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Front Immunol. 2015 Mar 24;6:134. doi: 10.3389/fimmu.2015.00134. eCollection 2015.

Behçet's Disease: Do Natural Killer Cells Play a Significant Role?.

Frontiers in immunology

Harry Petrushkin, Md Samiul Hasan, Miles R Stanford, Farida Fortune, Graham R Wallace

Affiliations

  1. Clinical and Diagnostic Oral Sciences, Queen Mary University of London , London , UK.
  2. Academic Unit of Ophthalmology, St Thomas's Hospital , London , UK.
  3. Centre for Translational Inflammation Research, University of Birmingham , Birmingham , UK.

PMID: 25852697 PMCID: PMC4371743 DOI: 10.3389/fimmu.2015.00134

Abstract

Behçet's disease (BD) is a complex inflammatory disease, of unknown etiology. While disease pathogenesis remains unclear, a strong relationship between BD and HLA-B*51 has been established over the last 30 years. A number of theories exist regarding the cause of BD; however, few are able to account for the increased rates of HLA-B*51 positive individuals, particularly around the Mediterranean basin and Middle-East where the prevalence is highest. This review outlines current immunogenetic data on BD and the immunoregulatory role natural killer cells may play. It also describes the interaction of the killer immunoglobulin-like receptor - KIR3DL1 with its ligand Bw4, which is found on HLA-B51. Finally, CD94/NKG2D, MICA, and ERAP are outlined with regard to their potential roles in BD.

Keywords: Behçet’s; Behçet’s disease; Behçet’s syndrome; HLA-B antigens; HLA-B*51; KIR; KIR3DL1; NK cells

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