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

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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]
Copyright of Nefrologia is the property of Revista Nefrologia and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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Items – Name: Title
  Label: Title
  Group: Ti
  Data: Anti-glomerular basement membrane disease in HLA-identical non-twin siblings.
– Name: TitleAlt
  Label: Alternate Title
  Group: TiAlt
  Data: Enfermedad antimembrana basal glomerular en hermanos no gemelos con HLA idéntico.
– Name: Author
  Label: Authors
  Group: Au
  Data: <searchLink fieldCode="AR" term="%22Castro+Fernández%2C+Paz%22">Castro Fernández, Paz</searchLink><relatesTo>1</relatesTo><i> pazcastro92@gmail.com</i><br /><searchLink fieldCode="AR" term="%22Sánchez+de+la+Nieta+García%2C+María+Dolores%22">Sánchez de la Nieta García, María Dolores</searchLink><relatesTo>2</relatesTo><br /><searchLink fieldCode="AR" term="%22Arambarri+Segura%2C+Minerva%22">Arambarri Segura, Minerva</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22González+López%2C+Lucía%22">González López, Lucía</searchLink><relatesTo>3</relatesTo><br /><searchLink fieldCode="AR" term="%22Sidel+Tambo%2C+Diego%22">Sidel Tambo, Diego</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Moral+Berrio%2C+Esperanza%22">Moral Berrio, Esperanza</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Ferrer+García%2C+Guillermo%22">Ferrer García, Guillermo</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Carreño+Parrilla%2C+Agustín%22">Carreño Parrilla, Agustín</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Martínez+Calero%2C+Alberto%22">Martínez Calero, Alberto</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Sánchez+Fructuoso%2C+Ana%22">Sánchez Fructuoso, Ana</searchLink><relatesTo>2</relatesTo><br /><searchLink fieldCode="AR" term="%22Vozmediano+Poyatos%2C+Carmen%22">Vozmediano Poyatos, Carmen</searchLink><relatesTo>1</relatesTo>
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  Data: <searchLink fieldCode="JN" term="%22Nefrologia%22">Nefrologia</searchLink>. Sep/Oct2022, Vol. 42 Issue 5, p607-610. 4p.
– Name: Subject
  Label: Subjects
  Group: Su
  Data: <searchLink fieldCode="DE" term="%22ANTI-glomerular+basement+membrane+disease%22">ANTI-glomerular basement membrane disease</searchLink><br /><searchLink fieldCode="DE" term="%22BASAL+lamina%22">BASAL lamina</searchLink><br /><searchLink fieldCode="DE" term="%22SIBLINGS%22">SIBLINGS</searchLink><br /><searchLink fieldCode="DE" term="%22KIDNEY+glomerulus+diseases%22">KIDNEY glomerulus diseases</searchLink>
– Name: Abstract
  Label: Abstract (English)
  Group: Ab
  Data: 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]
– Name: Abstract
  Label: Abstract (Spanish)
  Group: Ab
  Data: 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]
– Name: AbstractSuppliedCopyright
  Label:
  Group: Ab
  Data: <i>Copyright of Nefrologia is the property of Revista Nefrologia and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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      – Type: doi
        Value: 10.1016/j.nefroe.2021.04.010
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      – Code: eng
        Text: English
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    Subjects:
      – SubjectFull: ANTI-glomerular basement membrane disease
        Type: general
      – SubjectFull: BASAL lamina
        Type: general
      – SubjectFull: SIBLINGS
        Type: general
      – SubjectFull: KIDNEY glomerulus diseases
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      – TitleFull: Anti-glomerular basement membrane disease in HLA-identical non-twin siblings.
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              Text: Sep/Oct2022
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