Practice patterns in the management of myasthenia gravis: a cross-sectional survey of neurologists in the United States

Authors

  • Vera Bril MD Toronto General Hospital, University Health Network, University of Toronto, Toronto, Canada
  • Jacqueline Palace MD Department of Clinical Neurology, John Radcliffe Hospital, Oxford University Hospitals Trust, Oxford, UK
  • Tahseen Mozaffar MD UC Irvine-MDA ALS and Neuromuscular Center, University of California, Irvine, California, USA
  • Deborah Gelinas MD Medical Affairs, argenx, Boston, Massachusetts, USA
  • Edward Brauer PharmD Medical Affairs, argenx, Boston, Massachusetts, USA
  • Paul Nisbet PhD One Research, LLC, Charleston, South Carolina, USA
  • Gil I. Wolfe MD Department of Neurology, University at Buffalo Jacobs School of Medicine and Biomedical Sciences, State University of New York, Buffalo, New York, USA

DOI:

https://doi.org/10.17161/rrnmf.v2i5.15806

Keywords:

cross-sectional survey, myasthenia gravis, clinical practice guideline, clinical practice patterns, disease management

Abstract

Background: Management of myasthenia gravis (MG), a rare immunoglobulin G autoantibody–mediated neuromuscular junction disorder, is driven by physician experience. To gain insight into current practices and physician needs, neurologists’ use of guidelines and disease activity evaluations to manage MG was assessed. Methods: In November and December of 2020, a quantitative, cross-sectional, 51-item, online survey–based study was used to collect data from 100 community neurologists, from 31 US states, who treat MG. Differences across ratio variables were analyzed via Chi-square and t tests, at a significance level of P<0.05. Results: Of respondents, 76% reported using clinical judgment rather than guidelines to inform treatment decisions, and only 29% reported awareness of the updated 2020 International Consensus Guidance for Management of Myasthenia Gravis. Treatment patterns reported include use of prednisone-equivalent corticosteroid doses ≤10 mg/day for ≥6 months (76% of respondents). When corticosteroids are contraindicated or after failure of an initial nonsteroidal immunosuppressant therapy (NSIST), immunoglobulin therapy is the respondents’ preferred initial treatment in patients with acetylcholine receptor antibody–positive generalized MG (vs a second NSIST). Respondents expressed interest in more guidance on crisis management, initiating/titrating maintenance medications, and managing patients with comorbidities. Conclusions: Respondents to this survey reported varied approaches to MG management and, in some clinical settings, heavier reliance on clinical judgment than on available consensus-based guidance. Also observed was potential underutilization of NSISTs in patients for whom corticosteroids are contraindicated, with reliance, instead, on immunoglobulin.

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References

Gilhus NE. Myasthenia Gravis. N Engl J Med. 2016;375(26):2570-81. Epub 2016/12/29. doi: 10.1056/NEJMra1602678. PubMed PMID: 28029925.

Farrugia ME, Goodfellow JA. A Practical Approach to Managing Patients With Myasthenia Gravis-Opinions and a Review of the Literature. Front Neurol. 2020;11:604. Epub 2020/08/01. doi: 10.3389/fneur.2020.00604. PubMed PMID: 32733360; PubMed Central PMCID: PMCPMC7358547.

Lazaridis K, Tzartos SJ. Autoantibody Specificities in Myasthenia Gravis; Implications for Improved Diagnostics and Therapeutics. Front Immunol. 2020;11:212. Epub 2020/03/03. doi: 10.3389/fimmu.2020.00212. PubMed PMID: 32117321; PubMed Central PMCID: PMCPMC7033452.

Deenen JC, Horlings CG, Verschuuren JJ, Verbeek AL, van Engelen BG. The Epidemiology of Neuromuscular Disorders: A Comprehensive Overview of the Literature. J Neuromuscul Dis. 2015;2(1):73-85. Epub 2015/01/01. PubMed PMID: 28198707.

Menon D, Barnett C, Bril V. Novel Treatments in Myasthenia Gravis. Front Neurol. 2020;11:538. Epub 2020/07/28. doi: 10.3389/fneur.2020.00538. PubMed PMID: 32714266; PubMed Central PMCID: PMCPMC7344308.

