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Review
. 2021 Jun;36(3):348-363.
doi: 10.1002/jca.21868. Epub 2020 Dec 21.

Treatment of refractory myasthenia gravis by double-filtration plasmapheresis and rituximab: A case series of nine patients and literature review

Hamza N Bennani  1 , Emmeline Lagrange  2 , Johan Noble  1 , Paolo Malvezzi  1 , Lionel Motte  1 , Eloi Chevallier  1 , Lionel Rostaing  1   3 , Thomas Jouve  1   3
Affiliations
Review

Treatment of refractory myasthenia gravis by double-filtration plasmapheresis and rituximab: A case series of nine patients and literature review

Hamza N Bennani et al. J Clin Apher. 2021 Jun.

Abstract

Introduction: Myasthenia gravis (MG) is an autoimmune disease mediated by circulating autoantibodies (anti-AchR, anti-MuSK, etc.). More than 20% of myasthenic patients are refractory to conventional treatments (plasma exchange, IVIg, steroids, azathioprine, mycophenolate mofetil). Rituximab (B-lymphocyte-depleting anti-CD20) and apheresis (double-filtration plasmapheresis [DFPP] and immunoadsorption [IA]) are interesting therapeutic alternatives.

Methods: This monocentric pilot study included nine refractory myasthenic patients (March 2018 to May 2020) treated by DFPP and/or IA associated with rituximab (375 mg/m2 ). Clinical responses were assessed using the Myasthenia Gravis Foundation of America (MGFA) score.

Results: Average age of patients was 53 ± 17 years. Gender ratio (M/F) was 3:6. The combination of apheresis and rituximab reduced median MGFA score from IV to II after 12 months of follow-up. Clinical improvement assessed by MGFA score was sustained in the long-term for all patients, during an average follow-up of 14 ± 9 months, allowing them to be self-sufficient and out sick-leave. The median number of apheresis sessions was 7 (5-30). The dose of prednisolone was reduced in two patients from 40 mg/d and 30 mg/d to 7.5 mg/d and 10 mg/d, respectively. It was stopped in a patient who was taking 30 mg/d. No infectious, bleeding, or thrombosis complications were noted.

Conclusion: The combination of rituximab and DFPP was effective to treat refractory MG.

Keywords: apheresis; double-filtration plasmapheresis; immunoadsorption; myasthenia gravis; rituximab.

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References

REFERENCES

    1. Souto EB, Lima B, Campos JR, Martins-Gomes C, Souto SB, Silva AM. Myasthenia gravis: state of the art and new therapeutic strategies. J Neuroimmunol. 2019;337:577080.
    1. Devic P, Petiot P, Simonet T, et al. Antibodies to clustered acetylcholine receptor: expanding the phenotype. Eur J Neurol. 2014;21(1):130-134.
    1. Lazaridis K, Tzartos SJ. Autoantibody specificities in myasthenia gravis; implications for improved diagnostics and therapeutics. Front Immunol. 2020;11:212.
    1. Tandan R, Hehir MK, Waheed W, Howard DB. Rituximab treatment of myasthenia gravis: a systematic review. Muscle Nerve. 2017;56:185-196.
    1. Kohler W, Bucka C, Klingel R. A randomized and controlled study comparing immunoadsorption and plasma exchange in myasthenic crisis. J Clin Apher. 2011;26(6):347-355.

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