Division of Rheumatology, Allergy, and Clinical Immunology, University of California, Davis, CA, USA.
J Autoimmun. 2012 Aug;39(1-2):34-42. doi: 10.1016/j.jaut.2011.11.005. Epub 2011 Dec 16.
Primary biliary cirrhosis (PBC) has been often coined a model autoimmune disease based on the homogeneity amongst patients, the frequency and similarity of antimitochondrial antibodies, including the highly directed immune response to pyruvate dehydrogenase (PDC-E2). A significant number of patients with PBC suffer from sicca and amongst these, there are patients who also have classic Sjögren's syndrome. Indeed, both PBC and Sjögren's syndrome are characterized by inflammation of target epithelial elements. Both diseases can be considered on the basis of a number of other related clinical aspects, including proposed unique apoptotic features of the target tissue, the role of secretory IgA, and the frequency with which both diseases overlap with each other. Indeed, PBC may be considered a Sjögren's syndrome of the liver, whereas Sjögren's syndrome can be equally discussed as PBC of the salivary glands. Dissection of the genetic predispositions for both diseases and especially the molecular basis of effector mechanisms, will become critical elements in developing new therapies.
原发性胆汁性肝硬化 (PBC) 常被称为自身免疫性疾病的模型，基于患者的同质性、抗线粒体抗体的频率和相似性，包括对丙酮酸脱氢酶 (PDC-E2) 的高度定向免疫反应。相当数量的 PBC 患者患有干燥症，其中有些患者也患有经典的干燥综合征。事实上，PBC 和干燥综合征都以靶上皮细胞的炎症为特征。这两种疾病可以根据许多其他相关的临床方面来考虑，包括靶组织独特的凋亡特征、分泌型 IgA 的作用，以及这两种疾病相互重叠的频率。事实上，PBC 可以被认为是肝脏的干燥综合征，而干燥综合征也可以被同样地讨论为唾液腺的 PBC。对这两种疾病的遗传易感性的剖析，特别是效应机制的分子基础，将成为开发新疗法的关键因素。
