Mesh : Autoimmune Diseases / therapy Humans Lambert-Eaton Myasthenic Syndrome / immunology therapy Myasthenia Gravis / immunology therapy Neuromuscular Junction Diseases / immunology therapy

来  源:   DOI:10.1016/S1474-4422(21)00463-4

Abstract:
Myasthenia gravis and Lambert-Eaton myasthenic syndrome are antibody-mediated autoimmune diseases of the neuromuscular junction that usually present with weakness in ocular muscles and in proximal muscles of the limb and trunk. Prognosis regarding muscle strength, functional abilities, quality of life, and survival is generally good. However, some patients do not respond to treatment. Symptomatic drugs, corticosteroids, and steroid-sparing immunosuppressive drugs remain the cornerstone of treatment. In the past few years, new biological agents against complement, the FcRn receptor, or B-cell antigens have been tested in clinical trials. These new therapies extend the possibilities for targeted immunotherapies and promise exciting new options with a relatively rapid mode of action. Challenges in their use might occur, with barriers due to an increase in cost of care and additional considerations in the choice of drugs, and potential consequences of infection and vaccination due to the COVID-19 pandemic.
摘要:
重症肌无力和Lambert-Eaton肌无力综合征是抗体介导的神经肌肉接头自身免疫性疾病,通常表现为眼部肌肉以及肢体和躯干的近端肌肉无力。关于肌肉力量的预后,功能能力,生活质量,而且生存总体上是好的。然而,有些患者对治疗没有反应。对症药物,皮质类固醇,保留类固醇的免疫抑制药物仍然是治疗的基石。在过去的几年里,新的生物制剂抗补体,FcRn受体,或B细胞抗原已经在临床试验中进行了测试。这些新疗法扩展了靶向免疫疗法的可能性,并有望以相对快速的作用方式提供令人兴奋的新选择。它们的使用可能会出现挑战,由于护理成本增加和药物选择方面的额外考虑,以及COVID-19大流行引起的感染和疫苗接种的潜在后果。
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