Is Behçet's Disease a "class 1-opathy"? The role of HLA-B*51 in the pathogenesis of Behçet's Disease.
Clinical and experimental immunology
The association between carriage of the HLA-B*51 allele and development of Behçet's Disease (BD) has been known since the early 1970s, but the exact mechanisms responsible for its role in pathogenesis remain much-debated. In an effort to explain the disease process, it has been suggested that BD constitutes one of a newly-termed group of diseases, the 'MHC-I-opathies'. Other MHC-I-opathies' include Ankylosing Spondylitis and other HLA-B*27-associated spondyloarthropathies, and HLA-C*0602-associated skin psoriasis. Recent work analysing the peptidome of HLA-B*51 suggests that altered peptide presentation by HLA-B*51 is vital to the disease process. In this review, we argue that immune receptor interactions with HLA-B*51 or the HLA-B*51-peptide complex could lead to development of inflammation in BD. The evidence for CD8+ T-cell involvement is weak, and based on emerging studies it seems more likely that NK or other cell interactions, perhaps mediated by LILR or KIR receptors, are culpable in pathogenesis. HLA misfolding directly leading to inflammation is another hypothesis for BD pathogenesis that deserves greater investigation. Ultimately, greater understanding of HLA-B*51's unique role in BD will likely lead to improved development of therapeutic strategies. This article is protected by copyright. All rights reserved.
- 1Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK.
- 2Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK.
- 3Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK.
- 4Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK.
© 2017 British Society for Immunology.
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||Last Modified: 2016-03-27