifestation is the damaging arteries, veins or capillaries of nervous system; some patients treat the manifest of nervous system as their initial symptom. When peripheral nerves involves, the most patients shows neurological involvement or limb-like pain with severe burning sensation, pain and temperature sensation, deep sensory loss, paresthesia. Meanwhile, it may also showed muscle weakness, muscle atrophy and sensory ataxia. When nervous centralis involves, it is often show headache. ANCA-associated vasculitis peripheral neuropathy primary due to inflammatory reaction or blocking of arteriole on the surface of nerve, it leads to the ischemic damaging of nerves, furthermore, It may also lead to sarciniform nerve fiber modification and myelin fibers decreased. Central nervous system lesions often causes by encephalic small vessels inflammation and necrotizing granulomatous inflammation. Sural nerve biopsy is an effective neuropathy. With regard to ANCA-associated measure in the diagnosis of ANCA-associated vasculitis peripheral vasculitis patients with nervous involvement, in order to improveprognosis and quality of life, they should control protopathy positively associated with vasodilator nerve symptomatic treatment and nutrition.

关 键 词：抗中性粒细胞胞浆抗体 血管炎 神经损害 

分 类 号：R593.9[医药卫生—内科学]