He Xiao-Song, Ansari Aftab A, Ridgway William M, Coppel Ross L, Gershwin M Eric
Division of Rheumatology, Allergy and Clinical Immunology, School of Medicine, University of California, Davis, USA.
Cell Immunol. 2006 Jan;239(1):1-13. doi: 10.1016/j.cellimm.2006.04.006.
Primary biliary cirrhosis (PBC) is an autoimmune liver disease with profound changes in different compartments of the immune system, including those involved in innate, and adaptive immunity. New data from epidemiological studies of PBC have reinforced the thesis that the cause for this relatively uncommon disease is likely to be a combination of both environmental factors and a susceptible genetic predisposition. Recent findings of abnormalities of the innate immune system in PBC suggest that they may serve as links between the environmental factors and the early events in PBC development. Viral and bacterial infections as well as xenobiotics are some of the potential environmental factors that have been implicated in this complex process. Identification of the etiological factors for PBC will point to new preventive or therapeutic treatments.
原发性胆汁性肝硬化（PBC）是一种自身免疫性肝病，免疫系统的不同组成部分会发生深刻变化，包括那些参与固有免疫和适应性免疫的部分。PBC流行病学研究的新数据强化了这样一种观点，即这种相对罕见疾病的病因可能是环境因素和易感遗传倾向共同作用的结果。PBC中固有免疫系统异常的最新发现表明，它们可能是环境因素与PBC发展早期事件之间的联系。病毒和细菌感染以及外源性物质是这一复杂过程中涉及的一些潜在环境因素。确定PBC的病因将指向新的预防或治疗方法。
