International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update

doi:10.1212/WNL.0000000000011124

Review

. 2021 Jan 19;96(3):114-122.

doi: 10.1212/WNL.0000000000011124. Epub 2020 Nov 3.

International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update

Affiliations

¹ From the Beth Israel Deaconess Medical Center/Harvard Medical School (P.N.), Boston, MA; Department of Neurology (D.B.S., J.M.), Duke University Medical Center, Durham, NC; Department of Neurology (G.W.), Univ. at Buffalo Jacobs School of Medicine and Biomedical Sciences, State University of New York, Buffalo, NY; Department of Neurology (M.B.), University of Miami, Miller School of Medicine. Miami, FL; Gabriel Cea (G.C.), Departamento de Ciencias Neurologicas, Facultad de Medicina, Universidad de Chile, Santiago, Chile; Dipartimento di Neuroscienze (A.E.), Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Roma, Italy; Department of Clinical Medicine (N.E.G.), University of Bergen, Norway; Isabel Illa (I.I.), Department of Neurology, Hospital Santa Creu i Sant Pau. Universitat Autònoma de Barcelona, Barcelona, ERN EURO-NMD and CIBERER U762, Spain; Departments of Pediatrics and Neurology (N.L.K.), Northwestern Feinberg School of Medicine, Chicago, IL; Neurology (A.M.), University of Tübingen Medical Centre, Tübingen, Germany; Department of Neurology (H.M.), International University of Health and Welfare, Narita, Japan; Department of Clinical Neurological Sciences (M.N.), Western University, London, ON, Canada; Department of Clinical Neurology (J.P.), John Radcliffe Hospital, Oxford University Hospitals Trust, Oxford, UK; Department of Neurology (D.R.), University of California, Davis, Davis, CA; and Department of Neurology (J.V.), Leiden University Medical Centre, Leiden, the Netherlands. pnarayan@bidmc.harvard.edu.
² From the Beth Israel Deaconess Medical Center/Harvard Medical School (P.N.), Boston, MA; Department of Neurology (D.B.S., J.M.), Duke University Medical Center, Durham, NC; Department of Neurology (G.W.), Univ. at Buffalo Jacobs School of Medicine and Biomedical Sciences, State University of New York, Buffalo, NY; Department of Neurology (M.B.), University of Miami, Miller School of Medicine. Miami, FL; Gabriel Cea (G.C.), Departamento de Ciencias Neurologicas, Facultad de Medicina, Universidad de Chile, Santiago, Chile; Dipartimento di Neuroscienze (A.E.), Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Roma, Italy; Department of Clinical Medicine (N.E.G.), University of Bergen, Norway; Isabel Illa (I.I.), Department of Neurology, Hospital Santa Creu i Sant Pau. Universitat Autònoma de Barcelona, Barcelona, ERN EURO-NMD and CIBERER U762, Spain; Departments of Pediatrics and Neurology (N.L.K.), Northwestern Feinberg School of Medicine, Chicago, IL; Neurology (A.M.), University of Tübingen Medical Centre, Tübingen, Germany; Department of Neurology (H.M.), International University of Health and Welfare, Narita, Japan; Department of Clinical Neurological Sciences (M.N.), Western University, London, ON, Canada; Department of Clinical Neurology (J.P.), John Radcliffe Hospital, Oxford University Hospitals Trust, Oxford, UK; Department of Neurology (D.R.), University of California, Davis, Davis, CA; and Department of Neurology (J.V.), Leiden University Medical Centre, Leiden, the Netherlands.

PMID: 33144515
PMCID: PMC7884987
DOI: 10.1212/WNL.0000000000011124

Review

International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update

Pushpa Narayanaswami et al. Neurology. 2021.

. 2021 Jan 19;96(3):114-122.

doi: 10.1212/WNL.0000000000011124. Epub 2020 Nov 3.

