PMID- 35775051
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20230916
IS  - 1664-2295 (Print)
IS  - 1664-2295 (Electronic)
IS  - 1664-2295 (Linking)
VI  - 13
DP  - 2022
TI  - Long-Term Efficacy of Non-steroid Immunosuppressive Agents in 
      Anti-Muscle-Specific Kinase Positive Myasthenia Gravis Patients: A Prospective 
      Study.
PG  - 877895
LID - 10.3389/fneur.2022.877895 [doi]
LID - 877895
AB  - 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 chi(2) = 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.
CI  - Copyright (c) 2022 Tan, Shi, Huang, Li, Yan, Zhu, Guan and Cui.
FAU - Tan, Ying
AU  - Tan Y
AD  - Department of Neurology, Peking Union Medical College Hospital, Chinese Academy 
      of Medical Sciences, Beijing, China.
FAU - Shi, Jiayu
AU  - Shi J
AD  - Department of Neurology, Peking Union Medical College Hospital, Chinese Academy 
      of Medical Sciences, Beijing, China.
FAU - Huang, Yangyu
AU  - Huang Y
AD  - Department of Neurology, Peking Union Medical College Hospital, Chinese Academy 
      of Medical Sciences, Beijing, China.
FAU - Li, Ke
AU  - Li K
AD  - Department of Neurology, Peking Union Medical College Hospital, Chinese Academy 
      of Medical Sciences, Beijing, China.
FAU - Yan, Jingwen
AU  - Yan J
AD  - Department of Neurology, Peking Union Medical College Hospital, Chinese Academy 
      of Medical Sciences, Beijing, China.
FAU - Zhu, Li
AU  - Zhu L
AD  - Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese 
      Academy of Medical Science, Beijing, China.
FAU - Guan, Yuzhou
AU  - Guan Y
AD  - Department of Neurology, Peking Union Medical College Hospital, Chinese Academy 
      of Medical Sciences, Beijing, China.
FAU - Cui, Liying
AU  - Cui L
AD  - Department of Neurology, Peking Union Medical College Hospital, Chinese Academy 
      of Medical Sciences, Beijing, China.
LA  - eng
PT  - Journal Article
DEP - 20220613
PL  - Switzerland
TA  - Front Neurol
JT  - Frontiers in neurology
JID - 101546899
PMC - PMC9237788
OTO - NOTNLM
OT  - anti-AChR antibody
OT  - anti-MuSK antibody
OT  - autoimmune
OT  - minimal manifestation status
OT  - myasthenia gravis
COIS- The authors declare that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2022/07/02 06:00
MHDA- 2022/07/02 06:01
PMCR- 2022/06/13
CRDT- 2022/07/01 02:48
PHST- 2022/02/17 00:00 [received]
PHST- 2022/04/25 00:00 [accepted]
PHST- 2022/07/01 02:48 [entrez]
PHST- 2022/07/02 06:00 [pubmed]
PHST- 2022/07/02 06:01 [medline]
PHST- 2022/06/13 00:00 [pmc-release]
AID - 10.3389/fneur.2022.877895 [doi]
PST - epublish
SO  - Front Neurol. 2022 Jun 13;13:877895. doi: 10.3389/fneur.2022.877895. eCollection 
      2022.