视神经脊髓炎谱疾病叠加干燥综合征的临床研究

目的研究视神经脊髓炎谱疾病(NMOSD)叠加干燥综合征(SS)的临床特点、治疗及预后。方法收集2008年1月1日至2013年12月31日在北京同仁医院神经眼科住院的视神经炎、视神经脊髓炎叠加干燥综合征"患者的资料进行分析。结果共收集17例,其中男性1例、女性16例;平均发病年龄44.6±13.2岁、双眼受累(70.6%)、视功能损害较为严重(最佳矫正视力低于0.1的有22只眼,占75.9%)、视野主要表现为弥漫性视野缺损(共15只眼,占68.2%),水通道蛋白4lg G抗体(AQP4)抗体阳性的有8例(占57%),住院后虽经积极治疗,但患者的视功能改善不佳,16只眼(55.2%)治疗前后无变化,且4例(50%)AQP4抗体阳性的患者均复发。结论 NMOSD与SS可能有共同的发病机制;AQP4抗体的测定对于NMOSD治疗方案的选择和预后的评估至关重要。

Clinical study of neuromyelitis optica spectrum disease accombined with Sjogren syndrome

Objective To study the clinical features, treatment and prognosis on patients with neuromyelitis optica spectrum disorders ( neuromylitis optic spectrum disease, NMOSD ) accombined with Sjogren syndrome ( Sjogren syndrome, SS ) .Methods Information of patients who were hospitalized in the department of nerve ophthalmology ward of Beijing Tongren hospital and diagnosed with optic neuritis and neuromyelitis optic accombined withSjogrensyndromefromJanuary2008toDecember2013wereanalyzed.Results 17caseswerecollected,one patient was male and 16 patients were female;the average age was 44.6±13.2 years old, binocular were commonnly involved( 70.6%) , visual function was seriously damaged( the best corrected visual acuity below 0.1 accounted for 22 eyes , about 75.9%, visual field was mainly manifested as diffuse visual field defect ( 15 eyes, 68.2%) , AQP4 antibody positive existed in 8 patients which accounted for 57%, visual function of patients was not improve well although active treatment was givened,there was no variety in 16 eyes (55.2%) before and after treatment, and 4 (50%) patients that AQP4 antibody was positive were recurrence.Conclusion Common pathogenesis existed in NMOSD and SS;AQP4 was essential for the assessment of the selection of treatment and prognosis of NMOSD.