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A single low-dose rituximab infusion in severe chronic refractory myasthenia gravis in resource-limited settings

  • Jeannine M. Heckmann
    Correspondence
    Corresponding author at: Neurology Division E8-74, Groote Schuur Hospital, Observatory, 7925 Cape Town, South Africa.
    Affiliations
    Neurology Division, Department of Medicine, Health Sciences Faculty, University of Cape Town, Cape Town, South Africa
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Published:August 29, 2022DOI:https://doi.org/10.1016/j.jns.2022.120394

      Highlights

      • Single dose rituximab is worth trying in refractory AChR+ myasthenia.
      • Refractory MG in resource-limited areas may stabilise after a rituximab dose.
      • Prednisone dosing was reduced by >70% in responders.
      • The clinical response to a single dose of rituximab may be long-lasting.
      • CD19+ counts do not always predict MG treatment responsivity or relapse.

      Abstract

      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.

      Keywords

      Abbreviations:

      ACHR+MG (acetylcholine receptor antibody positive myasthenia gravis), DSN-MG (double seronegative (AChR-/MuSK-) myasthenia gravis), MuSK+M (muscle specific kinase antibody positive myasthenia gravis), MGFA (MG Foundation of America), MMS (minimal manifestation status), RhA (rheumatoid arthritis)
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