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克服重症肌无力诊断和治疗中的挑战。

Overcoming challenges in the diagnosis and treatment of myasthenia gravis.

作者信息

Evoli Amelia, Iorio Raffaele, Bartoccioni Emanuela

机构信息

a Institute of Neurology , Catholic University , Roma , Italy.

b Don Gnocchi ONLUS Foundation , Milan , Italy.

出版信息

Expert Rev Clin Immunol. 2016;12(2):157-68. doi: 10.1586/1744666X.2016.1110487. Epub 2015 Dec 16.

DOI:10.1586/1744666X.2016.1110487
PMID:26675896
Abstract

In recent years, the discovery of new autoantigens and the use of sensitive assays have expanded the clinical spectrum of myasthenia gravis (MG). In particular, antibodies binding to clustered acetylcholine receptors and to the low-density lipoprotein receptor-related protein 4 have not only bridged a significant gap in diagnosis but also have relevant clinical implications. MG management includes different therapeutic options, from symptomatic agents as the only therapy in mildly affected cases to combined long-term immunosuppression and thymectomy in patients with severe disabling disease. MG biological diversity can influence the response to therapies and should be taken into account when planning treatment. Biologic agents are promising, though their use is currently limited to patients with refractory disease.

摘要

近年来,新自身抗原的发现以及敏感检测方法的应用拓宽了重症肌无力(MG)的临床谱。特别是,与聚集型乙酰胆碱受体和低密度脂蛋白受体相关蛋白4结合的抗体不仅填补了诊断上的重大空白,还具有相关的临床意义。MG的治疗包括不同的选择,从轻度受累病例仅使用对症药物治疗到重度致残患者采用长期免疫抑制联合胸腺切除术。MG的生物学多样性会影响治疗反应,在制定治疗方案时应予以考虑。生物制剂前景广阔,不过目前仅限于用于难治性疾病患者。

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