Department of Neurology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Front Immunol. 2021 Jul 21;12:650782. doi: 10.3389/fimmu.2021.650782. eCollection 2021.
The changes in the serum levels of aquaporin-4-IgG (AQP4-IgG), immunoglobulins, and inflammatory mediators in neuromyelitis optica spectrum disorder (NMOSD) cases treated with immunoadsorption have been rarely described in detail. Here we report a 29-year-old steroid-resistant NMOSD female with a severe disability (bilateral blindness and paraplegia) who received protein-A immunoadsorption as a rescue treatment. During the total 5 sessions, the circulating level of AQP4-IgG, immunoglobulins, and complement proteins (C3 and C4) showed a rapid and sawtooth-like decrease, and the serum AQP4-IgG titer declined from 1:320 to below the detectable limit at the end of the 3rd procedure. Of all the antibodies, IgG had the biggest removal rate (>96.1%), followed by IgM (>66.7%) and IgA (53%), while complement C3 and C4 also dropped by 73% and 65%, respectively. The reduced pro-inflammatory cytokines (interleukin-8 and tumor necrosis factor-α) and marked increased lymphocyte (T and B cell) counts were also observed. The improvement of symptoms initiated after the last session, with a low AQP4-IgG titer (1:32) persisting thereafter. Accordingly, protein-A immunoadsorption treatment could be one of the potential rescue therapies for steroid-resistant NMOSD patients with a severe disability.
水通道蛋白 4 免疫球蛋白 G（AQP4-IgG）、免疫球蛋白和炎症介质在接受免疫吸附治疗的视神经脊髓炎谱系疾病（NMOSD）患者中的血清水平变化鲜有详细描述。在此，我们报告了 1 例 29 岁的女性类固醇耐药性 NMOSD 患者，病情严重（双侧失明和截瘫），接受蛋白 A 免疫吸附作为抢救治疗。在总共 5 个疗程中，AQP4-IgG、免疫球蛋白和补体蛋白（C3 和 C4）的循环水平呈快速锯齿状下降，血清 AQP4-IgG 滴度在第 3 次治疗结束时从 1:320 下降到检测不到。在所有抗体中，IgG 的清除率最高（>96.1%），其次是 IgM（>66.7%）和 IgA（53%），补体 C3 和 C4 也分别下降了 73%和 65%。促炎细胞因子（白细胞介素-8 和肿瘤坏死因子-α）减少，淋巴细胞（T 细胞和 B 细胞）计数明显增加。在最后一次治疗后症状开始改善，此后持续存在低 AQP4-IgG 滴度（1:32）。因此，蛋白 A 免疫吸附治疗可能是一种潜在的治疗类固醇耐药性 NMOSD 严重残疾患者的抢救治疗方法。
