干燥综合征合并视神经脊髓炎谱系疾病临床特征分析  

The clinical characteristics of Sjgren's syndrome with neuromyelitis optica spectrum disease

作　　者：王娟娟 林玲[1] 叶励超[2] WANG Juanjuan;LIN Ling;YE Lichao(Department of Rheumatology,the Second Affliated Hospital of Fujian Medical University,Quanzhou Fujian 362000,China)

机构地区：[1]福建医科大学附属第二医院免疫内科,362000 [2]福建医科大学附属第二医院神经内科,362000

出　　处：《中国神经免疫学和神经病学杂志》2018年第4期227-230,共4页Chinese Journal of Neuroimmunology and Neurology

摘　　要：目的研究干燥综合征(SS)合并视神经脊髓炎谱系疾病(NMOSD)的临床特点,以提高临床对该病的认识。方法回顾性分析2011-01—2017-12福建医科大学附属第二医院住院确诊的7例SS合并NMOSD患者的一般情况、临床表现、影像学及预后等资料。结果 7例SS合并NMOSD患者均为女性,发病中位年龄为50岁,6例患者缺少SS相关临床表现,7例患者均以NMOSD起病后发现SS,1例在起病初期被误诊为星形胶质细胞瘤,2例被误诊为多发性硬化(MS)。随病程延长,其复发率越高,预后较差。结论 SS合并NMOSD易被误诊、漏诊,且病程长时预后更差;应注意筛查抗核抗体、抗水通道蛋白4抗体,及时诊治,并动态随访,减少复发。Objective To investigate the clinical characteristics of Sjgren＇s syndrome（SS）with neuromyelitis optica spectrum disease（NMOSD）.Methods We retrospectively assessed 7 inpatients diagnosed as SS with NMOSD between January 2011 and December 2017 at the Second Affliated Hospital of Fujian Medical University.The clinical manifestations,imaging characteristics and the prognosis were analyzed.Results Seven patients were enrolled in this study.They were all females with the median age of 50 years old.Six cases lacked the classic clinical characteristics of SS such as dry mouth and dry eye.Seven patients were not found to have SS until the discovery of NMOSD.One case was misdiagnosed as astrocytoma,two misdiagnosed as multiple sclerosis（MS）.The longer the follow up,the higher the relapse rate and the worse the prognosis were.Conclusions SS with NMOSD was commonly misdiagnosed.Antinuclear antibodies and anti-AQP4 antibody were suggested to be screened.The prognosis was worse with a longer course which needed attention and careful follow up.

关 键 词：干燥综合征 视神经脊髓炎谱系疾病 视神经脊髓炎 脱髓鞘疾病 水通道蛋白4 

分 类 号：R744.5[医药卫生—神经病学与精神病学] R442.8[医药卫生—临床医学]