PMID- 33552238
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220420
IS  - 1756-2856 (Print)
IS  - 1756-2864 (Electronic)
IS  - 1756-2856 (Linking)
VI  - 14
DP  - 2021
TI  - VNTR2/VNTR3 genotype in the FCGRT gene is associated with reduced effectiveness 
      of intravenous immunoglobulin in patients with myasthenia gravis.
PG  - 1756286420986747
LID - 10.1177/1756286420986747 [doi]
LID - 1756286420986747
AB  - BACKGROUND: Intravenous immunoglobulin (IVIG) has been commonly used to treat 
      myasthenia gravis exacerbation, but is still ineffective in nearly 30% of 
      patients. A variable number of tandem repeat (VNTR) polymorphism in the FCGRT 
      gene has been found to reduce the efficiency of IgG biologics. However, whether 
      the polymorphism influences the efficacy of IVIG in generalized myasthenia gravis 
      (MG) patients with exacerbations remains unknown. METHODS: The distribution of 
      VNTR genotypes was analyzed in 334 patients with MG. Varied VNTR alleles were 
      determined by capillary electrophoresis and confirmed by Sanger sequencing. 
      Information of endogenous IgG levels were collected in patients without previous 
      immunotherapy (n = 26). Medical records of patients who received IVIG therapy 
      were retrospectively analyzed for therapeutic outcomes of IVIG treatment 
      (n = 61). Patients whose Activities of Daily Living scores decreased by 2 or more 
      points on day 14 were considered responders to the treatment. RESULTS: The 
      VNTR3/3 and VNTR2/3 genotypes were detected in 96.7% (323/334) and 3.4% (11/334) 
      patients, respectively. Patients with VNTR2/3 heterozygosity had lower endogenous 
      IgG levels than those with VNTR3/3 homozygosity (9.81 +/- 2.61 g/L versus 
      12.41 +/- 2.45g/L, p = 0.016). The response rate of IVIG therapy was 78.7% (48/61). 
      All responders and nine non-responders were VNTR3/3 homozygotes, whereas all the 
      patients with VNTR2/3 genotypes were non-responders (n = 4). In patients who took 
      IVIG treatments, endogenous IgG levels were significantly lower in non-responders 
      compared with responders (12.93 +/- 2.24 g/L versus 8.85 +/- 2.69 g/L, p = 0.006), 
      especially in VNTR2/3 heterozygotes (7.86 +/- 1.78 g/L, p = 0.001). CONCLUSION: The 
      VNTR2/3 genotype could influence endogenous IgG levels and serve as a predictive 
      marker for poor responses to IVIG in MG patients.
CI  - (c) The Author(s), 2021.
FAU - Su, Shengyao
AU  - Su S
AUID- ORCID: 0000-0002-5020-7056
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 
      China.
FAU - Liu, Qing
AU  - Liu Q
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Chang Chun 
      Street, Beijing, China.
FAU - Zhang, Xueping
AU  - Zhang X
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Chang Chun 
      Street, Beijing, China.
FAU - Wen, Xinmei
AU  - Wen X
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 
      China.
FAU - Lei, Lin
AU  - Lei L
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 
      China.
FAU - Shen, Faxiu
AU  - Shen F
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 
      China.
FAU - Fan, Zhirong
AU  - Fan Z
AUID- ORCID: 0000-0002-3118-9016
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 
      China.
FAU - Duo, Jianying
AU  - Duo J
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 
      China.
FAU - Lu, Yan
AU  - Lu Y
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 
      China.
FAU - Di, Li
AU  - Di L
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 
      China.
FAU - Wang, Min
AU  - Wang M
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 
      China.
FAU - Chen, Hai
AU  - Chen H
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 
      China.
FAU - Zhu, Wenjia
AU  - Zhu W
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 
      China.
FAU - Xu, Min
AU  - Xu M
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 
      China.
FAU - Wang, Suobin
AU  - Wang S
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 
      China.
FAU - Da, Yuwei
AU  - Da Y
AD  - Department of Neurology, Xuanwu Hospital, Capital Medical University, Chang Chun 
      Street, Beijing 100053, China.
LA  - eng
PT  - Journal Article
DEP - 20210127
PL  - England
TA  - Ther Adv Neurol Disord
JT  - Therapeutic advances in neurological disorders
JID - 101480242
PMC - PMC7844454
OTO - NOTNLM
OT  - FCGRT gene
OT  - intravenous immunoglobulin treatment
OT  - myasthenia gravis
OT  - variable number of tandem repeat polymorphism
COIS- Conflict of interest statement: The authors declare that there is no conflict of 
      interest.
EDAT- 2021/02/09 06:00
MHDA- 2021/02/09 06:01
PMCR- 2021/01/27
CRDT- 2021/02/08 05:36
PHST- 2020/06/03 00:00 [received]
PHST- 2020/12/11 00:00 [accepted]
PHST- 2021/02/08 05:36 [entrez]
PHST- 2021/02/09 06:00 [pubmed]
PHST- 2021/02/09 06:01 [medline]
PHST- 2021/01/27 00:00 [pmc-release]
AID - 10.1177_1756286420986747 [pii]
AID - 10.1177/1756286420986747 [doi]
PST - epublish
SO  - Ther Adv Neurol Disord. 2021 Jan 27;14:1756286420986747. doi: 
      10.1177/1756286420986747. eCollection 2021.