Generalized myasthenia gravis with acetylcholine receptor antibodies: A guidance for treatment

被引:11
|
作者
Gilhus, Nils Erik [1 ,2 ]
Andersen, Henning [3 ]
Andersen, Linda Kahr [4 ]
Boldingh, Marion [5 ]
Laakso, Sini [6 ,7 ]
Leopoldsdottir, Margret Oddny [8 ]
Madsen, Sidsel [9 ]
Piehl, Fredrik [10 ,11 ]
Popperud, Trine Haug [5 ]
Punga, Anna Rostedt [12 ,13 ]
Schirakow, Liselotte [14 ]
Vissing, John [4 ]
机构
[1] Haukeland Hosp, Dept Neurol, N-5021 Bergen, Norway
[2] Univ Bergen, Dept Clin Med, Bergen, Norway
[3] Aarhus Univ Hosp, Dept Neurol, Aarhus, Denmark
[4] Copenhagen Univ Hosp, Copenhagen Neuromuscular Ctr, Dept Neurol, Copenhagen, Denmark
[5] Oslo Univ Hosp, Dept Neurol, Oslo, Norway
[6] Helsinki Univ Hosp, Brain Ctr, Dept Neurol, Helsinki, Finland
[7] Univ Helsinki, Translat Immunol Res Program, Helsinki, Finland
[8] MG Felag Isl, Reykjavik, Iceland
[9] Natl Rehabil Ctr Neuromuscular Dis, Aarhus, Denmark
[10] Karolinska Inst, Dept Clin Neurosci, Stockholm, Sweden
[11] Karolinska Univ Hosp, Dept Neurol, Stockholm, Sweden
[12] Uppsala Univ, Dept Med Sci, Uppsala, Sweden
[13] Uppsala Univ Hosp, Dept Clin Neurophysiol, Uppsala, Sweden
[14] Danish Muscular Dystrophy Org, MG Grp, Aarhus, Denmark
关键词
acetylcholine receptor antibodies; immunosuppression; myasthenia gravis; thymus; treatment; INTERNATIONAL CONSENSUS GUIDANCE; DOUBLE-BLIND; PHYSICAL-EXERCISE; RANDOMIZED-TRIAL; RISK-FACTORS; THYMECTOMY; PREGNANCY; GUIDELINES; MANAGEMENT; EFFICACY;
D O I
10.1111/ene.16229
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
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页数:13
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