以周围神经病为首发症状的抗中性粒细胞胞浆抗体相关性血管炎的临床特点  

Clinical features of antineutrophil cytoplasmic antibody-associated vasculitis with peripheral neuropathy as the initial symptom

作　　者：张玉琴[1] 童海玲 侯晨 杨海晖 张卫茹[1] 杨光[1] ZHANG Yuqin;TONG Hailing;HOU Chen(Department of General Medicine,Xiangya Hospital of Central South University,Changsha 410000,China)

机构地区：[1]中南大学湘雅医院全科医学科,长沙410000 [2]湘乡市人民医院棋梓分院内科 [3]南华大学衡阳医学院

出　　处：《临床神经病学杂志》2024年第3期197-200,共4页Journal of Clinical Neurology

基　　金：湖南省自然科学基金项目(2021JJ41045)。

摘　　要：目的探讨以周围神经病为首发症状的抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)的临床特征,为临床诊治提供思路。方法总结2017年至2021年在中南大学湘雅医院就诊的6例以周围神经病为首发症状的AAV患者的临床资料。结果6例均为中老年男性,首发症状主要为肢体麻木、疼痛、无力。除神经系统受累外,部分患者伴有上呼吸道、肺部、肾脏、眼睛、耳、皮肤及全身关节受累,EMG提示多发性周围神经损害;5例患者pANCA、MPO阳性,1例患者cANCA、PR3阳性;经糖皮质激素和环磷酰胺治疗后,临床症状及实验室指标均有不同程度的改善。结论以周围神经病变为首发症状的AAV临床表现缺乏特异性,AAV的早期诊断和治疗可以改善患者生活质量和预后。Objective To investigate the clinical characteristics of antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV)with peripheral neuropathy as the initial symptom and to enhance the understanding of the disease.Methods The clinical data of 6 patients of AAV with peripheral neuropathy as the initial symptom at Xiangya Hospital of Central South University from 2017 to 2021 were collected.Results All 6 cases were middle-aged or elderly males,and the initial symptoms mainly consisted of limb numbness,pain,and weakness.In addition to neurological involvement,some patients had upper respiratory tract,lungs,kidneys,eyes,ears,skin and joints involved.Electromyography showed multiple peripheral nerve damage;Five patients were positive for pANCA and MPO,and one patient was positive for cANCA and PR3.Six patients were all treated with glucocorticoid and cyclophosphamide,and the clinical symptoms and laboratory indexes were improved to different degrees.Conclusions There were no characteristic symptoms in AAV with peripheral neuropathy as the first symptom.Early diagnosis and treatment of AAV can improve the quality of life and prognosis of patients.

关 键 词：抗中性粒细胞胞浆抗体相关血管炎 周围神经病 自身免疫性疾病 

分 类 号：R745[医药卫生—神经病学与精神病学]