Apheresis therapies in MOGAD: a retrospective study of 117 therapeutic interventions in 571 attacks.

IF 8.7 1区医学 Q1 CLINICAL NEUROLOGY

Journal of Neurology, Neurosurgery, and Psychiatry Pub Date : 2024-11-04 DOI:10.1136/jnnp-2024-334863

Carolin Schwake, Theodoros Ladopoulos, Vivien Häußler, Ingo Kleiter, Marius Ringelstein, Orhan Aktas, Tania Kümpfel, Daniel Engels, Joachim Havla, Martin W Hümmert, Julian Reza Kretschmer, Daria Tkachenko, Corinna Trebst, Ana Beatriz Ayroza Galvão Ribeiro Gomes, Anne-Katrin Pröbstel, Mirjam Korporal-Kuhnke, Brigitte Wildemann, Sven Jarius, Refik Pul, Mosche Pompsch, Markus Krämer, Florian Then Bergh, Clemens Gödel, Patricia Schwarz, Markus C Kowarik, Paulus Stefan Rommer, Ioannis Vardakas, Makbule Senel, Alexander Winkelmann, Nele Retzlaff, Martin S Weber, Leila Husseini, Annette Walter, Patrick Schindler, Judith Bellmann-Strobl, Friedemann Paul, Ralf Gold, Ilya Ayzenberg

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引用次数: 0

Abstract

Background: 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.

Methods: 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.

Results: 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.

Conclusion: Apheresis is frequently used in MOGAD attacks. An early start as first-line therapy and concomitant disease-modifying therapy predict full attack recovery.

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多发性骨髓增生异常综合征（MOGAD）的血液透析疗法：对 571 次发作中 117 次治疗干预的回顾性研究。

背景：发作缓解不彻底是髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）致残的主要原因。血浆置换和免疫吸附等无细胞疗法被广泛应用于神经免疫学领域。有关无细胞疗法在 MOGAD 攻击中疗效的数据仍然有限：我们回顾性评估了 2008 年至 2023 年间在 18 个神经脊髓炎视网膜研究组中心发生的所有经过无细胞疗法治疗的 MOGAD 患者发作。治疗反应分为完全缓解、部分缓解或无缓解。普雷塔克和随访扩展残疾状态量表（EDSS）和视觉功能系统评分（FSS）用于计算绝对疗效（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%）名患者的117/571（20.5%）次发作采用了分离疗法。与孤立的脊髓炎（25.2%，n=35）、脑部表现（21.0%，n=17）或孤立的视神经炎（17.6%，n=51）相比，同时患有视神经炎和脊髓炎的患者更常接受无细胞疗法治疗（42.4%，n=14）。12%的患者（发病后4.5（IQR 0-11）天）将血液透析作为一线疗法，62%的患者（发病后15（IQR 6.75-31）天）将血液透析作为二线疗法，26%的患者（发病后30（IQR 19-42）天）将血液透析作为三线疗法。21%的患者病情完全缓解，70%的患者病情部分缓解，9%的患者病情无缓解。一线无细胞疗法（OR 2.5，P=0.040）和同时进行的疾病修饰疗法（OR 1.5，P=0.011）与完全缓解相关。这两项参数也与ΔEDSS良好相关。不同类型的血液透析治疗结果无差异：结论：血液净化常用于 MOGAD 攻击。结论：无细胞疗法经常用于 MOGAD 发作，作为一线疗法及早开始并同时进行疾病修饰疗法可预示发作完全恢复。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Neurology, Neurosurgery, and Psychiatry 医学-精神病学

CiteScore

15.70

自引率

1.80%

发文量

888

审稿时长

6 months

期刊介绍： The Journal of Neurology, Neurosurgery & Psychiatry (JNNP) aspires to publish groundbreaking and cutting-edge research worldwide. Covering the entire spectrum of neurological sciences, the journal focuses on common disorders like stroke, multiple sclerosis, Parkinson’s disease, epilepsy, peripheral neuropathy, subarachnoid haemorrhage, and neuropsychiatry, while also addressing complex challenges such as ALS. With early online publication, regular podcasts, and an extensive archive collection boasting the longest half-life in clinical neuroscience journals, JNNP aims to be a trailblazer in the field.