视神经脊髓炎谱系疾病合并干燥综合征5例临床分析

Clinical analysis of 5 cases of optic nerve myelopathy pedigree disease complicated with Sjogren＇s syndrome

目的 分析视神经脊髓炎谱系疾病（NMOSD）合并干燥综合征（SS）患者的首发症状、临床表现、实验室检查、影像学特点及治疗预后情况。方法 选择既往有SS病史的NMOSD患者5例，并统计其临床资料。结果 NMOSD合并SS多发生于女性，首发症状及临床系统表现多样，主要为恶心呕吐，肢体疼痛、无力，视物模糊，浅感觉减退，自主神经系统功能障碍等，且合并多种自身免疫性抗体阳性。影像学多表现为大脑半球及脊髓受累，且≥3个节段，使用糖皮质激素冲击治疗后症状有所缓解，但视力恢复不明显。结论 ① NMOSD合并SS多发生于青年女性。②当患者出现视力下降、肢体麻木、恶心呕吐或小便障碍等临床症状，且有自身免疫性抗体阳性时，应考虑NMOSD合并SS的可能。

Objective To analyze the initial symptom, clinical manifestation, laboratory examination, imaging features and treatment prognosis of the patients with optic nerve myelopathy pedigree disease （NMOSD） combined with Sjogren’s syndrome （SS）.Methods Five cases of NMOSD with SS history were selected and their clinical data were statistically analyzed.Results NMOSD combined with SS occured mostly in women, and the initial symptom and clinical system performance were varied, mainly for nausea and vomiting, limb pain, weakness, blurred vision, shallow sensory decline, autonomic nervous system dysfunction, etc, and combined of multiple autoimmune antibodies positive.Most imaging findings included cerebral hemisphere and spinal cord involvement, and more than equal to or greater than 3 segments, symptoms were relieved after using corticosteroids, but visual recovery was not apparent.Conclusions ① NMOSD combined SS occured mostly in young women. ② When the patient has vision loss, limb numbness, nausea, vomiting, or urination disorders and other clinical symptoms, and autoimmune antibodies are positive, the possibility of NMOSD merging with SS should be considered.