Myasthenia gravis – Update

Myasthenia gravis – auch 2023 noch eine Herausforderung für Betroffene und Ärzte. Aber welche Therapie ist am besten geeignet? Unsere klinisch erfahrenen Experten haben die aktuellen Leitlinien zur Diagnostik und Therapie zusammengefasst, um Betroffene optimal zu unterstützen. Erfahren Sie, wie Sie eine schnelle und zielgerichtete Diagnostik durchführen können und welche Behandlungsoptionen zur Verfügung stehen, um den Verlauf der Erkrankung zu mildern.

Abstract

Myasthenia gravis – still a challenge for sufferers and doctors in 2023. But which therapy is best suited? Our clinically experienced experts have summarized the current guidelines for diagnosis and treatment in order to provide optimal support for those affected. Find out how you can carry out a quick and targeted diagnosis and which treatment options are available to alleviate the course of the disease.

Schlüsselwörter

Myastenia gravis - Glukokortikosteroide - Immuntherapie - Komplementinhibitoren - FcRN-Modulatoren - kongenitales myasthenes Syndrom - Lambert-Eaton-Myasthenie-Syndrom

Key word

Myastenia gravis - glucocorticosteroids - immunotherapy - complement inhibitors - FcRN modulators - congenital myasthenic syndrome - Lambert-Eaton myasthenic syndrome

• Literatur

• 1 Berrih-Aknin S, Frenkian-Cuvelier M, Eymard B. Diagnostic and clinical classification of autoimmune myasthenia gravis. J Autoimmun 2014; 48-49: 143-148
• 2 Gasperi C, Melms A, Schoser B. et al. Anti-agrin autoantibodies in myasthenia gravis. Neurology 2014; 82: 1976-1983
• 3 Wiendl H, Meisel A. et al. Diagnostik und Therapie myasthener Syndrome, S2k-Leitlinie. In: DGN ed, Leitlinien für Diagnostik und Therapie in der Neurologie. Deutsche Gesellschaft für Neurologie, Hrsg.; 2022. Zugriff am 19. Juli 2023 https://dgn.org/leitlinie/diagnostik-und-therapie-der-myasthenia-gravis-und-des-lambert-eaton-syndroms
• 4 Jaretzki A, Barohn RJ, Ernstoff RM. et al. Myasthenia gravis: recommendations for clinical research standards. Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America. Neurology 2000; 55: 16-23
• 5 Westerberg E, Punga AR. Epidemiology of myasthenia gravis in Sweden 2006-2016. Brain Behav 2020; 10: e01819
• 6 Popperud TH, Boldingh MI, Brunborg C. et al. Juvenile myasthenia gravis in Norway: A nationwide epidemiological study. Eur J Paediatr Neurol 2017; 21: 312-317
• 7 Mevius A, Jores L, Biskup J. et al. Epidemiology and treatment of myasthenia gravis: a retrospective study using a large insurance claims dataset in Germany. Neuromuscul Disord 2023; 33: 324-333
• 8 Browning J, Wallace M, Chana J. et al. Bedside testing for myasthenia gravis: the ice-test. BMJ Case Rep 2010; 2010
• 9 Benatar M. A systematic review of diagnostic studies in myasthenia gravis. Neuromuscul Disord 2006; 16: 459-467
• 10 Berkhoff M, Sturzenegger M, Schroth G. et al. Okulärer Myositis. Nervenarzt 1997; 68: 792-800
• 11 Sanders DB, Wolfe GI, Benatar M. et al. International consensus guidance for management of myasthenia gravis: Executive summary. Neurology 2016; 87: 419-425
• 12 Maggi L, Mantegazza R. Treatment of myasthenia gravis: focus on pyridostigmine. Clin Drug Investig 2011; 31: 691-701
• 13 Skeie GO, Apostolski S, Evoli A. et al. Guidelines for treatment of autoimmune neuromuscular transmission disorders. Eur J Neurol 2010; 17: 893-902
• 14 Schneider-Gold C, Gajdos P, Toyka KV. et al. Corticosteroids for myasthenia gravis. Cochrane Database Syst Rev 2005; 2005: CD002828
• 15 Howard JF, Utsugisawa K, Benatar M. et al. Safety and efficacy of eculizumab in anti-acetylcholine receptor antibodypositive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study. Lancet Neurol 2017; 16: 976-986
• 16 Howard JF, Barohn RJ, Cutter GR. et al. A randomized, double-blind, placebo-controlled phase II study of eculizumab in patients with refractory generalized myasthenia gravis. Muscle Nerve 2013; 48: 76-84
• 17 Muppidi S, Utsugisawa K, Benatar M. et al. Long-term safety and efficacy of eculizumab in generalized myasthenia gravis. Muscle Nerve 2019; 60: 14-24
• 18 Mantegazza R, Wolfe GI, Muppidi S. et al. Post-intervention status in patients with refractory myasthenia gravis treated with eculizumab during REGAIN and its open-label extension. Neurology 2021; 96: e610-e618
• 19 Howard JF, Karam C, Yountz M. et al. Long-term efficacy of eculizumab in refractory generalized myasthenia gravis: responder analyses. Ann Clin Transl Neurol 2021; 8: 1398-1407
• 20 Howard JF, Bresch S, Genge A. et al. Safety and efficacy of zilucoplan in patients with generalised myasthenia gravis (RAISE): a randomised, double-blind, placebo-controlled, phase 3 study. Lancet Neurol 2023; 22: 395-406
• 21 Howard JF, Bril V, Vu 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: 526-536
• 22 Ulrichts P, Guglietta A, Dreier T. et al. Neonatal Fc receptor antagonist efgartigimod safely and sustainably reduces IgGs in humans. J Clin Invest 2018; 128: 4372-4386
• 23 Bril V, Benatar M, Andersen H. et al. Efficacy and Safety of Rozanolixizumab in Moderate to Severe Generalized Myasthenia Gravis: A Phase 2 Randomized Control Trial. Neurology 2021; 96: e853-e865
• 24 Bril V, Druzdz A, Grosskreutz J. et al. Safety and efficacy of rozanolixizumab in patients with generalised myasthenia gravis (MycarinG): a randomised, double-blind, placebocontrolled, adaptive phase 3 study. Lancet Neurol 2023; 22: 383-394
• 25 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
• 26 Wolfe GI, Kaminski HJ, Aban IB. et al. Randomized Trial of Thymectomy in Myasthenia Gravis. N Engl J Med 2016; 375: 511-522
• 27 Wada S, Kamei M, Uehara N. et al. Paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome with SOX-1 antibodies. Intern Med 2021; 60: 1607-1610
• 28 Lipka AF, Verschuuren JJ, Titulaer MJ. SOX1 antibodies in Lambert-Eaton myasthenic syndrome and screening for small cell lung carcinoma. Ann N Y Acad Sci 2012; 1275: 70-77