Nishimura Hiroaki, Enokida Hideki, Sakamoto Taiji, Takahashi Toshiyuki, Hayami Hiroshi, Nakagawa Masayuki
Blood Purification Center, Kagoshima University Hospital, 8-35-1, Sakuragaoka, Kagoshima, Kagoshima, 890-8520, Japan.
Department of Urology, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan.
J Artif Organs. 2018 Sep;21(3):378-382. doi: 10.1007/s10047-018-1044-3. Epub 2018 Apr 19.
The pathogenesis in the exacerbation of neuromyelitis optica spectrum disorder (NMOSD) involves mainly the serum anti-aquaporin-4 (AQP4) immunoglobulin G antibody (anti-AQP4 antibody). If high-dose corticosteroid treatment is not achieved during remission, rescue plasmapheresis is recommended. However, there are few reports on the therapeutic efficacy of repetitive immunoadsorption plasmapheresis (IAPP) for the recurrent exacerbation of NMOSD with a fluctuating anti-AQP4 antibody level. A 36-year-old man presented with a reduction of visual acuity (VA) on the right eye (OD) to 20/250. At this reduction of VA OD, magnetic resonance imaging (MRI) showed right optic nerve swelling without cerebral, brainstem, or spinal cord lesions. The anti-AQP4 antibody was detected in the serum. We diagnosed the patient with NMOSD and treated him with high-dose corticosteroid therapy. To prevent exacerbation with this treatment, the sixth session of the first IAPP course was adopted and VA OD improved to 20/100. Seven months later, VA OD deteriorated to 20/125 and ocular pain occurred. At that time, the anti-AQP4 antibody was not detected, although MRI revealed the recurrence of right optic neuritis. A second IAPP course with seven sessions was conducted with a concomitant administration of 1000 mg methylprednisolone every 10 days for 30 days. Ocular pain improved, although VA OD had continued to decline during these treatments and was eventually preserved at 20/400. In conclusion, IAPP is effective for the treatment of exacerbated NMOSD with a seropositive anti-AQP4 antibody. However, further study is necessary to develop treatments for relapsing NMOSD with a seronegative anti-AQP4 antibody.
视神经脊髓炎谱系障碍（NMOSD）病情加重时的发病机制主要涉及血清抗水通道蛋白4（AQP4）免疫球蛋白G抗体（抗AQP4抗体）。如果在缓解期未进行大剂量皮质类固醇治疗，则建议进行挽救性血浆置换。然而，关于重复免疫吸附血浆置换（IAPP）治疗抗AQP4抗体水平波动的NMOSD复发加重的疗效报道较少。一名36岁男性患者右眼（OD）视力降至20/250。在右眼视力下降时，磁共振成像（MRI）显示右侧视神经肿胀，无脑、脑干或脊髓病变。血清中检测到抗AQP4抗体。我们诊断该患者为NMOSD，并对其进行了大剂量皮质类固醇治疗。为防止该治疗导致病情加重，采用了第一个IAPP疗程的第六次治疗，右眼视力提高到20/100。七个月后，右眼视力恶化为20/125，并出现眼痛。当时，虽然MRI显示右侧视神经炎复发，但未检测到抗AQP4抗体。进行了第二个IAPP疗程，共七次治疗，同时每10天静脉注射1000mg甲泼尼龙，持续30天。眼痛有所改善，尽管在这些治疗期间右眼视力持续下降，最终维持在20/400。总之，IAPP对抗AQP4抗体血清阳性的NMOSD病情加重有效。然而，对于抗AQP4抗体血清阴性的复发型NMOSD，还需要进一步研究来开发治疗方法。
