Abstract
<h3>Objective:</h3> This study aimed to evaluate the efficacy of oral immunosuppressants (IS) in preventing relapse in anti-muscle-specific kinase (MuSK) positive myasthenia gravis (MG). <h3>Background:</h3> Anti-muscle-specific kinase (MuSK) positive myasthenia gravis (MG) is characterized by a high relapsing rate, thus, choosing the appropriate oral drug regimen is a challenge. <h3>Design/Methods:</h3> This prospective cohort observational study included patients with MuSK-MG at Peking Union Medical College Hospital between January 1, 2018, and November 15,2021. The patients were divided into 2 groups: those with (IS+) or without (IS−) nonsteroid immunosuppressive agents. The primary outcome was relapsed at follow-up, and the log-rank test was used to compare the proportion of maintenance-free relapse between the groups; hazard ratio (HR) was calculated using the Cox proportional hazards models. <h3>Results:</h3> Fifty-three of 59 patients with MuSK-MG were included in the cohort, 14 were in the IS+ group, and 39 were in the IS− group. Twenty-four cases in the cohort experienced relapse at least once; the relapse rate was 2/14 (14.3%) in the IS+ group and 22/39 (56.4%) in the IS− group. At the end of follow-up, the proportion of maintenance-free relapse was significantly different between the two groups (log-rank χ<sup>2</sup> = 4.94, P = 0.02).Of all the potential confounders, only the use of IS was associated with a reduced risk of relapse. The HR for relapse among patients in the IS+ group was 0.21 (95%CI 0.05–0.58) and was 0.23 (95%CI 0.05–0.93) in a model adjusted for age, sex, relapse history, highest Myasthenia Gravis Foundation of America (MGFA), and accumulated time of steroid therapy. <h3>Conclusions:</h3> This study provides evidence that oral non-steroid immunosuppressive agents may be beneficial in reducing relapse in patients with MuSK-MG. <b>Disclosure:</b> Dr. Tan has nothing to disclose. Jiayu Shi has nothing to disclose. Dr. Huang has nothing to disclose. Dr. Li has nothing to disclose. Jingwen Yan has nothing to disclose. Yuzhou Guan has nothing to disclose. Liying Cui has nothing to disclose.
Published Version
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