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Long-term efficacy of non-steroid immunosuppressive agents in anti-muscle-specific kinase positive myasthenia gravis patients: a prospective study

Abstract:

Background and Purpose: 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 study aimed to evaluate the efficacy of oral immunosuppressants (IS) in preventing relapse in MuSK-MG.

Methods: 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-) non-steroid 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.

Results: 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.

Conclusions: This study provides evidence that oral non-steroid immunosuppressive agents may be beneficial in reducing relapse in patients with MuSK-MG.

Publication status:
Published
Peer review status:
Peer reviewed

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Publisher copy:
10.3389/fneur.2022.877895

Authors


More by this author
Institution:
University of Oxford
Division:
MSD
Department:
NDM
Oxford college:
Balliol College
Role:
Author
ORCID:
0000-0002-7707-3158


Publisher:
Frontiers Media
Journal:
Frontiers in Neurology More from this journal
Volume:
13
Article number:
877895
Place of publication:
Switzerland
Publication date:
2022-06-13
Acceptance date:
2022-04-25
DOI:
EISSN:
1664-2295
Pmid:
35775051


Language:
English
Keywords:
Pubs id:
1551742
Local pid:
pubs:1551742
Deposit date:
2023-10-21

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