原发性干燥综合征合并神经系统病变患者32例临床分析  

The neurological manifestations in 32 patients with primary Sjögren's syndrome

作　　者：邹婵娟[1] 何善智[1] 丁菱[1] 王明霞[1] 王敏[1] 颜丝语 ZOU Chan-juan;HE Shan-zhi;DING Ling;WANG Ming-xia;WANG Min;YAN Si-yu(Department of Rheumatology and Immunology,Zhongshan People′s Hospital,Zhongshan 528400,Guangdong,China)

机构地区：[1]中山市人民医院风湿免疫科,广东中山528400

出　　处：《广东医学》2024年第1期35-40,共6页Guangdong Medical Journal

基　　金：中山市医学科研项目(2016J033)。

摘　　要：目的了解原发性干燥综合征(pSS)合并神经系统病变患者的临床特征。方法回顾分析2013年3月至2022年1月诊断为pSS合并神经系统病变的32例患者的临床资料,并进一步分析定性诊断为脱髓鞘疾病及血管性疾病患者的临床特征。结果pSS合并神经系统病变发生率11.1%(32/287),以神经系统为首发表现的有56.3%(18/32)。pSS合并神经系统病变患者临床表现多样,单纯中枢神经系统病变27例(84.4%),单纯周围神经受累2例(6.3%),中枢神经系统合并周围神经系统病变2例(6.3%),神经-肌肉接头病变1例(3.1%)。32例患者中,脱髓鞘性疾病13例,血管性疾病13例,感染性病变3例,肌紧张性头痛2例,重症肌无力1例,比较脱髓鞘性疾病、血管性疾病患者的临床指标,结果发现脱髓鞘性疾病患者外周血淋巴细胞数量、血清IgM水平均高于血管性疾病患者[1.98(1.39,2.77)×10^(9)·L^(-1) vs.1.30(1.07,1.77)×10^(9)·L^(-1),P=0.005;1.31(1.12,1.88)g/L vs.0.98(0.74,1.18)g/L,P=0.009]。结论pSS合并神经系统病变发生率不低,临床表现多样复杂,脱髓鞘疾病、血管性疾病多见,目前发病机制不明,淋巴细胞活化、免疫系统激活与泛化可能在发病、疾病演变过程中发挥作用。Objective To investigate the clinical features of neurological involvement in primary Sjogren′s syndrome(pSS).Methods The clinical data of 32 pSS patients with neurological involvement from March 2013 to January 2022 were retrospectively reviewed.Clinical data were compared between patients with demyelinating disease and vascular disease.Results The prevalence of neurological involvement in pSS patients was 11.1%(32/287),while 56.3%(18/32)as the initial symptom of pSS.There were a variety of neuropsychiatric presentations in pSS.The incidence of central neuropathy,peripheral neuropathy and combination of the central neuropathy with peripheral neuropathy were 84.4%(27/32),6.3%(2/32)and 6.3%(2/32),respectively.Neuromuscular junction lesion was found in 1 patient(3.1%).Thirteen patients were diagnosed with demyelinating disease,13 had vascular disease,3 had infectious disease,2 patients had tension headache and 1 patient was diagnosed with myasthenia gravis.Compared with those patients with vascular disease,lymphocytes and serum IgM level were significantly higher in patients with demyelinating disease[1.98(1.39,2.77)×10^(9)·L^(-1) vs.1.30(1.07,1.77)×10^(9)·L^(-1),P=0.005;1.31(1.12,1.88)g/L vs.0.98(0.74,1.18)g/L,P=0.009].Conclusion The prevalence of neurological involvement in pSS is not low.The clinical manifestations are diverse and complex.Demyelinating diseases and vascular diseases are common.The pathogenesis is unknown.Lymphocyte activation and immune system over-stimulation may play roles in neurological involvement in pSS.

关 键 词：干燥综合征 神经系统病变 临床特征 

分 类 号：R593[医药卫生—内科学] R741[医药卫生—临床医学]