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利妥昔单抗治疗难治性重症肌无力及其在抗肌肉特异性激酶(MuSK)阳性和抗乙酰胆碱受体(AChR)阳性重症肌无力中疗效的变异性

Rituximab in the Management of Refractory Myasthenia Gravis and Variability of Its Efficacy in Anti-MuSK Positive and Anti-AChR Positive Myasthenia Gravis.

作者信息

Bastakoti Sanjiv, Kunwar Saru, Poudel Sujan, Quinonez Jonathan, Bista Seema, Singh Navpreet, Jha Vivek, Ruxmohan Samir, Paesani Sylvia, Cueva Wilson, Michel Jack

机构信息

Division of Research & Acadamic Affairs, Larkin Community Hospital, South Miami, USA.

Internal Medicine, KIST Medical College & Teaching Hospital, Kathmandu, NPL.

出版信息

Cureus. 2021 Nov 9;13(11):e19416. doi: 10.7759/cureus.19416. eCollection 2021 Nov.

Abstract

Myasthenia gravis affects the neuromuscular junction of the skeletal muscles. It results in muscle weakness involving skeletal muscles (diaphragm, extraocular muscles) and myasthenic crisis. Treatment options for myasthenia gravis management have expanded, including azathioprine, corticosteroids, plasma exchange, and tacrolimus. Unfortunately, a few cases of myasthenia gravis don't respond to conventional treatment modalities. Monoclonal antibodies, rituximab (RTX), are novel treatments that have garnered interest as of late due to their efficacy within the patient population presented with refractory form myasthenia gravis. This review aims to showcase how RTX is an effective treatment within different forms of myasthenia gravis. A limited review was performed using databases that include PubMed and Google Scholar. The following keywords were used: "myasthenia gravis," "rituximab," "monoclonal antibody," "anti-AChR antibody," and "refractory myasthenia." The review focused on case reports, human studies, or research surveys based on the inclusion criteria of human studies involving participants more than 18 years of age and published in English literature. Out of 69 articles, 14 were duplicates, and 29 were relevant and met the inclusion criteria. The findings from the study demonstrate that patients with refractory myasthenia gravis responded well to RTX treatment. Furthermore, RTX has been shown to decrease corticosteroid dependence, induce sustained remission, and have a favorable response to anti-MuSK antibody positive myasthenia gravis compared to anti-AChR antibody positive myasthenia gravis. This literature review suggests that patients with refractory myasthenia gravis can benefit from rituximab; however, it has a variable response in different forms of myasthenia gravis.

摘要

重症肌无力会影响骨骼肌的神经肌肉接头。它会导致涉及骨骼肌(膈肌、眼外肌)的肌肉无力以及重症肌无力危象。重症肌无力的治疗选择有所增加,包括硫唑嘌呤、皮质类固醇、血浆置换和他克莫司。不幸的是,少数重症肌无力病例对传统治疗方式没有反应。单克隆抗体利妥昔单抗(RTX)是一种新型治疗方法,由于其在难治性重症肌无力患者群体中的疗效,最近引起了人们的关注。这篇综述旨在展示RTX如何在不同形式的重症肌无力中作为一种有效的治疗方法。使用包括PubMed和谷歌学术在内的数据库进行了有限的综述。使用了以下关键词:“重症肌无力”、“利妥昔单抗”、“单克隆抗体”、“抗AChR抗体”和“难治性重症肌无力”。该综述聚焦于基于涉及18岁以上参与者的人体研究纳入标准且发表在英文文献中的病例报告、人体研究或研究调查。在69篇文章中,14篇是重复的,29篇相关且符合纳入标准。研究结果表明,难治性重症肌无力患者对RTX治疗反应良好。此外,与抗AChR抗体阳性的重症肌无力相比,RTX已被证明可降低对皮质类固醇的依赖性、诱导持续缓解,并且对抗MuSK抗体阳性的重症肌无力有良好反应。这篇文献综述表明,难治性重症肌无力患者可从利妥昔单抗中获益;然而,它在不同形式的重症肌无力中的反应存在差异。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/764a/8660595/9934c9833fe8/cureus-0013-00000019416-i01.jpg

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