抗中性粒细胞胞质抗体相关血管炎性周围神经病患者的临床、电生理特征及疗效分析  

Clinical and electrophysiological characteristics and treatment outcomes of anti-neutrophil cytoplasmic antibody ANCA-associated vasculitic neuropathy

作　　者：李一凡[1,2] 李懋[1] 杨飞[1] 王红芬[1] 徐菲[1] 陈思宇[2] 孙博[2] 陈朝晖[1] 黄旭升[1] Li Yifan;Li Mao;Yang Fei;Wang Hongfen;Xu Fei;Chen Siyu;Sun Bo;Chen Zhaohui;Huang Xusheng(Department of Neurology of the First Medical Center,Chinese PLA General Hospital,Beijing 100853,China;Geriatric Neurological Department of the Second Medical Center&National Clinical Research Center for Geriatric Diseases,Chinese PLA General Hospital,Beijing 100853,China)

机构地区：[1]解放军总医院第一医学中心神经内科,北京100853 [2]解放军总医院第二医学中心神经内科、国家老年疾病临床医学研究中心,北京100853

出　　处：《中华内科杂志》2024年第4期386-393,共8页Chinese Journal of Internal Medicine

摘　　要：目的研究不同类型抗中性粒细胞胞质抗体(ANCA)相关血管炎(AAV)性周围神经病(VN)患者的临床、电生理特征和治疗效果的影响因素。方法病例系列研究。收集2006年1月至2022年12月在解放军总医院第一医学中心连续收治的AAV患者652例, 其中合并VN91例, 排除存在其他病因者, 最终纳入61例, 其中嗜酸性肉芽肿性多血管炎(EGPA)17例、肉芽肿性多血管炎(GPA)11例、显微镜下多血管炎(MPA)33例。搜集患者的临床资料, 比较不同类型AAV患者间临床特征、VN临床表现、电生理[侧间波幅比(IAR)]及治疗差异, 使用logistic回归分析影响疗效的因素。结果 EGPA、GPA及MPA组VN的发生率分别62.1%(18/29)、8.3%(15/180)和13.1%(58/443)。MPA组患者就诊和发病年龄中位数高于EGPA及GPA组(P<0.01)。GPA组VN发生时间晚于EGPA组(P<0.01), 电生理受累神经数少于其他两组(P<0.016)。EGPA组下肢运动神经IAR异常比例高于MPA组(P<0.01)。logistic回归分析结果显示, 第3版伯明翰血管炎活动度评分(BVAS-V3)≥15(OR=6.85, 95%CI 1.33~35.30)患者VN疗效较好, 而中枢神经系统受累(OR=0.13, 95%CI 0.02~0.89)是疗效不佳的危险因素。结论不同类型AAV患者VN临床和电生理特点略有不同, GPA患者常表现为多发性周围神经病, 而电生理为局灶性受累, EGPA以多发单神经病最常见。BVAS-V3较高和中枢神经系统受累可能对VN治疗效果有预测作用。Objective To investigate the clinical and electrophysiological characteristics of ANCA-associated vasculitic neuropathy(VN)and analyze the predictors of treatment outcomes.Methods Retrospective case series.In all,652 consecutive patients with ANCA-associated vasculitis were admitted to the First Medical Center of the Chinese PLA General Hospital between January 2006 and December 2022.Peripheral neuropathy occurred in 91 patients.Patients were excluded if other known causes of neuropathy were present.Sixty-one patients were eventually enrolled,including 17 with eosinophilic granulomatosis with polyangiitis(EGPA),11 with granulomatosis polyangiitis(GPA),and 33 with microscopic polyangiitis(MPA).Their clinical data were collected and clinical characteristics,VN manifestations,electrophysiological findings(including interside amplitude ratio[IAR]),and treatment outcomes were compared among the three subsets of AAV.Then,factors influencing the treatment outcomes were analyzed using multivariable logistic regression analysis.Results Peripheral neuropathy occurred in 62.1%(18/29)of EGPA,8.3%(15/180)of GPA,and 13.1%(58/443)of MPA patients.The age at onset and examination was higher in patients with MPA than those with EGPA or GPA(P<0.01).The occurrence of VN was later in patients with GPA than those with EGPA(P<0.01),and the GPA group had fewer affected nerves than the other two groups(P<0.016).The abnormal IARs of motor nerves in lower limbs were more detected in the EGPA than the MPA group(P<0.01).Logistic regression analysis suggested that higher Birmingham vasculitis activity score-version 3(BVAS-V3)(OR=6.85,95%CI 1.33-35.30)was associated with better treatment outcomes of VN.However,central nervous system involvement was a risk factor for poor treatment outcomes(OR=0.13,95%CI 0.02-0.89).Conclusions The clinical and electrophysiological characteristics of VN were slightly different among subsets of AAV.Patients with GPA often presented with polyneuropathy and had fewer nerves affected;mononeuritis multiplex was more

关 键 词：显微镜下多血管炎 韦格纳肉芽肿病 血管炎 变应性肉芽肿性 周围神经系统疾病 

分 类 号：R593.2[医药卫生—内科学]