视神经脊髓炎谱系疾病合并干燥综合征临床病例研究

Clinical Analysis of Neuromyelitis Optica Spectrum Disorders with Primary Sjogrens' s Syndrome

目的探讨视神经脊髓炎谱系疾病合并干燥综合征的临床表现、影像特点、血中相关抗体和脑脊液、致残程度的特点。方法回顾性分析12例2008年5月—2015年9月住院的符合诊断标准的视神经脊髓炎谱系疾病合并干燥综合征患者的临床资料。结果 12例患者均为女性,年龄21-59岁,平均38.17岁,其中NMOSD发生于SS之前有4例,发生于SS之后有5例,同时被诊断有3例;12例中初发者为5例,其EDSS 3.0-6.5,平均5.2;8例患者行血AQP4检测6例阳性,SSA阳性者12例,SSB阳性者9例,12例患者脊髓MRI检查提示脊髓异常信号长度均大于3个椎体节段,大多数主要累及颈胸段脊髓,12例中MR检查强化者4例,12例患者均行唇腺组织活检符合SS诊断。结论NMOSD合并SS是两种各自独立的疾病,NMOSD患者中应注意筛查干燥综合征可能,在SS患者中,如果症状或影像有提示神经系统受累,亦应予以注意筛查NMOSD的可能。

Objective To investigate the clinical features of NMOSD with Sjogren＇s syndrome. Methods 12 inpatients（from May 2008 to September 2015） met with the criteria of NMOSD with Sjogren＇s syndrome were retrospectively analyzed. Results 12 patients were females, age 21-59 years old（mean 38.17）, 4 of 12 patients had NMOSD attack before Sjogren＇s syndrome,5 patients had Sjogren＇s syndrome before NMOSD, NMOSD and SS were diagnosed simultaneously in 3 patients;5 of 12 patients had first NMOSD attack; EDSS 3.0-6.5（mean 5.2）; Serum anti-AQP4-Ab were positive in 6 of 8 patients tested, SSA-Ab were positive in all 12 inpatients and SSB-Ab were positive in 9 patients; 12 patients had spinal cord involved extending morn than 3 s vertebral segment, cervical and/or thoracic cord were involved in most of patients, 4 patients had enhanced foci on MR, biopsy of labial gland in 12 patients fulfill SS. Conclusion NMOSD and SS were distinct disorders; Screen to comitant SS were suggested in NMOSD patients,and Screen to comitant NMOSD were suggested in SS patients who had clinical presentation or imaging of CNS involved