Systemic sclerosis (scleroderma): clinical, genetic, and serologic subsets.
Immunogenetic markers, autoantibodies, and clinical features were studied in 47 patients, 35 Caucasian and 12 black, with systemic sclerosis. Twenty-two had generalized scleroderma, while 25 had limited skin involvement. HLA-DR1 (RR = 2.1, p = 0.08) and DR5 (RR = 2.1, p = 0.08) were increased in Caucasian patients vs controls as was the supertypic specificity HLA-DRw52 (RR = 2.8, p = 0.02, pc = 0.04). HLA-DR6.1 was increased in black patients vs controls (RR = 15.4, p = 0.008, pc = 0.088). There were no significant increases in any of the complement allotypes in either racial group. Anticentromere antibody was noted in 10 patients, all Caucasian; 7 had limited disease. Anti-Scl-70 was noted in 4 patients; all had generalized disease (p = 0.036). HLA-DR2 was present in all anti-Scl-70 positive patients (RR = 22.5, p = 0.006). Our results suggest that clinical subsets of systemic sclerosis can be defined by genetic and serological markers.
D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000032:analysis; D044383:Black People; D002503:Centromere / Q000276:immunology; D003165:Complement System Proteins / Q000032:analysis; D005260:Female; D005819:Genetic Markers; D006684:HLA-DR Antigens / Q000032:analysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D012044:Regression Analysis; D012595:Scleroderma, Systemic / Q000145:classification* / Q000235:genetics / Q000276:immunology; D044465:White People
D001323:Autoantibodies; D005819:Genetic Markers; D006684:HLA-DR Antigens; D003165:Complement System Proteins