Ravulizumab对全身性重症肌无力患者预后和生活质量的影响。

IF 1.8 Q3 HEALTH CARE SCIENCES & SERVICES Patient Related Outcome Measures Pub Date : 2023-10-18 eCollection Date: 2023-01-01 DOI:10.2147/PROM.S408175
Carlo Antozzi, Renato Mantegazza
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引用次数: 0

摘要

重症肌无力(MG)是一种自身免疫性离子通道疾病,针对不同终板抗原的抗体会损害神经肌肉传递,最终导致肌肉无力和疲劳。在大约85%的MG患者中,抗乙酰胆碱受体(AChR)的自身抗体激活补体级联,对神经肌肉接头造成损伤。MG是一种慢性疾病,皮质类固醇、免疫抑制药物的标准治疗以及血浆交换或静脉注射免疫球蛋白的免疫调节可以改变疾病的进程,但身体、心理和社会残疾的残余负担突出了一些未满足的需求,其中包括对特定、靶向、,以及能够改善患者生活质量的耐受性良好的治疗。补体抑制为MG的精准治疗铺平了道路,因为首次设计、测试了一种针对关键致病步骤的特异性疗法,并证明其以可控的方式有效。Ravulizumab是首个被批准用于治疗全身性MG患者的长效补体抑制剂,能够提供快速、完全和持续的补体抑制。如CHAMPION MG试验所示,Ravulizumab在26周内改善了MG日常生活活动量表和其他临床参数,并通过其开放标签扩展,每8周给药一次,增加了价值。给药时间表可能会提高患者的依从性,从而提高他们的生活质量。补体抑制的引入将极大地改变MG的传统治疗策略。
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Impact of Ravulizumab on Patient Outcomes and Quality of Life in Generalized Myasthenia Gravis.

Myasthenia gravis (MG) is an autoimmune ion channel disorder in which antibodies to different end-plate antigens impair neuromuscular transmission, ultimately leading to muscle weakness and fatigability. In about 85% of patients with MG, autoantibodies against the acetylcholine receptor (AChR) activate the complement cascade, causing damage to the neuromuscular junction. MG is a chronic disorder for which standard therapies with corticosteroids, immunosuppressive drugs, and immunomodulation with plasma exchange or intravenous immunoglobulins modify the course of the disease, but the residual burden of physical, psychological, and social disability highlights several unmet needs, among these the need for specific, targeted, and well tolerated therapies able to improve the patients' quality of life. Complement inhibition paved the way to precision medicine in MG since, for the first time, a specific therapy targeting a crucial pathogenetic step has been designed, tested, and proven to be effective in a controlled fashion. Ravulizumab represents the first long-acting complement inhibitor approved for treatment of patients with generalized MG, able to provide rapid, complete, and sustained complement inhibition. Ravulizumab improved the MG Activity of Daily Living scale and other clinical parameters up to 26 weeks as shown by the CHAMPION MG trial, and by its open label extension, with the added value of being administered every 8 weeks. The schedule of administration is likely to improve patients' adherence and hence their quality of life. The introduction of complement inhibition will considerably change the traditional therapeutic strategy for MG.

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来源期刊
Patient Related Outcome Measures
Patient Related Outcome Measures HEALTH CARE SCIENCES & SERVICES-
自引率
4.80%
发文量
27
审稿时长
16 weeks
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