TY - JOUR
T1 - Generalized myasthenia gravis with acetylcholine receptor antibodies
T2 - A guidance for treatment
AU - Gilhus, Nils Erik
AU - Andersen, Henning
AU - Andersen, Linda Kahr
AU - Boldingh, Marion
AU - Laakso, Sini
AU - Leopoldsdottir, Margret Oddny
AU - Madsen, Sidsel
AU - Piehl, Fredrik
AU - Popperud, Trine Haug
AU - Punga, Anna Rostedt
AU - Schirakow, Liselotte
AU - Vissing, John
N1 - Publisher Copyright:
© 2024 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.
PY - 2024/5
Y1 - 2024/5
N2 - Background: Generalized myasthenia gravis (MG) with antibodies against the acetylcholine receptor is a chronic disease causing muscle weakness. Access to novel treatments warrants authoritative treatment recommendations. The Nordic countries have similar, comprehensive health systems, mandatory health registers, and extensive MG research. Methods: MG experts and patient representatives from the five Nordic countries formed a working group to prepare treatment guidance for MG based on a systematic literature search and consensus meetings. Results: Pyridostigmine represents the first-line symptomatic treatment, while ambenonium and beta adrenergic agonists are second-line options. Early thymectomy should be undertaken if a thymoma, and in non-thymoma patients up to the age of 50–65 years if not obtaining remission on symptomatic treatment. Most patients need immunosuppressive drug treatment. Combining corticosteroids at the lowest possible dose with azathioprine is recommended, rituximab being an alternative first-line option. Mycophenolate, methotrexate, and tacrolimus represent second-line immunosuppression. Plasma exchange and intravenous immunoglobulin are used for myasthenic crises and acute exacerbations. Novel complement inhibitors and FcRn blockers are effective and fast-acting treatments with promising safety profiles. Their use depends on local availability, refunding policies, and cost–benefit analyses. Adapted physical training is recommended. Planning of pregnancies with optimal treatment, information, and awareness of neonatal MG is necessary. Social support and adaptation of work and daily life activities are recommended. Conclusions: Successful treatment of MG rests on timely combination of different interventions. Due to spontaneous disease fluctuations, comorbidities, and changes in life conditions, regular long-term specialized follow-up is needed. Most patients do reasonably well but there is room for further improvement. Novel treatments are promising, though subject to restricted access due to costs.
AB - Background: Generalized myasthenia gravis (MG) with antibodies against the acetylcholine receptor is a chronic disease causing muscle weakness. Access to novel treatments warrants authoritative treatment recommendations. The Nordic countries have similar, comprehensive health systems, mandatory health registers, and extensive MG research. Methods: MG experts and patient representatives from the five Nordic countries formed a working group to prepare treatment guidance for MG based on a systematic literature search and consensus meetings. Results: Pyridostigmine represents the first-line symptomatic treatment, while ambenonium and beta adrenergic agonists are second-line options. Early thymectomy should be undertaken if a thymoma, and in non-thymoma patients up to the age of 50–65 years if not obtaining remission on symptomatic treatment. Most patients need immunosuppressive drug treatment. Combining corticosteroids at the lowest possible dose with azathioprine is recommended, rituximab being an alternative first-line option. Mycophenolate, methotrexate, and tacrolimus represent second-line immunosuppression. Plasma exchange and intravenous immunoglobulin are used for myasthenic crises and acute exacerbations. Novel complement inhibitors and FcRn blockers are effective and fast-acting treatments with promising safety profiles. Their use depends on local availability, refunding policies, and cost–benefit analyses. Adapted physical training is recommended. Planning of pregnancies with optimal treatment, information, and awareness of neonatal MG is necessary. Social support and adaptation of work and daily life activities are recommended. Conclusions: Successful treatment of MG rests on timely combination of different interventions. Due to spontaneous disease fluctuations, comorbidities, and changes in life conditions, regular long-term specialized follow-up is needed. Most patients do reasonably well but there is room for further improvement. Novel treatments are promising, though subject to restricted access due to costs.
KW - acetylcholine receptor antibodies
KW - immunosuppression
KW - myasthenia gravis
KW - thymus
KW - treatment
KW - Autoantibodies
KW - Neuromuscular Diseases
KW - Humans
KW - Middle Aged
KW - Thymus Neoplasms
KW - Myasthenia Gravis/drug therapy
KW - Pregnancy
KW - Thymectomy
KW - Pyridostigmine Bromide/therapeutic use
KW - Female
KW - Aged
KW - Immunosuppressive Agents/therapeutic use
KW - Infant, Newborn
KW - Receptors, Cholinergic
UR - http://www.scopus.com/inward/record.url?scp=85184395090&partnerID=8YFLogxK
U2 - 10.1111/ene.16229
DO - 10.1111/ene.16229
M3 - Journal article
C2 - 38321574
AN - SCOPUS:85184395090
SN - 1351-5101
VL - 31
JO - European Journal of Neurology
JF - European Journal of Neurology
IS - 5
M1 - e16229
ER -