Authors

Affiliations

¹ From the Beth Israel Deaconess Medical Center/Harvard Medical School (P.N.), Boston, MA; Department of Neurology (D.B.S., J.M.), Duke University Medical Center, Durham, NC; Department of Neurology (G.W.), Univ. at Buffalo Jacobs School of Medicine and Biomedical Sciences, State University of New York, Buffalo, NY; Department of Neurology (M.B.), University of Miami, Miller School of Medicine. Miami, FL; Gabriel Cea (G.C.), Departamento de Ciencias Neurologicas, Facultad de Medicina, Universidad de Chile, Santiago, Chile; Dipartimento di Neuroscienze (A.E.), Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Roma, Italy; Department of Clinical Medicine (N.E.G.), University of Bergen, Norway; Isabel Illa (I.I.), Department of Neurology, Hospital Santa Creu i Sant Pau. Universitat Autònoma de Barcelona, Barcelona, ERN EURO-NMD and CIBERER U762, Spain; Departments of Pediatrics and Neurology (N.L.K.), Northwestern Feinberg School of Medicine, Chicago, IL; Neurology (A.M.), University of Tübingen Medical Centre, Tübingen, Germany; Department of Neurology (H.M.), International University of Health and Welfare, Narita, Japan; Department of Clinical Neurological Sciences (M.N.), Western University, London, ON, Canada; Department of Clinical Neurology (J.P.), John Radcliffe Hospital, Oxford University Hospitals Trust, Oxford, UK; Department of Neurology (D.R.), University of California, Davis, Davis, CA; and Department of Neurology (J.V.), Leiden University Medical Centre, Leiden, the Netherlands. pnarayan@bidmc.harvard.edu.
² From the Beth Israel Deaconess Medical Center/Harvard Medical School (P.N.), Boston, MA; Department of Neurology (D.B.S., J.M.), Duke University Medical Center, Durham, NC; Department of Neurology (G.W.), Univ. at Buffalo Jacobs School of Medicine and Biomedical Sciences, State University of New York, Buffalo, NY; Department of Neurology (M.B.), University of Miami, Miller School of Medicine. Miami, FL; Gabriel Cea (G.C.), Departamento de Ciencias Neurologicas, Facultad de Medicina, Universidad de Chile, Santiago, Chile; Dipartimento di Neuroscienze (A.E.), Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Roma, Italy; Department of Clinical Medicine (N.E.G.), University of Bergen, Norway; Isabel Illa (I.I.), Department of Neurology, Hospital Santa Creu i Sant Pau. Universitat Autònoma de Barcelona, Barcelona, ERN EURO-NMD and CIBERER U762, Spain; Departments of Pediatrics and Neurology (N.L.K.), Northwestern Feinberg School of Medicine, Chicago, IL; Neurology (A.M.), University of Tübingen Medical Centre, Tübingen, Germany; Department of Neurology (H.M.), International University of Health and Welfare, Narita, Japan; Department of Clinical Neurological Sciences (M.N.), Western University, London, ON, Canada; Department of Clinical Neurology (J.P.), John Radcliffe Hospital, Oxford University Hospitals Trust, Oxford, UK; Department of Neurology (D.R.), University of California, Davis, Davis, CA; and Department of Neurology (J.V.), Leiden University Medical Centre, Leiden, the Netherlands.

PMID: 33144515
PMCID: PMC7884987
DOI: 10.1212/WNL.0000000000011124

Abstract

Objective: To update the 2016 formal consensus-based guidance for the management of myasthenia gravis (MG) based on the latest evidence in the literature.

Methods: In October 2013, the Myasthenia Gravis Foundation of America appointed a Task Force to develop treatment guidance for MG, and a panel of 15 international experts was convened. The RAND/UCLA appropriateness method was used to develop consensus recommendations pertaining to 7 treatment topics. In February 2019, the international panel was reconvened with the addition of one member to represent South America. All previous recommendations were reviewed for currency, and new consensus recommendations were developed on topics that required inclusion or updates based on the recent literature. Up to 3 rounds of anonymous e-mail votes were used to reach consensus, with modifications to recommendations between rounds based on the panel input. A simple majority vote (80% of panel members voting "yes") was used to approve minor changes in grammar and syntax to improve clarity.

