Mackay I R, Gershwin M E
Centre for Molecular Biology and Medicine, Monash University, Clayton Victoria, Australia.
Semin Liver Dis. 1997 Feb;17(1):3-11. doi: 10.1055/s-2007-1007178.
The primary function of the immune system is protection and defense against infection. However, the maintenance of normal immune function is a very intricate process, and the failure of these processes to establish and maintain tolerance to self results in autoimmunity. The damage that results from autoimmune disease can be extensive and can be directed at any tissue in the body. One of the major issues in autoimmunity is whether this is a natural or an unnatural phenomenon. Although there have been numerous and exciting theories proposed to explain the autoimmune response, the basic underlying risk factors remain unclear. Both genetic and environmental factors have been incriminated, but the highly variable expression of autoimmune disease and the multiple genetic and environmental determinants make conclusions difficult. Primary biliary cirrhosis is considered a model autoimmune disease because it reflects a highly directed and highly specific response to an identified target, the biliary epithelium. In this review we attempt to place these issues in perspective both from the generic perspective of autoimmunity and from the specific perspective of primary biliary cirrhosis.
免疫系统的主要功能是保护机体并抵御感染。然而，维持正常免疫功能是一个非常复杂的过程，这些过程若无法建立并维持对自身的耐受性，就会导致自身免疫性疾病。自身免疫性疾病所造成的损害可能是广泛的，可累及身体的任何组织。自身免疫性疾病的一个主要问题是，这是一种自然现象还是非自然现象。尽管已经提出了许多令人兴奋的理论来解释自身免疫反应，但基本的潜在风险因素仍不明确。遗传因素和环境因素都被认为与自身免疫性疾病有关，但自身免疫性疾病的高度可变表达以及多种遗传和环境决定因素使得得出结论很困难。原发性胆汁性肝硬化被视为一种典型的自身免疫性疾病，因为它反映了对已确定的靶标——胆管上皮细胞——的高度定向和高度特异性反应。在这篇综述中，我们试图从自身免疫性疾病的一般角度以及原发性胆汁性肝硬化的具体角度来审视这些问题。
