A single low-dose rituximab infusion in severe chronic refractory myasthenia gravis in resource-limited settings
Copyright © 2022 Elsevier B.V. All rights reserved..
The benefits of multi-dose rituximab cycles in patients with refractory anti-muscle-specific kinase antibody myasthenia gravis (MuSK+MG) are well reported, although less consistently in anti-acetylcholine receptor antibody MG (AChR+MG). Responsivity data to single low-dose rituximab infusions for refractory autoimmune myasthenia, are limited. Here, observational outcomes using MG grading scores and prednisone doses, before and after at least six months of a single-dose infusion of rituximab, were audited in previously treatment-refractory MG patients in a resource-limited setting. Seventeen moderately-severe to severely symptomatic MG patients received single low-dose rituximab infusions (median 500-600 mg) after a median MG duration of 6 years; 13 individuals responded including 5/5 MuSK+MG, 7/10 AChR+MG and 1/2 double seronegative MG. Three (60%) MuSK+MG and three (30%) AChR+MG achieved persistent asymptomatic status. Although more MuSK+MG vs AChR+MG cases stopped prednisone (80% vs 20%, respectively), the prednisone doses in the AChR+MG group was significantly reduced ≥30% (p = 0.008) due to improved MG composite scores (p = 0.016) and with durable benefit (median 12 months). There were no differences between responders and non-responders in MG duration and age at infusion. These results suggest that a single low-dose rituximab infusion is worth trying in refractory MG, including AChR+MG patients, as some patients showed good and durable responses. These results are particularly relevant to resource-limited settings.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:442 |
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| Enthalten in: |
Journal of the neurological sciences - 442(2022) vom: 15. Nov., Seite 120394 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Heckmann, Jeannine M [VerfasserIn] |
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| Links: |
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| Themen: |
4F4X42SYQ6 |
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| Anmerkungen: |
Date Completed 27.10.2022 Date Revised 23.02.2023 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.jns.2022.120394 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM345744667 |
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| 520 | |a Copyright © 2022 Elsevier B.V. All rights reserved. | ||
| 520 | |a The benefits of multi-dose rituximab cycles in patients with refractory anti-muscle-specific kinase antibody myasthenia gravis (MuSK+MG) are well reported, although less consistently in anti-acetylcholine receptor antibody MG (AChR+MG). Responsivity data to single low-dose rituximab infusions for refractory autoimmune myasthenia, are limited. Here, observational outcomes using MG grading scores and prednisone doses, before and after at least six months of a single-dose infusion of rituximab, were audited in previously treatment-refractory MG patients in a resource-limited setting. Seventeen moderately-severe to severely symptomatic MG patients received single low-dose rituximab infusions (median 500-600 mg) after a median MG duration of 6 years; 13 individuals responded including 5/5 MuSK+MG, 7/10 AChR+MG and 1/2 double seronegative MG. Three (60%) MuSK+MG and three (30%) AChR+MG achieved persistent asymptomatic status. Although more MuSK+MG vs AChR+MG cases stopped prednisone (80% vs 20%, respectively), the prednisone doses in the AChR+MG group was significantly reduced ≥30% (p = 0.008) due to improved MG composite scores (p = 0.016) and with durable benefit (median 12 months). There were no differences between responders and non-responders in MG duration and age at infusion. These results suggest that a single low-dose rituximab infusion is worth trying in refractory MG, including AChR+MG patients, as some patients showed good and durable responses. These results are particularly relevant to resource-limited settings | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Lambert-Eaton myasthenic syndrome | |
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| 650 | 4 | |a Refractory myasthenia gravis | |
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| 650 | 7 | |a Autoantibodies |2 NLM | |
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