Worman Howard J, Courvalin Jean Claude
Department of Medicine, College of Physicians and Surgeons, Columbia University, 630 West 168th Street, 10th Floor, Room 508, New York, NY 10032, USA.
Autoimmun Rev. 2003 Jun;2(4):211-7. doi: 10.1016/s1568-9972(03)00013-2.
The serological hallmark of primary biliary cirrhosis (PBC) is the presence of antimitochondrial antibodies. However, antinuclear antibodies (ANA) are also detectable in approximately 50% of subjects with PBC. Most clinical laboratories use indirect immunofluorescence microscopy to detect ANA and two labeling patterns that predominate in PBC are 'punctate nuclear rim' and 'multiple nuclear dots.' Work over the past several years has shown that antibodies giving these patterns most often recognize nuclear pore membrane protein gp210 and nuclear body protein sp100, respectively. These ANA are highly specific for PBC and detected in approximately 25% of patients. Less frequently, ANA apparently unique to PBC recognize other proteins of the nuclear envelope and nuclear bodies. While antibodies against gp210, sp100 and some other nuclear proteins are very specific to PBC and may therefore be useful diagnostic markers, their connection to pathogenesis remains to be elucidated.
原发性胆汁性肝硬化（PBC）的血清学标志是抗线粒体抗体的存在。然而，在大约50%的PBC患者中也可检测到抗核抗体（ANA）。大多数临床实验室使用间接免疫荧光显微镜检测ANA，PBC中占主导的两种标记模式是“点状核周缘”和“多个核点”。过去几年的研究表明，产生这些模式的抗体最常分别识别核孔膜蛋白gp210和核体蛋白sp100。这些ANA对PBC具有高度特异性，在大约25%的患者中可检测到。较少见的是，PBC特有的ANA识别核膜和核体的其他蛋白质。虽然针对gp210、sp100和其他一些核蛋白的抗体对PBC非常特异，因此可能是有用的诊断标志物，但它们与发病机制的联系仍有待阐明。
