Vázquez-Abad D, Rothfield N F
Department of Medicine, University of Connecticut Health Center, Farmington 06030-1310, USA.
Int Rev Immunol. 1995;12(2-4):145-57. doi: 10.3109/08830189509056709.
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.
硬皮病患者血清中有3种主要自身抗体：1）抗着丝点抗体（ACA），2）抗拓扑异构酶I（抗topo I），以及3）抗RNA聚合酶。每种抗体约存在于25%的患者中，且相互排斥。ACA见于原发性和继发性雷诺病患者以及原发性胆汁性肝硬化患者。抗topo I和抗RNA聚合酶仅见于硬皮病患者。每种自身抗体存在于硬皮病患者的特定亚组中。ACA和抗topo I已得到充分研究，其存在和滴度随时间稳定。抗topo I和ACA具有所有三种同种型，识别抗原上的多个表位，具有稳定的交叉反应性或独特型。抗原拓扑异构酶I具有与病毒蛋白同源的结构域。其他自身抗体主要识别核仁抗原，见于不到15%的患者，并非硬皮病所特有。
