Killer immunoglobulin-like receptor genes in uveitis

Ocul Immunol Inflamm. 2011 Jun;19(3):192-201. doi: 10.3109/09273948.2010.538798.


Purpose: to review the function and genetics of killer immunoglobulin-like receptors (KIRs) and studies of KIR genetic associations with uveitis.

Methods: Review of published studies.

Results: KIRs are receptors on NK and some T cells. They may inhibit or activate cellular function, such as cytotoxicity and cytokine production. Studies have been published examining KIR gene associations with birdshot chorioretinopathy (BCR), Vogt-Koyanagi-Harada (VKH) disease, and HLA-B27-associated acute anterior uveitis (AAU) and axial spondyloarthropathy. Evidence for increased activating and/or less inhibitory KIR and HLA gene combinations was found for BCR and VKH disease. In HLA-B27-associated disease, a trend toward decreased activation and stronger inhibition was found, except for the weakly inhibitory 3DL1 and Bw4(T80) combination. This latter combination was also found to confer risk in BCR.

Conclusions: KIR genetics are complex, as are the functions of KIR-bearing cells. Nonetheless, evidence for KIRs in the pathogenesis of uveitis has been found.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Choroid Diseases / genetics
  • Genetic Predisposition to Disease
  • HLA-B27 Antigen / metabolism
  • Humans
  • Receptors, KIR / genetics*
  • Receptors, KIR / metabolism
  • Retinal Diseases / genetics
  • Spondylarthropathies / genetics
  • Uveitis / genetics*
  • Uveitis / immunology
  • Uveitis / metabolism
  • Uveitis, Anterior / genetics*
  • Uveitis, Anterior / immunology
  • Uveitis, Anterior / metabolism
  • Uveomeningoencephalitic Syndrome


  • HLA-B27 Antigen
  • Receptors, KIR