Department of Nephrology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Department of Nephrology, China Rehabilitation Research Center, Beijing, China.
J Clin Apher. 2022 Jun;37(3):237-244. doi: 10.1002/jca.21965. Epub 2022 Feb 1.
Neuromyelitis optica spectrum disorders (NMOSD) is a rare inflammatory demyelinating disease of the central nervous system. NMOSD pathogenesis is mainly mediated by antibodies directed against aquaporin4 (AQP4 antibody). Immunoadsorption (IA) could specifically remove pathogenic antibody to alleviate the disease. Until now, prospective studies concerning the efficacy of IA on NMOSD are scarce. This study aims to prospectively evaluate the efficacy and safety of IA in the treatment of NMOSD.
We included patients with AQP4 antibody-positive NMOSD who were hospitalized from September 2019 to September 2020, with no significant improvement in symptoms after 1 week of high-dose intravenous steroid therapy. Tryptophan IA therapy was initiated with five sessions on alternate days. Expanded Disability Status Scale (EDSS), visual acuity, and laboratory values were measured before and after IA, with a follow-up of 6 months. Spinal magnetic resonance imaging (MRI) characteristics were collected. Related side effects were recorded.
Seven patients were enrolled in the present study. After five IA, the patients' EDSS decreased from 5.71 ± 2.04 to 4.64 ± 2.29, P = .006. The visual acuity of the three visually impaired patients was improved. AQP4-IgG decreased significantly from 80.00 (interquartile range [IQR], 21.00-80.00) (U/mL) to 9.72 (IQR, 5.21-55.57) (U/mL) (P = .018). MRI of the spinal cord showed the scope of the myelopathy was narrowed and no significant enhancement was observed on postcontrast T1-weighted image at 90 days after treatment. Only one patient had transient hypotension.
Tryptophan IA therapy effectively and safely improved neurological function and visual acuity, and reduced the AQP4 antibody concentration in patients with NMOSD.
视神经脊髓炎谱系疾病（NMOSD）是一种罕见的中枢神经系统炎症性脱髓鞘疾病。NMOSD 的发病机制主要由针对水通道蛋白 4（AQP4 抗体）的抗体介导。免疫吸附（IA）可以特异性去除致病性抗体，从而缓解疾病。到目前为止，关于 IA 在 NMOSD 中的疗效的前瞻性研究还很少。本研究旨在前瞻性评估 IA 在 NMOSD 治疗中的疗效和安全性。
我们纳入了 2019 年 9 月至 2020 年 9 月住院的 AQP4 抗体阳性 NMOSD 患者，在高剂量静脉类固醇治疗 1 周后症状无明显改善。每周 5 天进行色氨酸 IA 治疗。在 IA 前后测量扩展残疾状况量表（EDSS）、视力和实验室值，并进行 6 个月的随访。收集脊髓磁共振成像（MRI）特征。记录相关不良反应。
本研究共纳入 7 例患者。经过 5 次 IA 治疗后，患者的 EDSS 从 5.71±2.04 降至 4.64±2.29，P=0.006。3 名视力受损患者的视力有所改善。AQP4-IgG 显著下降，从 80.00（四分位距 [IQR]，21.00-80.00）（U/mL）降至 9.72（IQR，5.21-55.57）（U/mL）（P=0.018）。脊髓 MRI 显示脊髓病变范围缩小，治疗后 90 天对比增强 T1 加权图像未见明显强化。仅 1 例患者出现短暂性低血压。
色氨酸 IA 治疗有效且安全，可改善 NMOSD 患者的神经功能和视力，并降低 AQP4 抗体浓度。
