PDF(Pubmed)
Abstract：
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.
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.
摘要：
背景：带有抗乙酰胆碱受体抗体的广泛性重症肌无力（MG）是一种引起肌肉无力的慢性疾病。获得新的治疗方法需要权威的治疗建议。北欧国家有类似的，全面的卫生系统，强制性健康登记，和广泛的MG研究。
方法：来自北欧五国的MG专家和患者代表组成了一个工作组，以系统的文献检索和共识会议为基础，为MG制定治疗指南。
结果：吡啶斯的明代表一线对症治疗，而氨苯铵和β肾上腺素能激动剂是二线选择。如果胸腺瘤，应进行早期胸腺切除术，在50-65岁的非胸腺瘤患者中，如果对症治疗未得到缓解。大多数患者需要免疫抑制药物治疗。建议将皮质类固醇以最低剂量与硫唑嘌呤联合使用，利妥昔单抗是另一种一线选择。霉酚酸酯,甲氨蝶呤,他克莫司代表二线免疫抑制。血浆置换和静脉注射免疫球蛋白用于肌无力危象和急性加重。新型补体抑制剂和FcRn阻断剂是有效且快速作用的治疗方法，具有有希望的安全性。它们的使用取决于当地的可用性，退款政策，和成本效益分析。建议进行适应性体能训练。用最佳治疗计划怀孕，信息,对新生儿MG的认识是必要的。建议社会支持和适应工作和日常生活活动。
结论：MG的成功治疗取决于不同干预措施的及时组合。由于自发性疾病波动，合并症,和生活条件的变化，需要定期的长期专门随访。大多数患者做得相当好，但仍有进一步改善的空间。新的治疗方法很有希望，尽管由于费用限制进入。
方法：来自北欧五国的MG专家和患者代表组成了一个工作组，以系统的文献检索和共识会议为基础，为MG制定治疗指南。
结果：吡啶斯的明代表一线对症治疗，而氨苯铵和β肾上腺素能激动剂是二线选择。如果胸腺瘤，应进行早期胸腺切除术，在50-65岁的非胸腺瘤患者中，如果对症治疗未得到缓解。大多数患者需要免疫抑制药物治疗。建议将皮质类固醇以最低剂量与硫唑嘌呤联合使用，利妥昔单抗是另一种一线选择。霉酚酸酯,甲氨蝶呤,他克莫司代表二线免疫抑制。血浆置换和静脉注射免疫球蛋白用于肌无力危象和急性加重。新型补体抑制剂和FcRn阻断剂是有效且快速作用的治疗方法，具有有希望的安全性。它们的使用取决于当地的可用性，退款政策，和成本效益分析。建议进行适应性体能训练。用最佳治疗计划怀孕，信息,对新生儿MG的认识是必要的。建议社会支持和适应工作和日常生活活动。
结论：MG的成功治疗取决于不同干预措施的及时组合。由于自发性疾病波动，合并症,和生活条件的变化，需要定期的长期专门随访。大多数患者做得相当好，但仍有进一步改善的空间。新的治疗方法很有希望，尽管由于费用限制进入。