Results: The previous recommendations for thymectomy were updated. New recommendations were developed for the use of rituximab, eculizumab, and methotrexate as well as for the following topics: early immunosuppression in ocular MG and MG associated with immune checkpoint inhibitor treatment.

Conclusion: This updated formal consensus guidance of international MG experts, based on new evidence, provides recommendations to clinicians caring for patients with MG worldwide.

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References

1. Sanders DB, Wolfe GI, Benatar M, et al. . International consensus guidance for management of myasthenia gravis: executive summary. Neurology 2016;87:419–425. - PMC - PubMed
1. Fitch K, Bernstein SJ, Aguilar MD, et al. . The RAND/UCLA Appropriateness Method User's Manual. [online]. Available at: rand.org/pubs/monograph_reports/MR1269.html. Accessed July 10, 2020.
1. Wolfe GI, Kaminski HJ, Aban IB, et al. . Randomized trial of thymectomy in myasthenia gravis. N Engl J Med 2016;375:511–522. - PMC - PubMed
1. Wolfe GI, Kaminski HJ, Aban IB, et al. . Long-term effect of thymectomy plus prednisone versus prednisone alone in patients with non-thymomatous myasthenia gravis: 2-year extension of the MGTX randomised trial. Lancet Neurol 2019;18:259–268. - PMC - PubMed
1. Gronseth GS, Barohn R, Narayanaswami P. Practice advisory: thymectomy for myasthenia gravis (practice parameter update): report of the guideline development, dissemination, and implementation subcommittee of the American Academy of Neurology. Neurology 2020;94:705–709. - PubMed

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[1] Sanders DB, Wolfe GI, Benatar M, et al. . International consensus guidance for management of myasthenia gravis: executive summary. Neurology 2016;87:419–425. - PMC - PubMed

[2] Sanders DB, Wolfe GI, Benatar M, et al. . International consensus guidance for management of myasthenia gravis: executive summary. Neurology 2016;87:419–425. - PMC - PubMed

[3] Fitch K, Bernstein SJ, Aguilar MD, et al. . The RAND/UCLA Appropriateness Method User's Manual. [online]. Available at: rand.org/pubs/monograph_reports/MR1269.html. Accessed July 10, 2020.

[4] Fitch K, Bernstein SJ, Aguilar MD, et al. . The RAND/UCLA Appropriateness Method User's Manual. [online]. Available at: rand.org/pubs/monograph_reports/MR1269.html. Accessed July 10, 2020.

[5] Wolfe GI, Kaminski HJ, Aban IB, et al. . Randomized trial of thymectomy in myasthenia gravis. N Engl J Med 2016;375:511–522. - PMC - PubMed

[6] Wolfe GI, Kaminski HJ, Aban IB, et al. . Randomized trial of thymectomy in myasthenia gravis. N Engl J Med 2016;375:511–522. - PMC - PubMed

[7] Wolfe GI, Kaminski HJ, Aban IB, et al. . Long-term effect of thymectomy plus prednisone versus prednisone alone in patients with non-thymomatous myasthenia gravis: 2-year extension of the MGTX randomised trial. Lancet Neurol 2019;18:259–268. - PMC - PubMed

[8] Wolfe GI, Kaminski HJ, Aban IB, et al. . Long-term effect of thymectomy plus prednisone versus prednisone alone in patients with non-thymomatous myasthenia gravis: 2-year extension of the MGTX randomised trial. Lancet Neurol 2019;18:259–268. - PMC - PubMed

[9] Gronseth GS, Barohn R, Narayanaswami P. Practice advisory: thymectomy for myasthenia gravis (practice parameter update): report of the guideline development, dissemination, and implementation subcommittee of the American Academy of Neurology. Neurology 2020;94:705–709. - PubMed

[10] Gronseth GS, Barohn R, Narayanaswami P. Practice advisory: thymectomy for myasthenia gravis (practice parameter update): report of the guideline development, dissemination, and implementation subcommittee of the American Academy of Neurology. Neurology 2020;94:705–709. - PubMed

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International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update

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International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update

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