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Clinical and laboratory remission with rituximab in anti-MuSK-positive myasthenia gravis

Background Increasing data are available on the use and efficacy of rituximab (RTX) in patients with anti-muscle-specific tyrosine kinase (MuSK)-positive myasthenia gravis (MG), especially those steroid-dependent or unresponsive to traditional immunotherapies. Aims We aimed to evaluate the clinical...

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Bibliographic Details
Published in:Irish journal of medical science 2024-12, Vol.193 (6), p.2989-2994
Main Authors: Inan, Berin, Orhan, Irem Gul, Bekircan-Kurt, Can Ebru, Erdem-Ozdamar, Sevim, Tan, Ersin
Format: Article
Language:English
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Summary:Background Increasing data are available on the use and efficacy of rituximab (RTX) in patients with anti-muscle-specific tyrosine kinase (MuSK)-positive myasthenia gravis (MG), especially those steroid-dependent or unresponsive to traditional immunotherapies. Aims We aimed to evaluate the clinical characteristics and treatment responses of adult patients with generalized anti-MuSK-positive MG treated with RTX. Methods We retrospectively recruited 16 patients who were on RTX, between January 2010 and September 2023. RTX was given 1000 mg/day intravenously twice, two weeks apart. Maintenance treatment was administered at intervals of 3—6 months based on clinical evaluation. The outcome was assessed by Myasthenia Gravis Foundation of America (MGFA) and Myasthenia Gravis Status and Treatment Intensity (MGSTI) scores. Additionally, anti-MuSK antibody levels were retested after treatment in all patients except one. Results Twelve patients were female. The mean age at disease onset was 35.3 ± 17.3 years. The median duration between disease onset and RTX administration was 2.4 years (min-max: 0.5-36.5 years). The worst MGFA class before RTX was between IIIb-V. After RTX treatment, 81.3% of patients achieved MGFA minimal manifestations or better and MGSTI level 1 or better. Anti-MuSK antibodies became negative in 12 patients, while they remained positive in three. The changes in antibody levels seemed associated with clinical outcomes. Conclusions RTX is an effective treatment in anti-MuSK-positive MG. Furthermore, our results support the inhibition of antibody production by RTX and we recommend monitoring anti-MuSK antibody titers to follow disease progression and treatment response.
ISSN:0021-1265
1863-4362
1863-4362
DOI:10.1007/s11845-024-03763-w