Genetic and immunological differences between Japanese patients with diffuse scleroderma and limited scleroderma.
OBJECTIVE
To study the association between HLA-DR and scleroderma (SSc), subsets of SSc, and autoantibodies in SSc.


METHODS
HLA-DR antigens were determined in 45 Japanese patients with SSc. The association between HLA-DR and SSc, subsets of SSc, and autoantibodies was analyzed in 22 patients with SSc excluding mixed connective tissue disease (MCTD)/overlap syndrome (OL).


RESULTS
When the 20 patients with MCTD and 3 patients with OL were excluded from the original patient group, a significant increase of HLA-DR2 was observed (59 vs 29% of controls, p < 0.01). The frequency of DR2 increased to 69% in patients with diffuse SSc (p < 0.01). DR1, which was not found in diffuse SSc, was found in 2 of 9 patients with limited SSc. The frequency of DR2 was significantly higher in patients with antitopoisomerase I (10/12, 83%, p < 0.05). In contrast, DR1 was found only in 2 patients with anticentromere antibodies (ACA), and all 5 patients with ACA had no HLA-DR2 (p < 0.01).


CONCLUSIONS
Our results suggest that different HLA-DR markers may be associated with the production of distinct autoantibodies in diffuse SSc and limited SSc.
Satoh
M
M
Department of Medicine, Keio University School of Medicine, Tokyo, Japan.
Akizuki
M
M
Kuwana
M
M
Mimori
T
T
Yamagata
H
H
Yoshida
S
S
Homma
M
M
Yamamoto
T
T
Sasazuki
T
T
D000906:Antibodies / Q000032:analysis; D000918:Antibody Specificity; D001323:Autoantibodies / Q000276:immunology; D002503:Centromere / Q000276:immunology; D004264:DNA Topoisomerases, Type I / Q000276:immunology; D006684:HLA-DR Antigens / Q000032:analysis; D006801:Humans; D012594:Scleroderma, Localized / Q000235:genetics* / Q000276:immunology* / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000235:genetics* / Q000276:immunology* / Q000503:physiopathology
D000906:Antibodies; D001323:Autoantibodies; D006684:HLA-DR Antigens; D004264:DNA Topoisomerases, Type I