Abstract:
Objective: To explore the clinical features and conduct prognostic analysis about visual recovery and relapse of neuromyelitisoptica (NMO) spectrum disease (NMOSD) with sjogren syndrome (SS). Methods: A 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. Results: As 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). Conclusions: NMOSD-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.
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。.