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Abstract：
BACKGROUND: Important progress has been made in our understanding of the autoimmune neuromuscular transmission (NMT) disorders; myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS) and neuromyotonia (Isaacs\' syndrome).
METHODS: To prepare consensus guidelines for the treatment of the autoimmune NMT disorders, references retrieved from MEDLINE, EMBASE and the Cochrane Library were considered and statements prepared and agreed on by disease experts.
CONCLUSIONS: Anticholinesterase drugs should be given first in the management of MG, but with some caution in patients with MuSK antibodies (good practice point). Plasma exchange is recommended in severe cases to induce remission and in preparation for surgery (recommendation level B). IvIg and plasma exchange are effective for the treatment of MG exacerbations (recommendation level A). For patients with non-thymomatous MG, thymectomy is recommended as an option to increase the probability of remission or improvement (recommendation level B). Once thymoma is diagnosed, thymectomy is indicated irrespective of MG severity (recommendation level A). Oral corticosteroids are first choice drugs when immunosuppressive drugs are necessary (good practice point). When long-term immunosuppression is necessary, azathioprine is recommended to allow tapering the steroids to the lowest possible dose whilst maintaining azathioprine (recommendation level A). 3,4-Diaminopyridine is recommended as symptomatic treatment and IvIG has a positive short-term effect in LEMS (good practice point). Neuromyotonia patients should be treated with an antiepileptic drug that reduces peripheral nerve hyperexcitability (good practice point). For paraneoplastic LEMS and neuromyotonia optimal treatment of the underlying tumour is essential (good practice point). Immunosuppressive treatment of LEMS and neuromyotonia should be similar to MG (good practice point).
METHODS: To prepare consensus guidelines for the treatment of the autoimmune NMT disorders, references retrieved from MEDLINE, EMBASE and the Cochrane Library were considered and statements prepared and agreed on by disease experts.
CONCLUSIONS: Anticholinesterase drugs should be given first in the management of MG, but with some caution in patients with MuSK antibodies (good practice point). Plasma exchange is recommended in severe cases to induce remission and in preparation for surgery (recommendation level B). IvIg and plasma exchange are effective for the treatment of MG exacerbations (recommendation level A). For patients with non-thymomatous MG, thymectomy is recommended as an option to increase the probability of remission or improvement (recommendation level B). Once thymoma is diagnosed, thymectomy is indicated irrespective of MG severity (recommendation level A). Oral corticosteroids are first choice drugs when immunosuppressive drugs are necessary (good practice point). When long-term immunosuppression is necessary, azathioprine is recommended to allow tapering the steroids to the lowest possible dose whilst maintaining azathioprine (recommendation level A). 3,4-Diaminopyridine is recommended as symptomatic treatment and IvIG has a positive short-term effect in LEMS (good practice point). Neuromyotonia patients should be treated with an antiepileptic drug that reduces peripheral nerve hyperexcitability (good practice point). For paraneoplastic LEMS and neuromyotonia optimal treatment of the underlying tumour is essential (good practice point). Immunosuppressive treatment of LEMS and neuromyotonia should be similar to MG (good practice point).
摘要：
背景：我们对自身免疫性神经肌肉传递（NMT）疾病的理解取得了重要进展；重症肌无力（MG），Lambert-Eaton肌无力综合征（LEMS）和神经肌强直（Isaacs综合征）。
方法：为了制定治疗自身免疫性NMT疾病的共识指南，从MEDLINE检索的引用，考虑了EMBASE和Cochrane图书馆，并由疾病专家编写并同意了声明。
结论：MG的管理应首先使用抗胆碱酯酶药物，但对患有MuSK抗体的患者要谨慎（良好的做法）。建议在严重病例中进行血浆置换以诱导缓解并准备手术（建议B级）。IvIg和血浆置换可有效治疗MG加重（建议A级）。对于非胸腺瘤MG患者，推荐胸腺切除术作为增加缓解或改善概率的一种选择(推荐水平B)。一旦诊断出胸腺瘤，无论MG严重程度如何，都应进行胸腺切除术（建议A级）。当需要免疫抑制药物时，口服皮质类固醇是首选药物（良好的实践要点）。当需要长期免疫抑制时，建议硫唑嘌呤允许将类固醇逐渐减少至最低剂量，同时维持硫唑嘌呤（建议水平A）。3,4-二氨基吡啶被推荐作为对症治疗，IvIG在LEMS中具有积极的短期效果（良好的实践要点）。神经肌强直患者应使用减少周围神经兴奋过度的抗癫痫药物治疗（好的做法要点）。对于副肿瘤LEMS和神经肌强直，潜在肿瘤的最佳治疗至关重要（良好的实践要点）。LEMS和神经肌强直的免疫抑制治疗应与MG相似（良好的做法）。
方法：为了制定治疗自身免疫性NMT疾病的共识指南，从MEDLINE检索的引用，考虑了EMBASE和Cochrane图书馆，并由疾病专家编写并同意了声明。
结论：MG的管理应首先使用抗胆碱酯酶药物，但对患有MuSK抗体的患者要谨慎（良好的做法）。建议在严重病例中进行血浆置换以诱导缓解并准备手术（建议B级）。IvIg和血浆置换可有效治疗MG加重（建议A级）。对于非胸腺瘤MG患者，推荐胸腺切除术作为增加缓解或改善概率的一种选择(推荐水平B)。一旦诊断出胸腺瘤，无论MG严重程度如何，都应进行胸腺切除术（建议A级）。当需要免疫抑制药物时，口服皮质类固醇是首选药物（良好的实践要点）。当需要长期免疫抑制时，建议硫唑嘌呤允许将类固醇逐渐减少至最低剂量，同时维持硫唑嘌呤（建议水平A）。3,4-二氨基吡啶被推荐作为对症治疗，IvIG在LEMS中具有积极的短期效果（良好的实践要点）。神经肌强直患者应使用减少周围神经兴奋过度的抗癫痫药物治疗（好的做法要点）。对于副肿瘤LEMS和神经肌强直，潜在肿瘤的最佳治疗至关重要（良好的实践要点）。LEMS和神经肌强直的免疫抑制治疗应与MG相似（良好的做法）。
