Autoantibodies in systemic sclerosis.
There are 3 major autoantibodies in sera from patients with scleroderma: 1) anticentromere antibodies (ACA), 2) anti-topoisomerase I (anti-topo I), and 3) anti-RNA polymerases. Each is present in about 25% of patients and are mutually exclusive. ACA are found in patients with primary and secondary Raynaud's disease and in patients with primary biliary cirrhosis. Anti-topo I and anti-RNA polymerases are found exclusively in scleroderma. Each autoantibody is present in specific subsets of scleroderma patients. ACA and anti-topo I have been well studied and their presence and titer are stable over time. The anti-topo I and ACA are of all three isotypes, recognize multiple epitopes on the antigens and have stable cross reactive or private idiotypes. The antigen, topoisomerase I, has domains which have homology to viral proteins. Other autoantibodies predominantly recognize nucleolar antigens, are found in less than 15% of patients, and are not specific for scleroderma.
Vázquez-Abad
D
D
Department of Medicine, University of Connecticut Health Center, Farmington 06030-1310, USA.
Rothfield
N F
NF
D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens / Q000276:immunology; D017675:CREST Syndrome / Q000276:immunology; D002503:Centromere / Q000276:immunology; D004264:DNA Topoisomerases, Type I / Q000276:immunology; D012321:DNA-Directed RNA Polymerases / Q000276:immunology; D006801:Humans; D008928:Mitochondria / Q000276:immunology; D012261:Ribonucleoproteins / Q000276:immunology; D017411:Ribonucleoproteins, Small Nuclear; D012595:Scleroderma, Systemic / Q000276:immunology*; D055517:snRNP Core Proteins
D001323:Autoantibodies; D001324:Autoantigens; D012261:Ribonucleoproteins; D017411:Ribonucleoproteins, Small Nuclear; D055517:snRNP Core Proteins; D012321:DNA-Directed RNA Polymerases; D004264:DNA Topoisomerases, Type I