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

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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Journal
Journal of the Peripheral Nervous System
Published
2026-09-30
DOI
https://doi.org/10.1111/jns.70175
Primary Topic
Peripheral Neuropathies and Disorders
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article
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article

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

Jean‐Philippe Camdessanché, Jean‐Baptiste Chanson, Tanya Stojkovic, Boucraut Jose et al.
Journal of the Peripheral Nervous System
Peripheral Neuropathies and Disorders
article

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

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

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.

Journal of the Peripheral Nervous SystemVol. 31(4)
Inserm (FR), Aix-Marseille Université (FR), Université de Lille (FR), Commissariat à l'Énergie Atomique et aux Énergies Alternatives (FR), Centre Hospitalier Universitaire de Grenoble (FR), Centre Hospitalier Universitaire de Nice (FR), Centre Hospitalier Universitaire Henri-Mondor (FR), Hôpital de la Conception (FR), Assistance Publique – Hôpitaux de Paris (FR), Hôpital Pierre Wertheimer (FR), Hôpital Gui de Chauliac (FR), Institut de Myologie (FR), Centre d'Épidémiologie sur les Causes Médicales de Décès (FR), Centre Hospitalier Universitaire de Clermont-Ferrand (FR), Centre Hospitalier Régional Universitaire de Brest (FR), Institut de Neurosciences de la Timone (FR), Hôpital Purpan (FR), Hôpitaux Universitaires de Strasbourg (FR), Institut Paoli-Calmettes (FR), Centre Hospitalier Universitaire de Saint-Étienne (FR), Hôpital de la Timone (FR), Bicêtre Hospital (FR), Université d'Angers (FR), Université de Limoges (FR)
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Peripheral Neuropathies and Disorders
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