【摘要】 目的探究原发性干燥综合征(pSS)患者神经系统损害的临床特点。方法回顾性分析就诊于天津医科大学总医院风湿免疫科和北京协和医院风湿免疫科的pSS合并神经系统损害患者32例。分析pSS神经系统受累患者的人口学特征、临床表现、实验室检查及影像学检查特征。结果 32例pSS患者中,女30例(93.8%),男2例(6.2%),年龄(46±12)岁。16例(50%)患者以神经系统损害为首发症状,其余以干燥症状为首发症状。13例(40.6%)患者表现为单独周围神经系统(PNS)损害,10例(31.3%)患者表现为单独中枢神经系统(CNS)损害,9例(28.1%)患者同时合并两种损害(CNS-PNS)。PNS损害中最常见的是多发性神经病变(6例,18.8%),CNS损害中最常见是视神经脊髓炎谱系疾病(6例,18.8%)。PNS损害的患者较CNS损害的患者更易伴有雷诺现象(P=0.046)。PNS和CNS-PNS损害的患者肺间质病变(ILD)比例明显高于CNS损害的患者(P=0.013)。结论 pSS患者多发性神经病变和视神经脊髓炎谱系疾病分别是PNS和CNS损害的最常见形式,合并PNS损害的患者更易合并肺间质病变。更多还原

【Abstract】 Objective To investigate the clinical characteristics of nervous system involvement in patients with primary Sjogren’s syndrome(pSS).Methods Data of 32 pSS patients with nervous system involvement collected from Tianjin Medical University General Hospital and Peking Union Medical College Hospital were retrospectively analyzed. The demographic characteristics, clinical symptoms, laboratory parameters and imaging results were evaluated and analyzed.Results In 32 patients with pSS, there were 30(93.8%) females and 2(6.2%) males, with the average age 46±12 years. The neurological impairment was the first symptom in 16(50%) patients, and sicca symptoms was the first symptom in the rest patients. Thirteen(40.6%) patients developed isolated peripheral nervous system(PNS) damages, 10(31.3%) patients developed isolated central nervous system(CNS) damages, and 9(40.6%) patients showed both(CNS-PNS). The most common lesion of PNS was polyneuropathy(6, 18.8%). The most common lesion of CNS was optic neuromyelitis pedigree disease(NMODS, 6, 18.8%). Patients with PNS lesion were more likely to have Reynolds phenomenon than those with CNS lesion(P=0.046). The prevalence of interstitial lung disease(ILD) was significantly higher in pSS patients with PNS and CNS-PNS damages than patients with CNS lesion(P=0.013).Conclusion Polyneuropathy and NMODS are the most common damages in pSS patients with PNS and CNS lesions respectively. Patients with PNS lesions are more likely to have ILD.更多还原