Rituximab With or Without Chemotherapy for Anti-MAG Neuropathy: A Retrospective Multicenter Real-World Study

IF 3 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System Pub Date : 2026-09-30 DOI:10.1111/jns.70175

Delmont Emilien, Cintas Pascal, Tard Céline, Boucraut José, Magy Laurent, Frachet Simon, Labeyrie Céline, Echaniz-Laguna Andoni, Noel Nicolas, Svahn Juliette, Pegat Antoine, Bouhour Françoise, Cluse Florent, Puma Angela, Magot Armelle, Farnault Laure, Aurran Schleinitz Thérèse, Gendre Thierry, Planté-Bordeneuve Violaine, Dormeuil Alice, Cassereau Julien, Chanson Jean-Baptiste, Mallaret Martial, Stojkovic Tanya, Noury Jean-Baptiste, Taieb Guillaume, Camdessanché Jean-Philippe, Poncet-Megemont Louis, Grapperon Aude-Marie, Fortanier Etienne, Attarian Shahram

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引用次数: 0

Abstract

Background and Aims

In anti-myelin-associated glycoprotein (MAG) neuropathy, the efficacy of rituximab (RTX) is not demonstrated in randomized controlled trials. Combinations of RTX with conventional chemotherapy (immunochemotherapy, ICT) may provide potential benefit, but systematic evaluations are lacking. We aimed to compare the efficacy and safety of RTX and ICT in a multicenter retrospective real-world cohort.

Methods

Patients with anti-MAG antibody titers > 10 000 Buhlmann titer units (BTUs) were included. Treatment response was defined as an improvement of ≥ 1 point in the Overall Neuropathy Limitation Scale (ONLS) compared to baseline. Propensity score matching was used to account for differences in baseline characteristics between treatment groups.

Results

A total of 230 patients were included: 150 received RTX and 80 received ICT. Mean follow-up was 4 years (range, 1–20 years). One year after treatment, clinical improvement occurred in 61/147 (41%) patients treated with RTX and 39/78 (50%) treated with ICT (OR = 1.4 [0.8–2.5] p = 0.3). At last follow-up, clinical improvement occurred in 55/150 (37%) patients treated with RTX and 31/80 (39%) treated with ICT (OR = 1.1 [0.6–1.9] p = 0.8). Propensity score matching yielded similar results. Among ICT regimens, dexamethasone–RTX–cyclophosphamide was associated with the highest response rate. RTX induction, 1 g days 1 and 15, seemed superior to four-weekly infusions. Adverse events were more frequent with ICT than with RTX (28% vs. 15%; OR = 2.20 [1.07–4.54], p = 0.02).

Interpretation

In this cohort, adding chemotherapy to RTX was not associated with greater long-term improvement, despite a higher rate of adverse events. Prospective controlled studies are needed.

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利妥昔单抗联合或不联合化疗治疗抗mag神经病变：一项回顾性多中心真实世界研究

背景和目的：在抗髓鞘相关糖蛋白（MAG）神经病中，利妥昔单抗（RTX）的疗效尚未在随机对照试验中得到证实。RTX联合常规化疗（免疫化疗，ICT）可能提供潜在的益处，但缺乏系统的评估。我们的目的是比较RTX和ICT在多中心回顾性现实世界队列中的有效性和安全性。方法：纳入抗mag抗体滴度为100 ~ 10 000布尔曼效价单位（btu）的患者。治疗反应被定义为与基线相比，总体神经病变限制量表（ONLS）改善≥1分。倾向评分匹配用于解释治疗组之间基线特征的差异。结果：共纳入230例患者，其中150例接受RTX治疗，80例接受ICT治疗。平均随访4年（范围1-20年）。治疗1年后，RTX组有61/147（41%）患者出现临床改善，ICT组有39/78（50%）患者出现临床改善（OR = 1.4 [0.8-2.5] p = 0.3）。最后随访时，55/150 (37%)RTX组患者临床改善，31/80 (39%)ICT组患者临床改善（OR = 1.1 [0.6-1.9] p = 0.8）。倾向评分匹配产生了类似的结果。在ICT方案中，地塞米松- rtx -环磷酰胺与最高的应答率相关。1 g RTX诱导，第1天和第15天似乎优于4周输注。ICT组不良事件发生率高于RTX组（28% vs. 15%; OR = 2.20 [1.07-4.54], p = 0.02）。解释：在该队列中，尽管不良事件发生率较高，但在RTX中添加化疗与更大的长期改善无关。需要前瞻性对照研究。

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 the Peripheral Nervous System 医学-临床神经学

CiteScore

6.10

自引率

7.90%

发文量

审稿时长

>12 weeks

期刊介绍： The Journal of the Peripheral Nervous System is the official journal of the Peripheral Nerve Society. Founded in 1996, it is the scientific journal of choice for clinicians, clinical scientists and basic neuroscientists interested in all aspects of biology and clinical research of peripheral nervous system disorders. The Journal of the Peripheral Nervous System is a peer-reviewed journal that publishes high quality articles on cell and molecular biology, genomics, neuropathic pain, clinical research, trials, and unique case reports on inherited and acquired peripheral neuropathies. Original articles are organized according to the topic in one of four specific areas: Mechanisms of Disease, Genetics, Clinical Research, and Clinical Trials. The journal also publishes regular review papers on hot topics and Special Issues on basic, clinical, or assembled research in the field of peripheral nervous system disorders. Authors interested in contributing a review-type article or a Special Issue should contact the Editorial Office to discuss the scope of the proposed article with the Editor-in-Chief.