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重症肌无力患者管理的实用方法——观点及文献综述

A Practical Approach to Managing Patients With Myasthenia Gravis-Opinions and a Review of the Literature.

作者信息

Farrugia Maria Elena, Goodfellow John A

机构信息

Neurology Department, Institute of Neurological Sciences, Queen Elizabeth University Hospital, Glasgow, United Kingdom.

Neuroimmunology Laboratory, Laboratory Medicine and Facilities Building, Queen Elizabeth University Hospital, Glasgow, United Kingdom.

出版信息

Front Neurol. 2020 Jul 7;11:604. doi: 10.3389/fneur.2020.00604. eCollection 2020.

Abstract

When the diagnosis of myasthenia gravis (MG) has been secured, the aim of management should be prompt symptom control and the induction of remission or minimal manifestations. Symptom control, with acetylcholinesterase inhibitors such as pyridostigmine, is commonly employed. This may be sufficient in mild disease. There is no single universally accepted treatment regimen. Corticosteroids are the mainstay of immunosuppressive treatment in patients with more than mild MG to induce remission. Immunosuppressive therapies, such as azathioprine are prescribed in addition to but sometimes instead of corticosteroids when background comorbidities preclude or restrict the use of steroids. Rituximab has a role in refractory MG, while plasmapheresis and immunoglobulin therapy are commonly prescribed to treat MG crisis and in some cases of refractory MG. Data from the MGTX trial showed clear evidence that thymectomy is beneficial in patients with acetylcholine receptor (AChR) antibody positive generalized MG, up to the age of 65 years. Minimally invasive thymectomy surgery including robotic-assisted thymectomy surgery has further revolutionized thymectomy and the management of MG. Ocular MG is not life-threatening but can be significantly disabling when diplopia is persistent. There is evidence to support early treatment with corticosteroids when ocular motility is abnormal and fails to respond to symptomatic treatment. Treatment needs to be individualized in the older age-group depending on specific comorbidities. In the younger age-groups, particularly in women, consideration must be given to the potential teratogenicity of certain therapies. Novel therapies are being developed and trialed, including ones that inhibit complement-induced immunological pathways or interfere with antibody-recycling pathways. Fatigue is common in MG and should be duly identified from fatigable weakness and managed with a combination of physical therapy with or without psychological support. MG patients may also develop dysfunctional breathing and the necessary respiratory physiotherapy techniques need to be implemented to alleviate the patient's symptoms of dyspnoea. In this review, we discuss various facets of myasthenia management in adults with ocular and generalized disease, including some practical approaches and our personal opinions based on our experience.

摘要

当重症肌无力(MG)的诊断确立后,治疗的目标应是迅速控制症状并诱导缓解或使症状表现最小化。通常使用乙酰胆碱酯酶抑制剂如吡啶斯的明来控制症状。对于轻症疾病,这可能就足够了。目前尚无一种被普遍接受的单一治疗方案。皮质类固醇是治疗中度以上MG患者以诱导缓解的免疫抑制治疗的主要手段。当存在基础合并症妨碍或限制使用类固醇时,除皮质类固醇外,还会加用硫唑嘌呤等免疫抑制疗法,有时也会用其替代皮质类固醇。利妥昔单抗在难治性MG中发挥作用,而血浆置换和免疫球蛋白疗法常用于治疗MG危象以及某些难治性MG病例。MGTX试验的数据表明,有明确证据显示胸腺切除术对65岁以下乙酰胆碱受体(AChR)抗体阳性的全身型MG患者有益。包括机器人辅助胸腺切除术在内的微创胸腺切除术进一步革新了胸腺切除术及MG的治疗。眼肌型MG不会危及生命,但当复视持续存在时会导致严重功能障碍。有证据支持当眼球运动异常且对对症治疗无反应时,早期使用皮质类固醇进行治疗。在老年人群中,治疗需要根据具体合并症进行个体化调整。在年轻人群中,尤其是女性,必须考虑某些疗法的潜在致畸性。正在研发和试验新的疗法,包括抑制补体诱导的免疫途径或干扰抗体循环途径的疗法。疲劳在MG中很常见,应从易疲劳性肌无力中正确识别出来,并通过结合物理治疗(有无心理支持)进行管理。MG患者还可能出现呼吸功能障碍,需要实施必要的呼吸物理治疗技术以缓解患者的呼吸困难症状。在本综述中,我们讨论了成人眼肌型和全身型MG治疗的各个方面,包括一些实用方法以及基于我们经验的个人观点。

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