Okano Y
Nippon Kokan Hospital, Kawasaki, Japan.
Rheum Dis Clin North Am. 1996 Nov;22(4):709-35. doi: 10.1016/s0889-857x(05)70297-0.
The presence of autoantibodies to intracellular molecules is the hallmark immunologic finding of SSc. Recent sophisticated methods have contributed to characterization of unidentified antigens of ANA in sera from patients with SSc. Antibodies to RNA polymerases are the third major SSc-specific ANA, in addition to anti-topo I and anticentromere antibodies, and it is now possible to identify over 85% of SSc patients. These antibodies have proved helpful in diagnosis of this disease. An immunogenetic predisposition to synthesis of the ANAs has been unveiled. Both antigen-driven and molecular mimicry hypotheses have been proposed for ANA production in SSc. Although the role of ANAs in the pathogenesis of SSc is not yet known, it is logical to assume that the origin of ANAs is linked to the causes of this disease because each antibody is associated with its own unique constellation of clinical features.
针对细胞内分子的自身抗体的存在是系统性硬化症标志性的免疫学发现。最近的先进方法有助于对系统性硬化症患者血清中未鉴定的抗核抗体抗原进行特征描述。除抗拓扑异构酶I抗体和抗着丝点抗体外,抗RNA聚合酶抗体是系统性硬化症特异性抗核抗体的第三大主要类型,现在已能够识别超过85%的系统性硬化症患者。这些抗体已被证明有助于诊断这种疾病。已经揭示了抗核抗体合成的免疫遗传易感性。针对系统性硬化症中抗核抗体的产生提出了抗原驱动和分子模拟假说。虽然抗核抗体在系统性硬化症发病机制中的作用尚不清楚,但可以合理推测抗核抗体的产生与这种疾病的病因有关,因为每种抗体都与自身独特的临床特征组合相关。