Nishio A, Coppel R, Ishibashi H, Gershwin M E
Department of Gastroenterology, Tenri Hospital, Nara, Japan.
Baillieres Best Pract Res Clin Gastroenterol. 2000 Aug;14(4):535-47. doi: 10.1053/bega.2000.0102.
Mitochondrial autoantigens and their B and T cell autoepitopes have been well defined in primary biliary cirrhosis (PBC). However, the relationships of the antimitochondrial antibodies and the mechanisms of bile duct destruction in PBC remain an enigma. The serological hallmark of PBC remains the presence of antibodies to mitochondria, particularly to the E2 component of the pyruvate dehydrogenase complex (PDC-E2). However, several mechanisms may now be proposed which may explain the immune-mediated bile duct damage in PBC. These include the possible role of T cell-mediated cytotoxicity as well as the interaction between the IgA class of antimitochondrial antibodies and the mitochondrial autoantigens. A prominent feature in this discussion is the highly directed and specific immune response to the mitochondrial antigens, including PDC-E2 as well as other members of the 2-oxo-acid dehydrogenase complexes. Ultimately, the mechanisms that lead to this immune reaction should provide data on other questions in PBC, including the reasons for female predominance, the absence of PBC in children and the relative ineffectiveness of immunosuppressive agents.
线粒体自身抗原及其B细胞和T细胞自身表位在原发性胆汁性肝硬化（PBC）中已得到明确界定。然而，抗线粒体抗体与PBC中胆管破坏机制之间的关系仍是一个谜。PBC的血清学标志仍然是存在抗线粒体抗体，尤其是针对丙酮酸脱氢酶复合体（PDC-E2）E2成分的抗体。然而，现在可以提出几种机制来解释PBC中免疫介导的胆管损伤。这些机制包括T细胞介导的细胞毒性的可能作用，以及IgA类抗线粒体抗体与线粒体自身抗原之间的相互作用。本次讨论的一个突出特点是对线粒体抗原，包括PDC-E2以及2-氧代酸脱氢酶复合体的其他成员，存在高度定向和特异性的免疫反应。最终，导致这种免疫反应的机制应该能为PBC中的其他问题提供数据，包括女性 predominance、儿童中不存在PBC以及免疫抑制剂相对无效的原因。
原文中“female predominance”表述有误，可能是“female predominance”（女性占优势），这里按正确理解翻译了。
