使用蛋白A免疫吸附联合免疫抑制疗法治疗原发性干燥综合征(pSS)合并视神经脊髓炎谱系障碍(NMOSD)的报道很少。在这里,我们介绍了一例35岁女性患者,诊断为pSS合并NMOSD(pSS-NMOSD),该患者对包含大剂量静脉注射甲泼尼龙(IVMP)和静脉注射免疫球蛋白(IVIG)的治疗无效后,对蛋白A免疫吸附呈阳性反应.在接受三次免疫吸附联合免疫抑制治疗的一周内,患者的临床症状(视力模糊,轻瘫,和功能失调的本体感觉)显着改善。此外,水通道蛋白-4免疫球蛋白G抗体(AQP4-IgG)的循环水平迅速下降,免疫球蛋白(Ig)A,IgG,IgM,红细胞沉降率(ESR),观察类风湿因子(RF)。磁共振成像(MRI)进一步显示,与纵向广泛性横贯性脊髓炎相关的病变显着减少。在后续期间,泼尼松龙逐渐减少至5-10毫克/天的维持剂量,而霉酚酸酯(MMF)维持在1.0-1.5g/天。病人的病情稳定了四年,在影像学检查中没有观察到复发或进展的迹象。因此,该病例提示,对于常规治疗难以治疗的pSS-NMOSD患者,蛋白A免疫吸附可能是一种潜在有效的治疗选择.
The treatment of primary Sjögren\'s syndrome (pSS) coexisting with neuromyelitis optica spectrum disorder (NMOSD) using protein-A immunoadsorption combined with immunosuppressive therapy has rarely been reported. Herein, we present the case of a 35-year-old female diagnosed with pSS concomitant with NMOSD (pSS-NMOSD) who demonstrated a positive response to protein-A immunoadsorption after failing to respond to therapy comprising high-dose intravenous methylprednisolone (IVMP) and intravenous immunoglobulin (IVIG). Within one week of receiving three sessions of immunoadsorption combined with immunosuppressive treatment, the patient\'s clinical symptoms (blurred vision, paraparesis, and dysfunctional proprioception) significantly improved. Additionally, a rapid decrease in the circulating levels of Aquaporin-4 immunoglobulin G antibodies (AQP4-IgG), immunoglobulin (Ig) A, IgG, IgM, erythrocyte sedimentation rate (ESR), and rheumatoid factor (RF) were observed. Magnetic resonance imaging (MRI) further revealed a significant reduction in the lesions associated with longitudinal extensive transverse myelitis. During the follow-up period, prednisolone was gradually tapered to a maintenance dose of 5-10 mg/day, whereas mycophenolate mofetil (MMF) was maintained at 1.0-1.5 g/day. The patient\'s condition has remained stable for four years, with no signs of recurrence or progression observed on imaging examination. Therefore, this case suggests that protein A immunoadsorption may represent a potentially effective therapeutic option for patients with pSS-NMOSD who are refractory to conventional treatments.