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Antinuclear antibodies as ancillary markers in primary biliary cirrhosis.

机译:抗核抗体是原发性胆汁性肝硬化的辅助标志物。

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Antimitochondrial antibodies are the serological hallmark of primary biliary cirrhosis (PBC). Besides antimitochondrial antibodies, the autoantibody profile of PBC includes antinuclear antibodies (ANA) which are detectable by indirect immunofluorescence in up to 50% of PBC patients. Two immunofluorescence patterns are considered 'PBC-specific': the multiple nuclear dots and rim-like/membranous patterns. The target antigens of the multiple nuclear dots pattern have been identified as Sp100 and promyelocytic leukemia protein, whereas the rim-like/membranous pattern is given by autoantibodies recognizing multiple proteins such as gp210, nucleoporin p62 and the lamin B receptor. Other ANA, especially those already known in the rheumatological setting, such as anticentromere, anti-SSA/Ro and anti-dsDNA antibodies, can be frequently found in PBC, often coexisting in the same patient. In this article, we will report on recent progress in the antigenic characterization of ANA in PBC, their detection with both traditional assays and Western blot/ELISA with molecularly defined nuclear antigens, and we will discuss their clinical significance.
机译:抗线粒体抗体是原发性胆汁性肝硬化(PBC)的血清学标志。除抗线粒体抗体外,PBC的自身抗体谱还包括抗核抗体(ANA),可通过间接免疫荧光检测在多达50%的PBC患者中进行检测。两种免疫荧光模式被认为是“ PBC特异性的”:多核点和边缘样/膜模式。多核点模式的靶抗原已被鉴定为Sp100和早幼粒细胞白血病蛋白,而轮缘样/膜模式是由识别多种蛋白(例如gp210,核孔蛋白p62和层粘连蛋白B受体)的自身抗体产生的。其他ANA,尤其是风湿病学领域已知的那些ANA,例如抗着丝粒,抗SSA / Ro和抗dsDNA抗体,经常在PBC中发现,通常在同一患者中共存。在本文中,我们将报告PBC中ANA抗原表征的最新进展,传统检测方法的检测以及分子定义的核抗原的Western blot / ELISA的检测,并讨论它们的临床意义。

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