Blog
About

0
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Human leucocyte antigen-associated anti-glomerular basement membrane disease in siblings

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          We report a case of anti-glomerular basement membrane (GBM) disease in association with human leucocyte antigen (HLA) DRB1 15:01. A 71-year-old woman presented with oligoanuric acute kidney injury accompanied by high titre anti-GBM antibodies. Renal biopsy revealed a severe crescentic glomerulonephritis. Her brother had presented 6 years earlier with oligoanuric acute kidney injury. He was dual positive for MPO ANCA and anti-GBM antibodies. Renal biopsy was not performed. Both had an absence of pulmonary involvement. Tissue typing confirmed both were heterozygous for HLA DRB1 15:01 and DRB1 04:03.

          Related collections

          Most cited references 4

          • Record: found
          • Abstract: found
          • Article: not found

          The HLA complex in Goodpasture's disease: a model for analyzing susceptibility to autoimmunity.

          Human lymphocyte antigen (HLA) associations are recognized for many autoimmune diseases, but the mechanisms are not clear. Goodpasture's disease provides a unique opportunity to investigate possible mechanisms because strong HLA associations are known, the autoantigen is well defined, and major antigen-derived peptides presented bound to HLA molecules have been identified. Therefore, it may be possible to directly analyze interactions between the antigen and HLA molecules associated with the disease, and to examine influences on antigen presentation to T cells. Towards this goal, we present a detailed analysis of HLA associations with the disease and examine molecular mechanisms that could account for them.
            Bookmark
            • Record: found
            • Abstract: not found
            • Article: not found

            Spatial and Temporal Clustering of Anti-Glomerular Basement Membrane Disease

              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Susceptibility to anti-glomerular basement membrane disease is strongly associated with HLA-DRB1 genes.

              Anti-glomerular basement membrane (anti-GBM) disease is caused by autoimmunity to a component of glomerular basement membrane. The major autoantigen has been identified as the NC1 domain of the alpha 3 chain of type IV collagen, and patients are characterized by the presence of specific autoantibodies to this molecule. In common with other autoimmune disorders, there is a strong association with HLA genes, with up to 80% of patients inheriting an HLA-DR2 haplotype. To examine the genetic basis of susceptibility to anti-GBM disease in more detail, the HLA-DRB and DQB alleles inherited by 82 patients were analyzed using sequence specific oligonucleotides. This identified a hierachy of association of DRB1 genes with anti-GBM disease, including susceptibility (DRB1*15, DRB1*04), neutral (DRB1*03) and protective (DRB1*07) alleles. Analysis of inherited haplotypes, particularly DRB1*04 and DRB1*07 carrying haplotypes, provided further evidence that the primary association was with genes at the DRB1 locus. Comparison of the sequences of the positively and negatively associated alleles showed that polymorphic residues in the second peptide binding region of the HLA Class II antigen binding groove segregated with disease. This work supports the hypothesis that the HLA associations in anti-GBM disease reflect the ability of certain Class II molecules to bind and present peptides derived from the autoantigen to T helper cells.
                Bookmark

                Author and article information

                Journal
                Clin Kidney J
                Clin Kidney J
                ckj
                Clinical Kidney Journal
                Oxford University Press
                2048-8505
                2048-8513
                April 2020
                03 June 2019
                03 June 2019
                : 13
                : 2
                : 261-262
                Affiliations
                Department of Renal Medicine, Royal Preston Hospital , Preston, UK
                Author notes
                Correspondence and offprint requests to: Martin E. Durcan; E-mail: martin.durcan@ 123456mft.nhs.uk
                Article
                sfz058
                10.1093/ckj/sfz058
                7147311
                © The Author(s) 2019. Published by Oxford University Press on behalf of ERA-EDTA.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

                Page count
                Pages: 7
                Product
                Categories
                Exceptional Case

                Nephrology

                hereditary, goodpasture syndrome, familial, anti-gbm, acute kidney injury

                Comments

                Comment on this article