Association of killer cell immunoglobulin-like receptor genes with primary Sjögren's syndrome among Chinese population.

BACKGROUND

Primary Sjögren's syndrome (pSS) is a chronic and common autoimmune disorder which is caused by inflammatory infiltration of the exocrine glands salivary glands and lacrimal glands are the main sites of involvement. Killer cell immunoglobulin-like receptors (KIRs), are crucial for the development and function of natural killer (NK) cells. Moreover, the activity of NK cells is affected by the interaction of KIRs with HLA class I ligands. The pathogenesis of autoimmune diseases has been linked to some KIR genes. Further research on the relationship between KIR and these autoimmune diseases is of great significance for the diagnosis and treatment of these diseases. This study aimed to detect and analyze the connection between the polymorphism of the KIR and HLA-Cw genes and the pathogenesis of pSS.

METHODS

We took 56 pSS patients as the study group and 32 healthy people as the control group. Blood samples were collected to perform a polymerase chain reaction with sequence specific primers (PCR-SSP). Then a comparative analysis of gene frequencies of 16 KIR genes and HLA-Cw in the two groups was performed.

RESULTS

The genotype frequencies of both KIR2DS3 (P = 0.0027) and KIR3DS1 (P = 0.006) were significantly lower in patients with pSS compared to healthy individuals, indicating a negative correlation of these two activating KIR genes. There were more individuals who had more than three activating KIR genes in the control group than in the patient group (P = 0.004). And the KIR2DL1/HLA-C2 gene combination (P = 0.022) was significantly more in pSS group than in controls.

CONCLUSION

Our results may imply that via influencing the activity, development, capacitation, and functionality of NK cells, NKT cells, and part T cells, the decrease of activated KIR genes and the increase of the frequency of the inhibitory KIR and its specific ligand gene combination（KIR2DL1/HLA-C2) may contribute to the pathogenesis of pSS.