PMID- 35488492 OWN - NLM STAT- MEDLINE DCOM- 20220718 LR - 20221222 IS - 1468-1331 (Electronic) IS - 1351-5101 (Linking) VI - 29 IP - 8 DP - 2022 Aug TI - HLA-DRB1*03:01 is associated with female sex and younger age of anti-LGI1 encephalitis. PG - 2367-2375 LID - 10.1111/ene.15376 [doi] AB - BACKGROUND AND PURPOSE: This study was undertaken to investigate the association between human leukocyte antigen (HLA) genotype and clinical characteristics of anti-LGI1 encephalitis. METHODS: HLA genotyping was performed in 34 patients with anti-LGI1 encephalitis admitted to West China Hospital between April 2014 and May 2021, as well as in 305 healthy controls. The online tool NetMHCIIpan 4.0 and AutoDock Vina software were used to predict binding between LGI1 peptide and HLA class II molecules. RESULTS: Risk of anti-LGI1 encephalitis was strongly associated with the DRB1*03:01 allele (odds ratio [OR] = 4.31, 95% confidence interval [CI] = 1.96-9.25, corrected p = 2.75 x 10(-4) ) and the DRB1*03:01-DQB1*02:01 haplotype (OR = 4.45, 95% CI = 2.02-9.59, corrected p = 2.94 x 10(-4) ). Compared to carriers of the DRB1*07:01 allele, those with the DRB1*03:01 allele were more likely to be female (93.3% vs. 33.3%, p = 0.004) and to be younger (median age = 38 vs. 65 years, p < 0.001). DRB1*03:01 carriers showed stronger response to immunotherapy than carriers of the DRB1*07:01 allele, based on median score decrease on the modified Rankin scale (2, interquartile range = 1-2 vs. 1, interquartile range = 0-1; p = 0.03) at 4 weeks after immunotherapy. Prediction and docking algorithms suggested that the LGI1 peptide can bind to the DRB1*03:01 molecule strongly. CONCLUSIONS: The strong association between anti-LGI1 encephalitis and certain HLA class II alleles supports a primary autoimmune origin for the disease. Carriers of the DRB1*03:01 allele in Chinese patients with anti-LGI1 encephalitis are more likely to be female, to suffer earlier disease onset, and to respond better to immunotherapy. CI - (c) 2022 European Academy of Neurology. FAU - Liu, Xu AU - Liu X AD - Department of Neurology, West China Hospital, Sichuan University, Chengdu, China. AD - Institute of Brain Science and Brain-Inspired Technology, West China Hospital, Sichuan University, Chengdu, China. FAU - Guo, Kun Dian AU - Guo KD AD - Department of Neurology, West China Hospital, Sichuan University, Chengdu, China. AD - Institute of Brain Science and Brain-Inspired Technology, West China Hospital, Sichuan University, Chengdu, China. FAU - Lin, Jingfang AU - Lin J AD - Department of Neurology, West China Hospital, Sichuan University, Chengdu, China. AD - Institute of Brain Science and Brain-Inspired Technology, West China Hospital, Sichuan University, Chengdu, China. FAU - Gong, Xue AU - Gong X AD - Department of Neurology, West China Hospital, Sichuan University, Chengdu, China. AD - Institute of Brain Science and Brain-Inspired Technology, West China Hospital, Sichuan University, Chengdu, China. FAU - Li, Ai Qing AU - Li AQ AD - Department of Neurology, West China Hospital, Sichuan University, Chengdu, China. AD - Institute of Brain Science and Brain-Inspired Technology, West China Hospital, Sichuan University, Chengdu, China. FAU - Zhou, Dong AU - Zhou D AUID- ORCID: 0000-0001-7101-4125 AD - Department of Neurology, West China Hospital, Sichuan University, Chengdu, China. AD - Institute of Brain Science and Brain-Inspired Technology, West China Hospital, Sichuan University, Chengdu, China. FAU - Hong, Zhen AU - Hong Z AUID- ORCID: 0000-0002-0014-6873 AD - Department of Neurology, West China Hospital, Sichuan University, Chengdu, China. AD - Institute of Brain Science and Brain-Inspired Technology, West China Hospital, Sichuan University, Chengdu, China. AD - Department of Neurology, Chengdu Shangjin Nanfu Hospital, Chengdu, China. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20220510 PL - England TA - Eur J Neurol JT - European journal of neurology JID - 9506311 RN - 0 (Autoantibodies) RN - 0 (HLA-DRB1 Chains) RN - 0 (HLA-DRB1*03:01 antigen) RN - 0 (anti-leucine-rich glioma-inactivated 1 autoantibody) RN - Autoimmune limbic encephalitis SB - IM MH - Adult MH - Alleles MH - Autoantibodies/blood MH - *Autoimmune Diseases/genetics MH - Female MH - Gene Frequency MH - Genetic Predisposition to Disease MH - *HLA-DRB1 Chains/genetics MH - Haplotypes MH - Humans MH - *Limbic Encephalitis/genetics MH - Male MH - Sex Factors OTO - NOTNLM OT - anti-LGI1 encephalitis OT - human leukocyte antigen EDAT- 2022/05/01 06:00 MHDA- 2022/07/19 06:00 CRDT- 2022/04/30 03:13 PHST- 2022/03/04 00:00 [revised] PHST- 2022/02/01 00:00 [received] PHST- 2022/04/20 00:00 [accepted] PHST- 2022/05/01 06:00 [pubmed] PHST- 2022/07/19 06:00 [medline] PHST- 2022/04/30 03:13 [entrez] AID - 10.1111/ene.15376 [doi] PST - ppublish SO - Eur J Neurol. 2022 Aug;29(8):2367-2375. doi: 10.1111/ene.15376. Epub 2022 May 10.