Professor of Gastroenterology, University of Crete Medical School, Head, Department Of Gastroenterology, University Hospital, PO Box 1352, 71100 Heraklion, Crete, Greece. kouroum@meduocgr
Dis Markers. 2010;29(6):329-36. doi: 10.3233/DMA-2010-0769.
Primary biliary cirrhosis (PBC) is an immune mediated chronic cholestatic liver disease with a slowly progressive course It is a universal disease with a reported latitudinal gradient in prevalence and incidence. The aetiology of primary biliary cirrhosis is still unknown. It is characterized by a 60% concordance in monozygotic twins and is considered an autoimmune disease because of several features common to other autoimmune conditions and the relatively homogeneous serological and biochemical features. However geoepidemiological and clinical studies strongly imply that environmental factors also play an important role. It is accepted that the disease is clearly the result of a combination of genetic and environmental factors. Several risk factors have been suggested to be associated with PBC, including exposure to infectious agents and chemical xenobiotics. This review will attempt to place such factors in perspective.
原发性胆汁性肝硬化（PBC）是一种免疫介导的慢性胆汁淤积性肝病，具有缓慢进展的病程。它是一种普遍存在的疾病，其发病率和患病率呈纬度梯度分布。原发性胆汁性肝硬化的病因尚不清楚。它的特征是同卵双胞胎中有 60%的一致性，并且由于与其他自身免疫性疾病共同的几个特征以及相对同质的血清学和生化特征，被认为是一种自身免疫性疾病。然而，地理流行病学和临床研究强烈表明，环境因素也起着重要作用。人们普遍认为，这种疾病显然是遗传和环境因素共同作用的结果。已经有一些危险因素被认为与 PBC 有关，包括接触感染因子和化学异源物质。这篇综述将尝试从不同角度来探讨这些因素。
