BMC Nephrology (Jun 2023)

Primary membranous nephropathy in two siblings with one combined with anti-glomerular basement membrane disease: a case report

  • Yan-jiao Cheng,
  • Xiao-yu Jia,
  • Hong-ru Cao,
  • Xiao-yi Zhao,
  • Xu-jie Zhou,
  • Xiao-juan Yu,
  • Rong Xu,
  • Fu-de Zhou,
  • Su-xia Wang,
  • Zhao Cui,
  • Ming-hui Zhao

DOI
https://doi.org/10.1186/s12882-023-03132-2
Journal volume & issue
Vol. 24, no. 1
pp. 1 – 5

Abstract

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Abstract 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.

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