重症肌无力管理的实践模式:美国神经科医生的横断面调查

V. Bril, Jacqueline A Palace, T. Mozaffar, D. Gelinas, E. Brauer, Paul Nisbet, G. Wolfe
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引用次数: 1

摘要

背景:重症肌无力(MG)是一种罕见的免疫球蛋白G自身抗体介导的神经肌肉连接障碍,其治疗是由医生经验驱动的。为了深入了解当前的实践和医生的需求,对神经科医生使用指南和疾病活动评估来管理MG进行了评估。方法:在2020年11月和12月,采用了一项定量、横断面、51项在线调查的研究,收集了来自美国31个州的100名治疗MG的社区神经科医生的数据。比值变量间差异分析采用卡方检验和t检验,P<0.05为显著水平。结果:在受访者中,76%的人报告使用临床判断而不是指南来指导治疗决策,只有29%的人报告了解最新的2020年重症肌无力管理国际共识指南。报告的治疗模式包括使用强的松等效皮质类固醇剂量≤10mg /天,持续≥6个月(76%的应答者)。当皮质类固醇是禁忌症或初始非甾体免疫抑制剂治疗(nist)失败后,免疫球蛋白治疗是应答者对乙酰胆碱受体抗体阳性的广泛性MG患者的首选初始治疗(与第二种nist相比)。受访者表示有兴趣获得更多关于危机管理、启动/滴定维持药物和管理合并症患者的指导。结论:本次调查的应答者报告了MG管理的各种方法,在一些临床环境中,对临床判断的依赖比现有的基于共识的指导更严重。还观察到,在皮质类固醇禁忌症患者中,nist的潜在利用不足,而依赖于免疫球蛋白。
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Practice patterns in the management of myasthenia gravis: a cross-sectional survey of neurologists in the United States
Background: Management of myasthenia gravis (MG), a rare immunoglobulin G autoantibody–mediated neuromuscular junction disorder, is driven by physician experience. To gain insight into current practices and physician needs, neurologists’ use of guidelines and disease activity evaluations to manage MG was assessed. Methods: In November and December of 2020, a quantitative, cross-sectional, 51-item, online survey–based study was used to collect data from 100 community neurologists, from 31 US states, who treat MG. Differences across ratio variables were analyzed via Chi-square and t tests, at a significance level of P<0.05. Results: Of respondents, 76% reported using clinical judgment rather than guidelines to inform treatment decisions, and only 29% reported awareness of the updated 2020 International Consensus Guidance for Management of Myasthenia Gravis. Treatment patterns reported include use of prednisone-equivalent corticosteroid doses ≤10 mg/day for ≥6 months (76% of respondents). When corticosteroids are contraindicated or after failure of an initial nonsteroidal immunosuppressant therapy (NSIST), immunoglobulin therapy is the respondents’ preferred initial treatment in patients with acetylcholine receptor antibody–positive generalized MG (vs a second NSIST). Respondents expressed interest in more guidance on crisis management, initiating/titrating maintenance medications, and managing patients with comorbidities. Conclusions: Respondents to this survey reported varied approaches to MG management and, in some clinical settings, heavier reliance on clinical judgment than on available consensus-based guidance. Also observed was potential underutilization of NSISTs in patients for whom corticosteroids are contraindicated, with reliance, instead, on immunoglobulin.
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