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Abstract Details

Long-Term Efficacy of Non-steroid Immunosuppressive Agents in Anti-Muscle-Specific Kinase Positive Myasthenia Gravis Patients: A Prospective Study
Neuromuscular and Clinical Neurophysiology (EMG)
P2 - Poster Session 2 (11:45 AM-12:45 PM)
10-005
This study aimed to evaluate the efficacy of oral immunosuppressants (IS) in preventing relapse in anti-muscle-specific kinase (MuSK) positive myasthenia  gravis (MG).
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.
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.
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 χ2 = 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.
This study provides evidence that oral non-steroid immunosuppressive agents may be beneficial in reducing relapse in patients with MuSK-MG.
Authors/Disclosures
Ying Tan, MD
PRESENTER
Dr. Tan has nothing to disclose.
No disclosure on file
No disclosure on file
Ke Li, MD (University of Oxford) Dr. Li has nothing to disclose.
No disclosure on file
Yuzhou Guan No disclosure on file
Liying Cui, PhD (Peking Union Medical College Hospital) No disclosure on file