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自身免疫性神经肌肉传递障碍的治疗指南。

Guidelines for the treatment of autoimmune neuromuscular transmission disorders.

作者信息

Skeie G O, Apostolski S, Evoli A, Gilhus N E, Hart I K, Harms L, Hilton-Jones D, Melms A, Verschuuren J, Horge H W

机构信息

Department of Neurology, University of Bergen, Bergen, Norway.

出版信息

Eur J Neurol. 2006 Jul;13(7):691-9. doi: 10.1111/j.1468-1331.2006.01476.x.

Abstract

Important progress has been made in our understanding of the cellular and molecular processes underlying the autoimmune neuromuscular transmission (NMT) disorders; myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS) and neuromyotonia (peripheral nerve hyperexcitability; Isaacs syndrome). 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 and a patient representative. The proposed practical treatment guidelines are agreed upon by the Task Force: (i) Anticholinesterase drugs should be the first drug to be given in the management of MG (good practice point). (ii) Plasma exchange is recommended as a short-term treatment in MG, especially in severe cases to induce remission and in preparation for surgery (level B recommendation). (iii) Intravenous immunoglobulin (IvIg) and plasma exchange are equally effective for the treatment of MG exacerbations (level A Recommendation). (iv) For patients with non-thymomatous autoimmune MG, thymectomy (TE) is recommended as an option to increase the probability of remission or improvement (level B recommendation). (v) Once thymoma is diagnosed TE is indicated irrespective of the severity of MG (level A recommendation). (vi) Oral corticosteroids is a first choice drug when immunosuppressive drugs are necessary in MG (good practice point). (vii) In patients where long-term immunosuppression is necessary, azathioprine is recommended together with steroids to allow tapering the steroids to the lowest possible dose whilst maintaining azathioprine (level A recommendation). (viii) 3,4-diaminopyridine is recommended as symptomatic treatment and IvIg has a positive short-term effect in LEMS (good practice point). (ix) All neuromyotonia patients should be treated symptomatically with an anti-epileptic drug that reduces peripheral nerve hyperexcitability (good practice point). (x) Definitive management of paraneoplastic neuromyotonia and LEMS is treatment of the underlying tumour (good practice point). (xi) For immunosuppressive treatment of LEMS and NMT it is reasonable to adopt treatment procedures by analogy with MG (good practice point).

摘要

我们对自身免疫性神经肌肉传递(NMT)障碍(重症肌无力(MG)、兰伯特 - 伊顿肌无力综合征(LEMS)和神经性肌强直(周围神经兴奋性过高;艾萨克斯综合征))背后的细胞和分子过程的理解取得了重要进展。为制定自身免疫性NMT障碍的治疗共识指南。检索了MEDLINE、EMBASE和Cochrane图书馆的参考文献,并由疾病专家和一名患者代表制定并商定了相关声明。工作组就拟议的实用治疗指南达成一致:(i)抗胆碱酯酶药物应作为MG治疗的首选药物(良好实践要点)。(ii)血浆置换推荐作为MG的短期治疗方法,尤其适用于严重病例以诱导缓解和为手术做准备(B级推荐)。(iii)静脉注射免疫球蛋白(IvIg)和血浆置换在治疗MG病情加重方面同样有效(A级推荐)。(iv)对于非胸腺瘤性自身免疫性MG患者,推荐胸腺切除术(TE)作为增加缓解或改善可能性的一种选择(B级推荐)。(v)一旦诊断出胸腺瘤,无论MG的严重程度如何,均应进行TE(A级推荐)。(vi)当MG需要使用免疫抑制药物时,口服皮质类固醇是首选药物(良好实践要点)。(vii)对于需要长期免疫抑制的患者,推荐硫唑嘌呤与类固醇联合使用,以便在维持硫唑嘌呤的同时将类固醇逐渐减量至尽可能低的剂量(A级推荐)。(viii)推荐3,4 - 二氨基吡啶作为对症治疗药物,IvIg在LEMS中有积极的短期疗效(良好实践要点)。(ix)所有神经性肌强直患者均应使用降低周围神经兴奋性的抗癫痫药物进行对症治疗(良好实践要点)。(x)副肿瘤性神经性肌强直和LEMS的确定性治疗是治疗潜在肿瘤(良好实践要点)。(xi)对于LEMS和NMT的免疫抑制治疗,采用与MG类似的治疗程序是合理的(良好实践要点)。

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