Shahar Shelly, Marc Gotkine, Adi Wilf Yarkoni, Itay Lotan, Alon Abraham, Amir Dori, Gil I Wolfe, Keren Regev, Adi Vaknin, Tamir Ben-Hur
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引用次数: 0
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.
重症肌无力(MG)是一种慢性自身免疫性神经肌肉疾病,主要表现为影响眼球、球、躯干、肢体和呼吸肌的波动性骨骼肌无力。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(muscle specific kinase)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,治疗可及性和做法差异方面的差距仍然普遍存在。为了解决这些差距,已经制定了更新的国家指南,整合了最新的国际证据,并使其适应当地的医疗保健状况、法规和人口多样性。这个国家指南强调通过全面的临床评估、标准化的抗体测试、神经生理研究和胸腺病理评估的纵隔成像来精确诊断评估。使用标准化的量表,包括日常生活MG活动、定量MG评分和美国MG基金会干预后状态,对疾病分期和治疗决策至关重要。治疗目标优先考虑达到完全缓解或疾病表现最小的状态,治疗相关的副作用可以忽略不计。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。推荐胸腺切除术用于全身性AChR抗体阳性的MG患者,理想情况下在发病2年内。孕妇、老年人、儿童和癌症患者需要特异性免疫治疗方法。多学科护理、结构化患者教育和社会心理支持是有效管理MG的必要条件。这些国家指南旨在使临床实践标准化,提高患者预后,并减少以色列MG管理中的医疗保健差异。
期刊介绍:
Therapeutic Advances in Neurological Disorders is a peer-reviewed, open access journal delivering the highest quality articles, reviews, and scholarly comment on pioneering efforts and innovative studies across all areas of neurology. The journal has a strong clinical and pharmacological focus and is aimed at clinicians and researchers in neurology, providing a forum in print and online for publishing the highest quality articles in this area.