Bibliographic Details
| Title: |
Anti-glomerular basement membrane disease in HLA-identical non-twin siblings. |
| Alternate Title: |
Enfermedad antimembrana basal glomerular en hermanos no gemelos con HLA idéntico. |
| Authors: |
Castro Fernández, Paz1 pazcastro92@gmail.com, Sánchez de la Nieta García, María Dolores2, Arambarri Segura, Minerva1, González López, Lucía3, Sidel Tambo, Diego1, Moral Berrio, Esperanza1, Ferrer García, Guillermo1, Carreño Parrilla, Agustín1, Martínez Calero, Alberto1, Sánchez Fructuoso, Ana2, Vozmediano Poyatos, Carmen1 |
| Source: |
Nefrologia. Sep/Oct2022, Vol. 42 Issue 5, p607-610. 4p. |
| Subjects: |
ANTI-glomerular basement membrane disease, BASAL lamina, SIBLINGS, KIDNEY glomerulus diseases |
| Abstract (English): |
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 HLADR15 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. [ABSTRACT FROM AUTHOR] |
| Abstract (Spanish): |
La enfermedad antimembrana basal glomerular (EMBG) es un trastorno autoinmune caracterizado por la presencia de anticuerpos anti-membrana basal glomerular (AMBG), hemorragia pulmonar, glomerulonefritis necrotizante y depósito lineal de inmunoglobulinas en inmunofluorescencia directa. La predisposición genética, entre otros factores, posee un papel importante en el desarrollo de la enfermedad. Estudios previos han demostrado que el HLA-DR15 y HLA-DR4 se asocian con mayor riesgo de presentarla, mientras que el HLA-DR1 y HLA-DR7 han demostrado ser factor de protección frente a su desarrollo. Describimos el primer caso de dos hermanos no gemelos con EMBG con tipaje HLA idéntico, con factor de riesgo HLA-DR4 y factor de protección HLA-DR7. Planteamos la importancia de analizar el tipaje de histocompatibilidad en hermanos de pacientes con EMBG, para determinar el grado de susceptibilidad genética y plantear en ellos un seguimiento estrecho, con el objetivo de lograr un diagnóstico y tratamiento precoces en caso de presentar la enfermedad. [ABSTRACT FROM AUTHOR] |
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| Database: |
MedicLatina |
