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以色列重症肌无力诊断、治疗及管理国家指南。

National guidelines for diagnosis, treatment, and management of myasthenia gravis in Israel.

作者信息

Shelly Shahar, Gotkine Marc, Wilf Yarkoni Adi, Lotan Itay, Abraham Alon, Dori Amir, Wolfe Gil I, Regev Keren, Vaknin Adi, Ben-Hur Tamir

机构信息

Ruth and Bruce Rappaport Faculty of Medicine, Technion, Israel Institute of Technology, Haifa 3525408, Israel.

Department of Neurology, Rambam Medical Center, Haifa, Israel.

出版信息

Ther Adv Neurol Disord. 2025 Aug 3;18:17562864251361607. doi: 10.1177/17562864251361607. eCollection 2025.

Abstract

Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disorder characterized primarily by fluctuating skeletal muscle weakness affecting ocular, bulbar, truncal, limb, and respiratory muscles. The disease is typically mediated by anti-acetylcholine receptor (AChR) antibodies, and less commonly by anti-muscle-specific kinase) or anti-low-density lipoprotein receptor-related protein 4 antibodies. Despite significant advancements in diagnostics and immunotherapy, disparities in treatment access and practice variability remain prevalent in Israel. To address these gaps, updated national guidelines have been developed, integrating the latest international evidence and adapting it to the local healthcare landscape, regulation, and population diversity. This national guideline emphasizes precise diagnostic evaluation through comprehensive clinical assessment, standardized antibody testing, neurophysiological studies, and mediastinal imaging for thymic pathology assessment. Utilizing standardized scales, including MG activities of daily living, quantitative MG score, and MG Foundation of America post-intervention status, is crucial for disease staging and therapeutic decision-making. Therapeutic goals prioritize achieving full remission or a state of minimal manifestations of disease with negligible treatment-related side effects. Guidelines for treatment strategies are based on antibody status, disease severity, patient age, and comorbidities. Thymectomy is recommended for patients with generalized AChR antibody-positive MG, ideally within 2 years of disease onset. Pregnant women, older adults, children, and patients with cancer need specific immunotherapy approaches. Multidisciplinary care, structured patient education, and psychosocial support are integral to managing MG effectively. These national guidelines aim to standardize clinical practices, enhance patient outcomes, and reduce healthcare disparities in the management of MG across Israel.

摘要

重症肌无力(MG)是一种慢性自身免疫性神经肌肉疾病,主要特征为波动性骨骼肌无力,累及眼肌、延髓肌、躯干肌、肢体肌和呼吸肌。该疾病通常由抗乙酰胆碱受体(AChR)抗体介导,较少由抗肌肉特异性激酶或抗低密度脂蛋白受体相关蛋白4抗体介导。尽管在诊断和免疫治疗方面取得了重大进展,但在以色列,治疗可及性差异和实践变异性仍然普遍存在。为弥补这些差距,已制定了更新后的国家指南,整合了最新的国际证据并使其适应当地的医疗环境、法规和人群多样性。该国家指南强调通过全面的临床评估、标准化抗体检测、神经生理学研究以及用于胸腺病理评估的纵隔成像进行精确的诊断评估。使用标准化量表,包括MG日常生活活动量表、定量MG评分和美国重症肌无力基金会干预后状态量表,对于疾病分期和治疗决策至关重要。治疗目标优先考虑实现完全缓解或疾病表现最小化且治疗相关副作用可忽略不计的状态。治疗策略指南基于抗体状态、疾病严重程度、患者年龄和合并症。对于全身性AChR抗体阳性的MG患者,建议进行胸腺切除术,理想情况下在疾病发作后2年内进行。孕妇、老年人、儿童和癌症患者需要特定的免疫治疗方法。多学科护理、结构化患者教育和心理社会支持是有效管理MG的重要组成部分。这些国家指南旨在规范临床实践,改善患者预后,并减少以色列全国范围内MG管理中的医疗差异。

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本文引用的文献

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Myasthenia Gravis - Redemption for B-Cell Depletion.重症肌无力——B细胞耗竭的救赎。
N Engl J Med. 2025 Jun 19;392(23):2382-2384. doi: 10.1056/NEJMe2505722.
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A Phase 3 Trial of Inebilizumab in Generalized Myasthenia Gravis.依奈利珠单抗治疗全身型重症肌无力的3期试验。
N Engl J Med. 2025 Jun 19;392(23):2309-2320. doi: 10.1056/NEJMoa2501561. Epub 2025 Apr 8.
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Survival and autoimmune risks post-thymectomy.胸腺切除术后的生存和自身免疫风险。
Front Immunol. 2025 Mar 13;16:1504496. doi: 10.3389/fimmu.2025.1504496. eCollection 2025.
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Terminal Complement Inhibitor Ravulizumab in Generalized Myasthenia Gravis.瑞昔珠单抗治疗全身性重症肌无力。
NEJM Evid. 2022 May;1(5):EVIDoa2100066. doi: 10.1056/EVIDoa2100066. Epub 2022 Apr 26.
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Guideline for the management of myasthenic syndromes.肌无力综合征管理指南。
Ther Adv Neurol Disord. 2023 Dec 26;16:17562864231213240. doi: 10.1177/17562864231213240. eCollection 2023.

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