Gotterer L, Li Y. Maintenance immunosuppression in myasthenia gravis. J Neurol Sci. 2016;369:294-302. Epub 2016/09/23. doi: 10.1016/j.jns.2016.08.057. PubMed PMID: 27653912.

Sanders DB, Wolfe GI, Benatar M, Evoli A, Gilhus NE, Illa I, et al. International consensus guidance for management of myasthenia gravis: Executive summary. Neurology. 2016;87(4):419-25. Epub 2016/07/01. doi: 10.1212/WNL.0000000000002790. PubMed PMID: 27358333; PubMed Central PMCID: PMCPMC4977114.

Narayanaswami P, Sanders DB, Wolfe G, Benatar M, Cea G, Evoli A, et al. International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update. Neurology. 2021;96(3):114-22. Epub 2020/11/05. doi: 10.1212/WNL.0000000000011124. PubMed PMID: 33144515; PubMed Central PMCID: PMCPMC7884987.

Gable KL, Guptill JT. Antagonism of the Neonatal Fc Receptor as an Emerging Treatment for Myasthenia Gravis. Front Immunol. 2019;10:3052. Epub 2020/01/31. doi: 10.3389/fimmu.2019.03052. PubMed PMID: 31998320; PubMed Central PMCID: PMCPMC6965493.

Farmakidis C, Pasnoor M, Dimachkie MM, Barohn RJ. Treatment of Myasthenia Gravis. Neurol Clin. 2018;36(2):311-37. Epub 2018/04/16. doi: 10.1016/j.ncl.2018.01.011. PubMed PMID: 29655452; PubMed Central PMCID: PMCPMC6690491.

Dalakas MC. Progress in the therapy of myasthenia gravis: getting closer to effective targeted immunotherapies. Curr Opin Neurol. 2020;33(5):545-52. Epub 2020/08/25. doi: 10.1097/WCO.0000000000000858. PubMed PMID: 32833750.

Mantegazza R, Antozzi C. When myasthenia gravis is deemed refractory: clinical signposts and treatment strategies. Ther Adv Neurol Disord. 2018;11:1756285617749134. Epub 2018/02/07. doi: 10.1177/1756285617749134. PubMed PMID: 29403543; PubMed Central PMCID: PMCPMC5791553.

Howard JF, Jr., Bril V, Vu T, Karam C, Peric S, Margania T, et al. Safety, efficacy, and tolerability of efgartigimod in patients with generalised myasthenia gravis (ADAPT): a multicentre, randomised, placebo-controlled, phase 3 trial. Lancet Neurol. 2021;20(7):526-36. Epub 2021/06/20. doi: 10.1016/S1474-4422(21)00159-9. PubMed PMID: 34146511.

Jayalakshmi S, Vooturi S. Legal challenges in neurological practice. Ann Indian Acad Neurol. 2016;19(Suppl 1):S3-S8. Epub 2016/11/29. doi: 10.4103/0972-2327.192888. PubMed PMID: 27891018; PubMed Central PMCID: PMCPMC5109758.

American Academy of Neurology. Policy & Guidelines/Neuromuscular [February 10, 2021]. Available from: https://www.aan.com/Guidelines/home/ByTopic?topicId=19.

Buttgereit F, Bijlsma JW. Glucocorticoids in rheumatoid arthritis: the picture is shaping up. Ann Rheum Dis. 2017;76(11):1785-7. Epub 2017/05/06. doi: 10.1136/annrheumdis-2017-211187. PubMed PMID: 28473424.

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Published

2021-12-20

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Section

New Discoveries/New Stuff (Original Research)

How to Cite

Bril, V., Palace, J., Mozaffar, T., Gelinas, D. ., Brauer, E., Nisbet, P., & Wolfe, G. (2021). Practice patterns in the management of myasthenia gravis: a cross-sectional survey of neurologists in the United States. RRNMF Neuromuscular Journal, 2(5), 29-49. https://doi.org/10.17161/rrnmf.v2i5.15806