Multiple sclerosis and Demyelinating disorders Clinic, National Institute of Neurology and Neurosurgery, Insurgentes Sur 3877, La Fama, Tlalpan, Mexico City 14269, Mexico.
Multiple sclerosis and Demyelinating disorders Clinic, National Institute of Neurology and Neurosurgery, Insurgentes Sur 3877, La Fama, Tlalpan, Mexico City 14269, Mexico.
Mult Scler Relat Disord. 2021 Jul;52:103022. doi: 10.1016/j.msard.2021.103022. Epub 2021 May 15.
Neuromyelitis optica spectrum disorders (NMOSDs) are a group of chronic immune-mediated demyelinating diseases of the central nervous system. Their pathophysiology dependent on humoral mediated responses caused by autoreactive IgG antibodies against aquaporin-4 water channels (AQP4-IgG) or myelin oligodendrocyte glycoprotein (MOG-IgG). Plasma exchange (PLEX) has proved to be a beneficial therapy in patients with severe relapses. We present the largest series of Latin American patients treated with PLEX for acute NMOSDs relapses.
A retrospective study was conducted. Selection included patients diagnosed with NMOSDs who received PLEX between 2010-2019, irrespective of their AQP4-IgG serostatus. All patients received 5 grams of IV methylprednisolone. PLEX therapy could be initiated simultaneously or after IV steroids. Baseline and post-PLEX therapy Expanded Disability Status Scale (EDSS) was measured to identify acute response to therapy. Comparison between responders and non-responders was also conducted. Subgroup analysis stratified response by serostatus, type of clinical relapse and time to PLEX.
A total of 89 patients were included. Mean age at onset was 38 ± 12.97 years. 49 (55.1%) patients were AQP4-IgG seropositive. Most patients had unilateral optic neuritis (34.8%) or longitudinally extensive transverse myelitis (33.7%). Mean time from onset to PLEX initiation was 20.9 ± 18.1 days. Response rate was 39.3% and mean decline in EDSS was 0.7 ± 0.9 (p <0.001). Decline in EDSS and response rate were independent of serostatus, type of clinical relapse or time to PLEX initiation.
PLEX appears to be an effective therapy for NMOSDs relapses even in limited resources setting where treatment initiation may be delayed. The benefit seems to be independent of the type of clinical relapse and AQP4 IgG serostatus.
视神经脊髓炎谱系疾病(NMOSD)是一组中枢神经系统慢性免疫介导的脱髓鞘疾病。其病理生理学依赖于自身反应性 IgG 抗体针对水通道蛋白-4(AQP4)或髓鞘少突胶质细胞糖蛋白(MOG)的体液介导反应。血浆置换(PLEX)已被证明对严重复发的患者是一种有益的治疗方法。我们报告了拉丁美洲最大系列接受 PLEX 治疗急性 NMOSD 复发的患者。
进行了一项回顾性研究。入选标准为 2010 年至 2019 年期间接受 PLEX 治疗的 NMOSD 患者,无论其 AQP4-IgG 血清状态如何。所有患者均接受 5 克 IV 甲基强的松龙。PLEX 治疗可以与 IV 类固醇同时或之后开始。测量基线和 PLEX 治疗后扩展残疾状态量表(EDSS),以确定对治疗的急性反应。还对反应者和非反应者进行了比较。根据血清状态、临床复发类型和 PLEX 时间进行亚组分析。
共纳入 89 例患者。发病时的平均年龄为 38 ± 12.97 岁。49 例(55.1%)患者为 AQP4-IgG 血清阳性。大多数患者有单侧视神经炎(34.8%)或纵向广泛横断性脊髓炎(33.7%)。从发病到开始 PLEX 的平均时间为 20.9 ± 18.1 天。反应率为 39.3%,EDSS 平均下降 0.7 ± 0.9(p <0.001)。EDSS 的下降和反应率与血清状态、临床复发类型或 PLEX 开始时间无关。
即使在资源有限的情况下,PLEX 似乎也是 NMOSD 复发的有效治疗方法,即使在开始治疗可能延迟的情况下也是如此。这种益处似乎独立于临床复发类型和 AQP4 IgG 血清状态。