AUTHOR=Petrushkin Harry , Hasan Md. Samiul , Stanford Miles R. , Fortune Farida , Wallace Graham R. TITLE=Behçet’s Disease: Do Natural Killer Cells Play a Significant Role? JOURNAL=Frontiers in Immunology VOLUME=Volume 6 - 2015 YEAR=2015 URL=https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2015.00134 DOI=10.3389/fimmu.2015.00134 ISSN=1664-3224 ABSTRACT=Behçet's Disease is a complex inflammatory disease, of unknown aetiology. While disease pathogenesis remains unclear, a strong relationship between Behçet’s Disease and HLA-B*51 has been established over the last 30 years. A number of theories exist regarding the cause of Behçet's Disease, 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 Behçet’s disease 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 Behçet’s disease.