Abstract: Objective To explore the clinical manifestations, laboratory examination, MRI characteristics, treatment and prognosis of primary Sjögren's syndrome (PSS) with neuromyelitis optica spectrum disorders (NMOSDs). Methods  The clinical manifestations, laboratory examination, MRI characteristics, treatment and prognosis of 4 patients diagnosed as PSS with NMOSDs were retrospectively analyzed. Results  There were 2 PSS patients with neuromyelitis optica (NMO), 1 PSS patient combined with recurrent myelitis associated with longitudinally extensive spinal cord lesions (RLESLs), and 1 PSS patient with retrobulbar optic neuritis. Laboratory examination showed serum Sjögren's syndrome antigen A (SSA) antibody positive in 4 cases, Sjögren's syndrom antigen B (SSB) antibody positive in 3 cases and serum specific aquaporin 4 (AQP4) antibody positive in 3 patients with NMO. Abnormal features in cranial MRI mainly distributed in bilateral basal ganglia, cerebral peduncle corticospinal tract, beside lateral ventricles, and brainstem. Spinal MRI showed the lesions were mainly at cervical or thoracic spinal cord (≥ 3 vertebral segments) and also involved medullary bulb (2 cases). In acute stage, glucocorticoid and prednisone combined with immunosuppressive drugs were used. Conclusion  PSS and NMO are autoimmune diseases, and the two diseases are closely linked. Patients with NMO and (or) NMOSDs were suggested to detect serum auto-antibody and AQP4 antibody. In patients with serum AQP4 antibody positive, the recurrence rate is high and prognosis is poor. Immunosuppressive therapy is recommended to reduce recurrence.

Key words: Sjogren's syndrome, Neuromyelitis optica, Aquaporins, Magnetic resonance imaging

摘要： 目的 探讨原发性干燥综合征合并视神经脊髓炎谱系疾病的临床表现、实验室检查和MRI 特点，以及治疗和预后。方法 回顾分析4 例诊断明确的原发性干燥综合征合并视神经脊髓炎谱系疾病患者的临床资料，分析其发病特点。结果 临床症状与体征主要表现为原发性干燥综合征合并复发性纵向延伸性脊髓炎或球后视神经炎，实验室检查血清抗干燥综合征抗原A 抗体（4 例）、抗干燥综合征抗原B 抗体（3 例）、视神经脊髓炎特异性抗水通道蛋白4（AQP4）抗体（3 例）阳性。头部MRI 病灶主要分布于双侧基底节和大脑脚皮质脊髓束走行区、双侧额叶、右侧皮质脊髓束区、双侧侧脑室旁和脑干；脊髓MRI病灶以累及颈胸髓为主，≥ 3 个椎体节段（3 例）或累及延髓（2 例）。急性期以大剂量糖皮质激素冲击和序贯口服泼尼松，并辅助免疫抑制药治疗为主。结论 原发性干燥综合征和视神经脊髓炎均为自身免疫性疾病，二者关系密切。对于视神经脊髓炎和（或）视神经脊髓炎谱系疾病患者建议行血清自身抗体和抗AQP4 抗体检测，抗AQP4 抗体阳性者复发率高，预后不良。建议辅助应用免疫抑制药，以减少复发。

关键词: 干燥综合征, 视神经脊髓炎, 水孔蛋白质类, 磁共振成像