RESEARCH - Association of killer cell immunoglobulin-like receptors with primary Sjogren's syndrome

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Rheumatology Advance Access published online on January 30, 2009

Rheumatology, doi:10.1093/rheumatology/ken503

Association of killer cell immunoglobulin-like receptors with primary

Sjögren's syndrome

P. Lowe1, Mark A. Cook2, Simon J. Bowman3, C. Briggs1 and

the UK Sjögren's Interest Group*

1Department of Histocompatibility and Immunogenetics, NHS Blood and

Transplant, 2Department of Haematology, University Hospital Birmingham

NHS Foundation Trust, Edgbaston, 3Department of Rheumatology,

University Hospital Birmingham, NHS Foundation Trust, Birmingham, UK.

Abstract

Objective. SS is a chronic inflammatory condition characterized by

systemic and tissue-specific autoimmune features. In view of recent

findings indicating a role for killer cell immunoglobulin-like

receptors (KIRs) in the pathogenesis of other autoimmune rheumatic

disorders such as SSc, and the autoimmune disorders RA and PsA, we

sought to determine whether KIRs predict general or specific

susceptibility in SS.

Methods. Eleven separate KIR genes were typed using PCR

sequence-specific primers on genomic DNA from 72 patients diagnosed

with primary SS and a control panel consisting of 223 blood donors.

Results. We found no individual KIR genes to be associated with SS. In

contrast, 11 patients with primary SS (15%) and 9 control blood donors

(4%) had KIR genotypes with the activating KIR2DS2 in the absence of

its corresponding inhibitory homologue KIR2DL2 (P = 0.01). Further

analysis of these individuals showed that seven SS patients were

positive for HLA-C ligand for KIR2DS2 only compared with one control

sample (P = 0.00026).

Conclusion. The genetic combination of KIR2DS2+ and KIR2DL2– in the

presence of HLA-C ligand specific for activating KIR2DS2 is associated

with primary SS. This implies that autologous KIR–ligand interaction

is a contributory factor to predisposition for this disease.

http://rheumatology.oxfordjournals.org/cgi/content/abstract/ken503v1?papetoc

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