Anti-glomerular basement membrane disease in HLA-identical non-twin siblings

Nefrologia (Engl Ed). 2022 Sep-Oct;42(5):607-610. doi: 10.1016/j.nefroe.2021.04.010. Epub 2023 Jan 28.

Abstract

Anti glomerular basement membrane disease (AGBM) is an autoinmune disorder characterised by the presence of anti-glomerular basement membrane (Anti-GBM) antibodies, alveolar hemorrhage, necrotizing glomerulonephritis, and linear deposition of immunoglobulins through direct inmunofluorescence. Genetic predisposition, among other factors, plays an important role in the development of the disease. Previous studies have shown that HLA-DR15 and HLA-DR4 increase the risk of presenting it, while HLA-DR1 and HLA-DR7 protect against its development. We describe the first case of two non-twin siblings with AGBM and identical HLA, with HLA-DR4 as risk factor and HLA-DR7 as protection factor. We propose the importance of analysing HLA in siblings of patients with AGBM, to determine the degree of genetic susceptibility and to carry out a close follow-up on them, with the aim of achieving an early diagnosis and treatment in case of presenting the disease.

Keywords: Anti glomerular basement membrane disease; Enfermedad antimembrana basal glomerular; HLA.

Publication types

  • Case Reports

MeSH terms

  • Anti-Glomerular Basement Membrane Disease* / diagnosis
  • Glomerulonephritis*
  • HLA-DR4 Antigen
  • HLA-DR7 Antigen
  • Humans
  • Siblings

Substances

  • HLA-DR7 Antigen
  • HLA-DR4 Antigen