摘要： 目的 总结视神经脊髓炎谱系疾病合并其他自身免疫性疾病的临床特点。方法与结果 回顾分析2014年6月至2019年1月北京大学第三医院收治的55例视神经脊髓炎谱系疾病患者,10例合并其他自身免疫性疾病,其中5例合并干燥综合征、2例合并桥本甲状腺炎、1例合并类风湿关节炎、1例合并系统性红斑狼疮、1例合并未分化结缔组织病。合并自身免疫性疾病组女性(校正χ²=3.842,P=0.050)和抗AQP4抗体阳性(校正χ²=4.303,P=0.038)比例高于未合并自身免疫性疾病组。10例合并其他自身免疫性疾病患者中9例脊髓受累,其中颈髓3例、胸髓2例、颈髓和胸髓4例,4例视神经受累,其中单纯视神经受累仅1例,2例存在脑部病灶,1例延髓受累。脊髓病灶的影像学特点符合视神经脊髓炎谱系疾病,病变节段> 3个椎体长度,最长累及10个节段。结论 视神经脊髓炎谱系疾病易合并其他自身免疫性疾病,以女性多见,抗AQP4抗体阳性率更高,应引起重视。

关键词: 视神经脊髓炎, 免疫系统疾病, 水通道蛋白质 4

Abstract: Objective To investigate the clinical characteristics of neuromyelitis optica spectrum disorders (NMOSDs) with other autoimmune diseases. Methods and Results Retrospective analysis was performed on 55 NMOSDs patients admitted to Peking University Third Hospital from June 2014 to January 2019. There were 10 NMOSDs patients with autoimmune diseases, including 5 patients with Sj?gren's syndrome (SS), 2 patients with Hashimone's thyroiditis (HT), one patient with rheumatoid arthritis (RA), one patient with systemic lupus erythematosus (SLE), and one patient with undifferentiated connective tissue disease. The proportion of females with autoimmune disease (adjusted χ²=3.842, P=0.050) and positive of anti-AQP4 antibody (adjusted χ²=4.303, P=0.038) was higher than that of females without autoimmune diseases. Among 10 patients of NMOSDs with other autoimmune diseases, 9 patients involved spinal cord, 3 cases only involved cervical spinal, 2 cases only involved thoracic spinal, 4 cases involved both cervical and thoracic spinal at the same time, 4 cases involved optic nerve, one case involved only optic nerve. There was brain lesions in 2 cases, one case involved medulla oblongata. The imaging characteristics of spinal cord lesions were consistent with those of patients with NMOSDs. The length of the lesions were all longer than 3 vertebral bodies, and the longest lesions involved 10 segments. Conclusions NMOSDs is easy to be combined with other autoimmune diseases, which are more common in women, and the rate of positive of anti-AQP4 antibody is higher, which should be paid attention to.

Key words: Neuromyelitis optica, Immune system diseases, Aquaporin 4 Conflicts of interest:none declared