摘要
目的 了解pSS合并神经系统受累的临床及实验室特点.方法 回顾性分析了148例符合2002年美国-欧洲分类诊断标准的pSS患者,根据患者神经系统症状和体格检查,结合肌电图、神经肌肉组织活检等确诊神经系统受累类型,比较合并神经系统受累组和未合并神经系统受累组的临床及实验室特征.采用Mann-Whitney秩和检验、x2检验和Fisher确切概率法进行分析.结果 pSS患者神经系统受累的总体发生率为20.3％（30/148）,其中,单纯周围神经病变、单纯中枢神经病变以及中枢神经合并周围神经病变的发生率分别为10.1％（15/148）、9.5％（14/148）和0.7％（1/148）.周围神经病变以单纯感觉神经病（40％）与感觉运动神经病（33％）为主,中枢神经病变表现为运动神经元病居多（71％,10/14）.与未合并神经系统病变组相比,合并神经系统病变组的病程较短[（55±76）个月与（100±108）个月,Z=-2.682,P〈0.05],而ANA和RF滴度较低[（234±248）与（377±339）,Z=-2.008,P〈0.05;（126±279） U/ml与（359±1445） U/ml,Z=-2.243,P〈0.05].合并神经系统病变的pSS患者临床表现以肢体麻木（50％）、肢体疼痛（23％）及肌无力（63％）为主.结论 pSS合并神经系统病变发生率高,病程相对较短,疾病活动度高,但干燥的相关表现不典型,容易被风湿科医师忽略.
Objective To analyze the clinical and laboratory manifestations of primary Sjogren＇s syndrom （pSS） with neurological involvement.Methods One hundred and forty eight patients fulfilling the 2002 American-European pSS classification criteria were retrospectively analyzed.Neurological manifestations were diagnosed based on the clinical,biological,electrophysiological,and imaging findings.Biographical,clinical,and laboratory data were compared between patients with and without neurological manifestations.Statistical methods used were Mann-Whitney U test,Chi-square test and Fisher exact probability.Results The prevalence of neurological involvement in pSS was 20.3% （30/148）,and the incidence of peripheral neuropathy,the central neuropathy and combination of the central neuropathy with peripheral neuropathy were 10.1%（15/148）,9.5%（14/148） and 0.7%（1/148）,respectively.The clinical spectrum of peripheral neuropathies encountered in Sj（o）gren＇s syndrome （SS） patients varied,with the pure sensory neuropathies being the most common,followed by sensorimotor neurophathies.Motor neuron disease was the most common type of central neurophathies.Compared with those without neurological manifestations,the duration of peripheral nerve system/central nerve system （PNS/CNS）-pSS patients was relatively short [（55±76） months vs （100±108） months,Z=-2.682,P〈0.05],and the antinuclear antibody （ANA） titer and RF titer were lower [（234±248） vs （377±339）,Z=-2.008,P〈0.05;（126±279） U/ml vs （359±1 445） U/ml,Z=-2.243,P〈0.05].In PNS/CNS-pSS patients,the most common clinical manifestations included numbness （50%）,pain （23%）,and muscle weakness （63%）.Conclusion The prevalence of neurological involvement in pSS is high.The duration is relatively short and the disease activity is high,but the disease features are atypical and may be neglected by rheumatologists.
出处
《中华风湿病学杂志》
CSCD
北大核心
2017年第6期375-380,共6页
Chinese Journal of Rheumatology
基金
国家自然科学基金（81571573,81471599）
关键词
干燥综合征
神经系统病变
周围神经系统
中枢神经系统
临床特点
Sjogren＇s syndrome
Neurologic manifestations
Peripheral nerve system
Central nerve system
Clinical features
