KIRs and autoimmune disease: studies in systemic lupus erythematosus and scleroderma.
We investigated killer immunoglobulin-like receptors (KIRs) and the human leukocyte antigen (HLA)-C ligands for the corresponding inhibitory KIRs in Caucasian patients, 304 with systemic lupus erythematosus (SLE) and 90 with scleroderma [or progressive systemic sclerosis (PSS)] compared with 416 Caucasian controls. Compared with controls, KIR2DS1 in the absence of KIR2DS2 was increased in both SLE (P= 0.04) and PSS (P= 0.02). Only 42% of KIR2DS1-positive PSS patients had the appropriate HLA-C ligand for the corresponding inhibitory KIR compared with 61% of KIR2DS1 positive controls (P= 0.02). In the PSS group the presence of at least either activating KIR2DS1 and/or 2DS2 was significantly increased in patients when compared with controls (P= 0.001). This suggests that KIR receptors play a role in susceptibility to both PSS and SLE.
Pellett
F
F
HLA laboratory, Toronto Western Research Institute, Toronto, Ontario, Canada.
Siannis
F
F
Vukin
I
I
Lee
P
P
Urowitz
M B
MB
Gladman
D D
DD
D002102:Cadaver; D016022:Case-Control Studies; D005838:Genotype; D006680:HLA Antigens / Q000235:genetics*; D006801:Humans; D007694:Killer Cells, Natural / Q000276:immunology; D008180:Lupus Erythematosus, Systemic / Q000235:genetics*; D011446:Prospective Studies; D011971:Receptors, Immunologic / Q000235:genetics*; D054340:Receptors, KIR; D045743:Scleroderma, Diffuse / Q000235:genetics*; D014019:Tissue Donors
D006680:HLA Antigens; C496212:KIR2DS1 protein, human; C433932:KIR2DS2 protein, human; D011971:Receptors, Immunologic; D054340:Receptors, KIR