Biomaterial Database

Anti-glomerular basement membrane disease in HLA-identical non-twin siblings. 2022

Paz Castro Fernández, and María Dolores Sánchez de la Nieta García, and Minerva Arambarri Segura, and Lucía González López, and Diego Sidel Tambo, and Esperanza Moral Berrio, and Guillermo Ferrer García, and Agustín Carreño Parrilla, and Alberto Martínez Calero, and Ana Sánchez Fructuoso, and Carmen Vozmediano Poyatos

Servicio de Nefrología, Hospital General Universitario de Ciudad Real, Ciudad Real, Spain. Electronic address: pazcastro92@gmail.com.

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.

UI	MeSH Term	Description	Entries
D005921	Glomerulonephritis	Inflammation of the renal glomeruli (KIDNEY GLOMERULUS) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. These structural and functional abnormalities usually lead to HEMATURIA; PROTEINURIA; HYPERTENSION; and RENAL INSUFFICIENCY.	Bright Disease,Kidney Scarring,Glomerulonephritides,Scarring, Kidney
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D015804	HLA-DR4 Antigen	An HLA-DR antigen which is associated with HLA-DRB1 CHAINS encoded by DRB1*04 alleles.	HLA-DR4,Antigen, HLA-DR4,HLA DR4 Antigen
D015809	HLA-DR7 Antigen	A HLA-DR antigen that is associated with HLA-DRB1 CHAINS encoded by DRB1*07 alleles.	HLA-DR7,Antigen, HLA-DR7,HLA DR7 Antigen
D019867	Anti-Glomerular Basement Membrane Disease	An autoimmune disease of the KIDNEY and the LUNG. It is characterized by the presence of circulating autoantibodies targeting the epitopes in the non-collagenous domains of COLLAGEN TYPE IV in the basement membranes of kidney glomeruli (KIDNEY GLOMERULUS) and lung alveoli (PULMONARY ALVEOLI), and the subsequent destruction of these basement membranes. Clinical features include pulmonary alveolar hemorrhage and glomerulonephritis.	Anti-GBM Disease,Goodpasture Syndrome,Goodpasture's Syndrome,Lung Purpura with Nephritis,Anti GBM Disease,Anti Glomerular Basement Membrane Disease,Goodpastures Syndrome,Syndrome, Goodpasture,Syndrome, Goodpasture's
D035781	Siblings	Persons or animals having at least one parent in common. (American College Dictionary, 3d ed)	Brothers,Sisters,Brother,Sibling,Sister