PMID- 37349681 OWN - NLM STAT- MEDLINE DCOM- 20230626 LR - 20231121 IS - 1471-2369 (Electronic) IS - 1471-2369 (Linking) VI - 24 IP - 1 DP - 2023 Jun 22 TI - Primary membranous nephropathy in two siblings with one combined with anti-glomerular basement membrane disease: a case report. PG - 183 LID - 10.1186/s12882-023-03132-2 [doi] LID - 183 AB - BACKGROUND: The phospholipase A2 receptor (PLA2R) associated with membranous nephropathy (MN) is an organ-specific autoimmune disease associated with PLA2R and human leukocyte antigen (HLA) genes. Familial PLA2R-related MN is rarely reported. The combination of anti-GBM disease and MN has been well documented, though the mechanism behind it remains unclear. CASE PRESENTATION: We describe two siblings diagnosed with pathology-confirmed PLA2R-related MN 1 year apart. And one of the two siblings developed an anti-GBM disease. The high-resolution HLA typing showed identical alleles in both siblings, specifically heterozygotes of DRB1*15:01/*03:01. CONCLUSION: We describe a familial case of PLA2R-related MN supporting the role of genetic factors that HLA-DRB1*15:01 and DRB1*03:01 predispose patients in the development of PLA2R-related MN in the Han Chinese population. The combination of MN and anti-GBM disease may also partially be associated with the same susceptible HLA allele DRB1*15:01. CI - (c) 2023. The Author(s). FAU - Cheng, Yan-Jiao AU - Cheng YJ AD - Renal Division, Peking University First Hospital, Beijing, 100034, PR China. AD - Institute of Nephrology, Peking University, Beijing, 100034, PR China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, 100034, PR China. AD - Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, 100034, PR China. FAU - Jia, Xiao-Yu AU - Jia XY AD - Renal Division, Peking University First Hospital, Beijing, 100034, PR China. constancej@163.com. AD - Institute of Nephrology, Peking University, Beijing, 100034, PR China. constancej@163.com. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, 100034, PR China. constancej@163.com. AD - Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, 100034, PR China. constancej@163.com. FAU - Cao, Hong-Ru AU - Cao HR AD - Renal Division, Affiliated Hospital of Chifeng University, Chifeng, 024005, PR China. FAU - Zhao, Xiao-Yi AU - Zhao XY AD - Renal Division, Affiliated Hospital of Chifeng University, Chifeng, 024005, PR China. zxy5080@163.com. FAU - Zhou, Xu-Jie AU - Zhou XJ AD - Renal Division, Peking University First Hospital, Beijing, 100034, PR China. AD - Institute of Nephrology, Peking University, Beijing, 100034, PR China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, 100034, PR China. AD - Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, 100034, PR China. FAU - Yu, Xiao-Juan AU - Yu XJ AD - Renal Division, Peking University First Hospital, Beijing, 100034, PR China. AD - Institute of Nephrology, Peking University, Beijing, 100034, PR China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, 100034, PR China. AD - Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, 100034, PR China. FAU - Xu, Rong AU - Xu R AD - Renal Division, Peking University First Hospital, Beijing, 100034, PR China. AD - Institute of Nephrology, Peking University, Beijing, 100034, PR China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, 100034, PR China. AD - Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, 100034, PR China. FAU - Zhou, Fu-de AU - Zhou FD AD - Renal Division, Peking University First Hospital, Beijing, 100034, PR China. AD - Institute of Nephrology, Peking University, Beijing, 100034, PR China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, 100034, PR China. AD - Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, 100034, PR China. FAU - Wang, Su-Xia AU - Wang SX AD - Renal Division, Peking University First Hospital, Beijing, 100034, PR China. AD - Institute of Nephrology, Peking University, Beijing, 100034, PR China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, 100034, PR China. AD - Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, 100034, PR China. FAU - Cui, Zhao AU - Cui Z AD - Renal Division, Peking University First Hospital, Beijing, 100034, PR China. AD - Institute of Nephrology, Peking University, Beijing, 100034, PR China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, 100034, PR China. AD - Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, 100034, PR China. FAU - Zhao, Ming-Hui AU - Zhao MH AD - Renal Division, Peking University First Hospital, Beijing, 100034, PR China. AD - Institute of Nephrology, Peking University, Beijing, 100034, PR China. AD - Key Laboratory of Renal Disease, Ministry of Health of China, Beijing, 100034, PR China. AD - Key Laboratory of CKD Prevention and Treatment, Ministry of Education of China, Beijing, 100034, PR China. AD - Peking-Tsinghua Center for Life Sciences, Beijing, 100871, PR China. LA - eng PT - Case Reports PT - Journal Article DEP - 20230622 PL - England TA - BMC Nephrol JT - BMC nephrology JID - 100967793 RN - 0 (Autoantibodies) SB - IM MH - Humans MH - *Glomerulonephritis, Membranous/complications/diagnosis/genetics MH - *Anti-Glomerular Basement Membrane Disease MH - Siblings MH - Alleles MH - *Nephritis, Hereditary/genetics MH - Autoantibodies PMC - PMC10286333 OTO - NOTNLM OT - Anti-PLA2R antibody OT - Anti-glomerular basement membrane disease OT - Case report OT - HLA OT - Primary membranous nephropathy COIS- The authors have no conflicts of interest to disclose. EDAT- 2023/06/23 01:10 MHDA- 2023/06/26 06:41 PMCR- 2023/06/22 CRDT- 2023/06/22 23:34 PHST- 2021/12/30 00:00 [received] PHST- 2023/03/20 00:00 [accepted] PHST- 2023/06/26 06:41 [medline] PHST- 2023/06/23 01:10 [pubmed] PHST- 2023/06/22 23:34 [entrez] PHST- 2023/06/22 00:00 [pmc-release] AID - 10.1186/s12882-023-03132-2 [pii] AID - 3132 [pii] AID - 10.1186/s12882-023-03132-2 [doi] PST - epublish SO - BMC Nephrol. 2023 Jun 22;24(1):183. doi: 10.1186/s12882-023-03132-2.