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
{"title":"Ravulizumab对全身性重症肌无力患者预后和生活质量的影响。","authors":"Carlo Antozzi, Renato Mantegazza","doi":"10.2147/PROM.S408175","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":19747,"journal":{"name":"Patient Related Outcome Measures","volume":"14 ","pages":"305-312"},"PeriodicalIF":1.8000,"publicationDate":"2023-10-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/db/f9/prom-14-305.PMC10590807.pdf","citationCount":"0","resultStr":"{\"title\":\"Impact of Ravulizumab on Patient Outcomes and Quality of Life in Generalized Myasthenia Gravis.\",\"authors\":\"Carlo Antozzi, Renato Mantegazza\",\"doi\":\"10.2147/PROM.S408175\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":19747,\"journal\":{\"name\":\"Patient Related Outcome Measures\",\"volume\":\"14 \",\"pages\":\"305-312\"},\"PeriodicalIF\":1.8000,\"publicationDate\":\"2023-10-18\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/db/f9/prom-14-305.PMC10590807.pdf\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Patient Related Outcome Measures\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.2147/PROM.S408175\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2023/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q3\",\"JCRName\":\"HEALTH CARE SCIENCES & SERVICES\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Patient Related Outcome Measures","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.2147/PROM.S408175","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2023/1/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"HEALTH CARE SCIENCES & SERVICES","Score":null,"Total":0}
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。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
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
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信