Rituximab treatment in AChR-positive generalized myasthenia gravis within two years of diagnosis

Objectives Newly diagnosed acetylcholine receptor (AChR)–positive generalized myasthenia gravis (gMG) is typically managed with corticosteroids, but long-term exposure carries substantial adverse effects. We evaluated the safety and effectiveness of rituximab (RTX) as the first and the only add-on to steroid therapy in AChR-positive gMG within two years from diagnosis. Materials and Methods We studied 24 adults who received RTX within 24 months from gMG diagnosis (mean 9.3 ± 7.1 months). This was a retrospective, single-center observational cohort study including consecutive eligible patients. Clinical outcomes (MG-ADL, MGFA-PIS), corticosteroid doses, and AChR antibody titers were assessed at baseline and 3, 6, and 12 months. Results Mean age at RTX initiation was 63 ± 15 years; 10 patients (41.7%) were ≥65 and 4 (17%) had thymoma. Follow-up averaged 15.5 ± 6.9 months. Patients were stratified as steroid responders (n = 16), partial responders (n = 5), and refractory (n = 3). MG-ADL scores improved from 1.9 ± 2.4 to 0.32 ± 0.78 at 6-months and 0.0 at 12 months. Corticosteroid dose decreased from 22.4 ± 9.0 mg/day at initiation to 11.4 ± 4.7 mg/day at 6 months and 7.7 ± 2.2 mg/day at 12 months. At 6-months, 61% achieved or maintained pharmacological remission and 30% minimal manifestations; at 12-months, 64% were in remission and 36% had minimal manifestations. AChR antibody titers declined by ~70% at both time points. No serious RTX-related adverse events requiring treatment discontinuation were observed. Conclusions Our study highlights RTX’s dual role both as a steroid-sparing maintenance therapy and as an early immunosuppressive option for steroid partial responders in addition to refractory AChR-positive gMG patients.

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Journal
Clinical Neurology and Neurosurgery
Published
2026-08-21
DOI
https://doi.org/10.1016/j.clineuro.2026.109624
Primary Topic
Myasthenia Gravis and Thymoma
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article
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article

Rituximab treatment in AChR-positive generalized myasthenia gravis within two years of diagnosis

Vasiliki Zouvelou, Aigli G. Vakrakou, Eleni Strataki, Kleopas A. Kleopa et al.
Clinical Neurology and Neurosurgery
Myasthenia Gravis and Thymoma
article

Rituximab treatment in AChR-positive generalized myasthenia gravis within two years of diagnosis

Vasiliki Zouvelou, Aigli G. Vakrakou, Eleni Strataki, Kleopas A. Kleopa, Dimitra Tzavella, Tasos Tsokkos, Maria Belimezi
article en

Abstract

Objectives Newly diagnosed acetylcholine receptor (AChR)–positive generalized myasthenia gravis (gMG) is typically managed with corticosteroids, but long-term exposure carries substantial adverse effects. We evaluated the safety and effectiveness of rituximab (RTX) as the first and the only add-on to steroid therapy in AChR-positive gMG within two years from diagnosis. Materials and Methods We studied 24 adults who received RTX within 24 months from gMG diagnosis (mean 9.3 ± 7.1 months). This was a retrospective, single-center observational cohort study including consecutive eligible patients. Clinical outcomes (MG-ADL, MGFA-PIS), corticosteroid doses, and AChR antibody titers were assessed at baseline and 3, 6, and 12 months. Results Mean age at RTX initiation was 63 ± 15 years; 10 patients (41.7%) were ≥65 and 4 (17%) had thymoma. Follow-up averaged 15.5 ± 6.9 months. Patients were stratified as steroid responders (n = 16), partial responders (n = 5), and refractory (n = 3). MG-ADL scores improved from 1.9 ± 2.4 to 0.32 ± 0.78 at 6-months and 0.0 at 12 months. Corticosteroid dose decreased from 22.4 ± 9.0 mg/day at initiation to 11.4 ± 4.7 mg/day at 6 months and 7.7 ± 2.2 mg/day at 12 months. At 6-months, 61% achieved or maintained pharmacological remission and 30% minimal manifestations; at 12-months, 64% were in remission and 36% had minimal manifestations. AChR antibody titers declined by ~70% at both time points. No serious RTX-related adverse events requiring treatment discontinuation were observed. Conclusions Our study highlights RTX’s dual role both as a steroid-sparing maintenance therapy and as an early immunosuppressive option for steroid partial responders in addition to refractory AChR-positive gMG patients.

Clinical Neurology and NeurosurgeryVol. 271
Pasteur Hellenic Institute (GR), Eginition Hospital (GR), Cyprus Institute of Neurology and Genetics (CY)
Good health and well-being
Openalex Percentile: Top 10%
Myasthenia Gravis and Thymoma
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