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Myasthenia gravis.
Nature Reviews Disease Primers ( IF 76.9 ) Pub Date : 2019-05-02 , DOI: 10.1038/s41572-019-0079-y Nils Erik Gilhus 1, 2 , Socrates Tzartos 3 , Amelia Evoli 4, 5 , Jacqueline Palace 6 , Ted M Burns 7 , Jan J G M Verschuuren 8
Nature Reviews Disease Primers ( IF 76.9 ) Pub Date : 2019-05-02 , DOI: 10.1038/s41572-019-0079-y Nils Erik Gilhus 1, 2 , Socrates Tzartos 3 , Amelia Evoli 4, 5 , Jacqueline Palace 6 , Ted M Burns 7 , Jan J G M Verschuuren 8
Affiliation
Myasthenia gravis (MG) is an autoimmune disease caused by antibodies against the acetylcholine receptor (AChR), muscle-specific kinase (MuSK) or other AChR-related proteins in the postsynaptic muscle membrane. Localized or general muscle weakness is the predominant symptom and is induced by the antibodies. Patients are grouped according to the presence of antibodies, symptoms, age at onset and thymus pathology. Diagnosis is straightforward in most patients with typical symptoms and a positive antibody test, although a detailed clinical and neurophysiological examination is important in antibody-negative patients. MG therapy should be ambitious and aim for clinical remission or only mild symptoms with near-normal function and quality of life. Treatment should be based on MG subgroup and includes symptomatic treatment using acetylcholinesterase inhibitors, thymectomy and immunotherapy. Intravenous immunoglobulin and plasma exchange are fast-acting treatments used for disease exacerbations, and intensive care is necessary during exacerbations with respiratory failure. Comorbidity is frequent, particularly in elderly patients. Active physical training should be encouraged.
中文翻译:
重症肌无力。
重症肌无力(MG)是一种由突触后肌膜中的针对乙酰胆碱受体(AChR),肌肉特异性激酶(MuSK)或其他AChR相关蛋白的抗体引起的自身免疫性疾病。局部或全身性肌肉无力是主要症状,由抗体诱发。根据抗体的存在,症状,发病年龄和胸腺病理将患者分组。尽管对抗体阴性的患者进行详细的临床和神经生理学检查很重要,但大多数具有典型症状且抗体检测阳性的患者的诊断很简单。MG疗法应雄心勃勃,其目标是临床缓解或仅具有功能和生活质量接近正常的轻度症状。治疗应基于MG亚组,包括使用乙酰胆碱酯酶抑制剂的对症治疗,胸腺切除术和免疫疗法。静脉内免疫球蛋白和血浆置换是用于疾病加重的速效治疗,在因呼吸衰竭加重的过程中,需要加护病房。合并症很常见,尤其是在老年患者中。应鼓励积极的体育锻炼。
更新日期:2019-11-18
中文翻译:
重症肌无力。
重症肌无力(MG)是一种由突触后肌膜中的针对乙酰胆碱受体(AChR),肌肉特异性激酶(MuSK)或其他AChR相关蛋白的抗体引起的自身免疫性疾病。局部或全身性肌肉无力是主要症状,由抗体诱发。根据抗体的存在,症状,发病年龄和胸腺病理将患者分组。尽管对抗体阴性的患者进行详细的临床和神经生理学检查很重要,但大多数具有典型症状且抗体检测阳性的患者的诊断很简单。MG疗法应雄心勃勃,其目标是临床缓解或仅具有功能和生活质量接近正常的轻度症状。治疗应基于MG亚组,包括使用乙酰胆碱酯酶抑制剂的对症治疗,胸腺切除术和免疫疗法。静脉内免疫球蛋白和血浆置换是用于疾病加重的速效治疗,在因呼吸衰竭加重的过程中,需要加护病房。合并症很常见,尤其是在老年患者中。应鼓励积极的体育锻炼。