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

• Published in: Nefrología
• Main Authors: Castro Fernández, Paz, Sánchez de la Nieta García, María Dolores, Arambarri Segura, Minerva, González López, Lucía, Sidel Tambo, Diego, Moral Berrio, Esperanza, Ferrer García, Guillermo, Carreño Parrilla, Agustín, Martínez Calero, Alberto, Sánchez Fructuoso, Ana, Vozmediano Poyatos, Carmen
• Format: Journal Article
• Language: English
• Published: Spain 14-08-2021
• Subjects: Anti-glomerular basement membrane disease; Enfermedad antimembrana basal glomerular; HLA
• EISSN: 2013-2514

Anti-glomerular basement membrane disease (AGBM) is an autoinmune disorder characterized 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 analyzing 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.