Schwake Carolin, Ladopoulos Theodoros, Häußler Vivien, Kleiter Ingo, Ringelstein Marius, Aktas Orhan, Kümpfel Tania, Engels Daniel, Havla Joachim, Hümmert Martin W, Kretschmer Julian Reza, Tkachenko Daria, Trebst Corinna, Ayroza Galvão Ribeiro Gomes Ana Beatriz, Pröbstel Anne-Katrin, Korporal-Kuhnke Mirjam, Wildemann Brigitte, Jarius Sven, Pul Refik, Pompsch Mosche, Krämer Markus, Then Bergh Florian, Gödel Clemens, Schwarz Patricia, Kowarik Markus C, Rommer Paulus Stefan, Vardakas Ioannis, Senel Makbule, Winkelmann Alexander, Retzlaff Nele, Weber Martin S, Husseini Leila, Walter Annette, Schindler Patrick, Bellmann-Strobl Judith, Paul Friedemann, Gold Ralf, Ayzenberg Ilya
Department of Neurology, St Josef Hospital, Ruhr University Bochum, Bochum, Germany.
Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
J Neurol Neurosurg Psychiatry. 2025 Jun 12;96(7):639-646. doi: 10.1136/jnnp-2024-334863.
Incomplete attack remission is the main cause of disability in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Apheresis therapies such as plasma exchange and immunoadsorption are widely used in neuroimmunology. Data on apheresis outcomes in MOGAD attacks remain limited.
We retrospectively evaluated all apheresis treated attacks occurring in patients with MOGAD between 2008 and 2023 at 18 Neuromyelitis Optica Study Group centres. Treatment response was categorised as complete, partial or no remission. Preattack and follow-up Expanded Disability Status Scale (EDSS) and visual Functional System Scores (FSS) were used to calculate absolute outcomes (ΔEDSS/Δvisual FSS). Predictors of complete remission were analysed using a generalised linear mixed model.
Apheresis was used for 117/571 (20.5%) attacks in 85/209 (40.7%) patients. Attacks with simultaneous optic neuritis and myelitis were treated more often with apheresis (42.4%, n=14) than isolated myelitis (25.2%, n=35), cerebral manifestation (21.0%, n=17) or isolated optic neuritis (17.6%, n=51). Apheresis was initiated as first-line therapy in 12% (4.5 (IQR 0-11) days after attack onset), second-line therapy in 62% (15 (IQR 6.75-31) days) and third-line therapy in 26% (30 (IQR 19-42) days). Complete remission was achieved in 21%, partial remission in 70% and no remission in 9% of patients. First-line apheresis (OR 2.5, p=0.040) and concomitant disease-modifying therapy (OR 1.5, p=0.011) were associated with complete remission. Both parameters were also associated with a favourable ΔEDSS. No differences in outcomes were observed between the apheresis types.
Apheresis is frequently used in MOGAD attacks. An early start as first-line therapy and concomitant disease-modifying therapy predict full attack recovery.
发作缓解不完全是髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）导致残疾的主要原因。血浆置换和免疫吸附等血液净化疗法在神经免疫学中被广泛应用。关于MOGAD发作时血液净化治疗结果的数据仍然有限。
我们回顾性评估了2008年至2023年期间在18个视神经脊髓炎研究组中心接受血液净化治疗的所有MOGAD患者的发作情况。治疗反应分为完全缓解、部分缓解或未缓解。使用发作前和随访时的扩展残疾状态量表（EDSS）和视觉功能系统评分（FSS）来计算绝对结果（ΔEDSS/Δ视觉FSS）。使用广义线性混合模型分析完全缓解的预测因素。
85/209（40.7%）例患者中的117/571（20.5%）次发作采用了血液净化治疗。同时患有视神经炎和脊髓炎的发作采用血液净化治疗的比例（42.4%，n = 14）高于单纯脊髓炎（25.2%，n = 35）、脑部表现（21.0%，n = 17）或单纯视神经炎（17.6%，n = 51）。血液净化作为一线治疗开始于发作开始后12%（4.5（四分位间距0 - 11）天），二线治疗于62%（15（四分位间距6.75 - 31）天），三线治疗于26%（30（四分位间距19 - 42）天）。21%的患者实现了完全缓解，70%的患者部分缓解，9%的患者未缓解。一线血液净化治疗（比值比2.5，p = 0.040）和联合疾病改善治疗（比值比1.5，p = 0.011）与完全缓解相关。这两个参数也与有利的ΔEDSS相关。不同血液净化类型之间在结果上未观察到差异。
血液净化在MOGAD发作中经常使用。作为一线治疗尽早开始和联合疾病改善治疗可预测发作完全恢复。
