中文摘要：

目的了解原发性干燥综合征（pSS）合并周围神经病变的临床及实验室特点。方法入选符合2002年美国一欧洲（US．Euro）分类标准的pSS患者86例，由风湿免疫专科医师调查及分析患者的临床症状和实验室指标；由神经内科专科医师完成神经系统查体，并完善肌电图检查。对患者的临床症状、查体、实验室检查及肌电图检查进行分析，判断患者是否合并周围神经病变，并比较2组临床症状及实验室特征。数据分析采用t检验、r检验及Logistic回归分析。结果共人组86例pSS患者，男性4例，女性82例，合并周围神经系统病变者占26％（22／86），pSS患者的周围神经病变症状多样，受累神经主要见于正中神经、胫神经、腓神经、腓肠神经等，受累部位以下肢常见（16／22，73％），神经病变类型以感觉运动纤维受累为主。此外，周围神经病均在Dss发病后出现，其中约有45％的患者在pSS发病早期出现周围神经病变。Dss合并周围神经系统病变组与无周围神经病变组相比，除手足麻木（68％与5％，P〈0．01）外，雷诺现象（32％与5％，P=0．002）较为常见，且该组患者的欧洲抗风湿病联盟干燥综合征疾病活动度评分（ESSDAI）明显升高（5．3＋2．5与3．4~1．9，P〈0．01）。雷诺现象（OR=9．489，95％CI2．191．41．093，P=0．003）及ESSDAI（OR=1．528，95％C11．179～1．979，P=O,001）是周围神经病的独立危险因素。pSS合并周围神经系统病变组抗核抗体滴度（雎0．003）和类风湿因子滴度明显升高（辟O。023）。结论pSS患者合并周围神经系统病变的发生率较高，病程早期即可出现周围神经系统病变，雷诺现象、高疾病活动度是合并周围神经病变的独立危险因素。

英文摘要：

[Abstract] Objective To analyze the clinical manifestations of primary Sjfigren＇s syndrome （pSS） with peripheral neuropathies. Methods Eighty-six patients who fulfdled the 2002 American-European Con- sensus Group criteria for pSS were enrolled in the study. For each patient, medical data, including clinical, laboratory, immunologic and electromyography data were collected and analyzed. The clinical manifestations of primary Sjfigren＇s syndrome were compared between patients with and without peripheral neuropathy. Statistical methods used were t-test, chi-square test and Logistic regression. Results Eighty-six patients were analyzed, and neurological involvement was noted in 26% （22/86） patients. The clinical spectrum of peri- pheral neuropathies encountered in Sjsgren＇s syndrome patients was wide, with sensory neuropathies being the most common. Median nerve, peroneal nerve and sural nerve were the most likely involved, and lower limb involvement accounted for 73% （16/22）. Peripheral neuropathy was diagnosed during the Sjfigren＇s syndrome course in all patients, and about 45% patients＇ neurological involvement were diagnosed early in the course of the disease. The frequency of Raynaud＇s phenomenon was significantly higher （32% vs 5%, P=-0.002） as well as acroanesthesia （68% vs 5%, P〈O.O1） in pSS with peripheral neurological involvement than in pSS without peripheral neuropathy. The median values of EULAR Sjfigren＇s syndrome disease activity index （ESSDAI） were 5.3 （range 2.8-7.8） and 3.4 （range 1.5-5.3） in the PNS and non-PNS groups respectively （P〈0.01）. Wefound a significant rise of peripheral neuropathy risk associated with Raynaud＇s phenomenon （relative risk 9.489, 95%CI 2.191-41.093, P=0.003） and ESSDAI （relative risk 1.528, 95%CI 1.179-1.979, P=0.001）. Elevated titers of rheumatoid factor （P=-0.023） and ANA （P=0.003） were common in patients with peripheral neuropathy. Conclusion Peripheral neuropathy is not a rare manifestation of pSS.