伴干燥综合征的视神经脊髓炎谱系疾病预后分析

Cfinical features and prognostic analysis of neuromyelitisoptica spectrum disease with sjogrensyndrome

目的观察合并干燥综合征的视神经脊髓炎谱系疾病（NMOSD-SS）的临床特征，分析其视力恢复、复发的预后因素。方法回顾性＋前瞻性观察性研究。对2013年7月至2016年6月首都医科大学附属北京同仁医院神经内科确诊的172例视神经脊髓炎谱系疾病（NMOSD）患者进行观察、随访，比较56例NMOSD-SS与116例NMOSD-非SS的临床及影像学特征，分析NMOSD-SS患者视力恢复、复发的预后因素。结果（1）NMOSD-SS较NMOSD-非SS患者最差视力〈0.1的比例高（83.9%比69.8%，P〈0.05）、SSA阳性率高（92.9%比0.0%,P〈0.05）、眼干或口干比例高（66.1%比5.2%,P〈0.05）、视觉诱发电位（VEP）波幅降低比例高（60.7%比43.1%，P〈0.05）。（2）NMOSD-SS较NMOSD-非SS患者年复发率高（0.58比0.53）、复发时间短（中位数6.7个月比12.4个月，P〈0.05）。（3）对所有NMOSD患者进行至少半年的随访，发现本次发病为复发眼、发病时最差视力〈0.1为其视力恢复不良（视力〈0.1）的危险因素（OR值分别为6.410、9.434，P〈0.05），而应用免疫抑制剂是防止NMOSD复发的保护因素（OR值0.107，P〈0.05）。结论NMOSD-SS较NMOSD-非SS视力损害严重，且更容易复发，本次发病为复发眼、发病时最差视力〈0.1的NMOSD-SS患者视力恢复不良，使用免疫抑制剂可减少NMOSD-SS复发概率。

ObjectiveTo explore the clinical features and conduct prognostic analysis about visual recovery and relapse of neuromyelitisoptica （NMO） spectrum disease （NMOSD） with sjogren syndrome （SS）. MethodsA retrospective and prospective observational study was conducted.Between July 2013 and June 2016, 172 patients with NMOSD （NMOSD-non SS： 116/172, 67.4%; NMOSD-SS： 56/172, 32.6%） were assessed at Beijing Tongren Hospital, Capital Medical University, Beijing, China.The prognostic factors of NMOSD-SS patients were also analyzed. ResultsAs compared with NMOSD-non SS patients, NMOSD-SS patients had worse visual impairment （percentage of patients with visual acuity less than 0.1, 83.9% vs 69.8%, P〈0.05）, higher positive rate of SSA （92.9% vs 0.0%, P〈0.05）, higher proportion of dryness of mouth and eye （66.1% vs 5.2%, P〈0.05） as well as higher percentage of reduced visual evoked potential （VEP） amplitude （60.7% vs 43.1%, P〈0.05）. NMOSD-SS patients had a significantly higher average year recurrent frequency （0.58 vs 0.53） and significantly shorter mean recurrence time （6.7 months vs 12.4 months, P〈0.05）. The results showed that recurrent eyes, the worst visual acuities of onset less than 0.1 were independent risk factors of visual impairment （visual activity 〈0.1）, according to at least six months＇ follow-up of all NMOSD-SS patients （OR=6.410 and 9.434, respectively, P〈0.05）. Meanwhile, immunosuppressive drugs were protective factors of relapse in NMOSD-SS patients （OR=0.107, P〈0.05）. ConclusionsNMOSD-SS patients have worse visual impairment, and they are more vulnerable to relapse than NMOSD-non SS patients, and the vision is lack of recovery for NMOSD-SS with recurrent eyes or the worst vision of onset less than 0.1.Immunosuppressive drugs can reduce the recurrence of NMOSD-SS relapse.