Mackay I R
Centre for Molecular Biology and Medicine, Monash University, Clayton, Victoria, Australia.
J Gastroenterol Hepatol. 1990 May-Jun;5(3):352-9. doi: 10.1111/j.1440-1746.1990.tb01637.x.
The concept of auto-immune hepatitis as a disease entity evolved from the descriptions of 'chronic active hepatitis' (CAH) in the 1950s. Several types of CAH are distinguished by disease-specific features. The distinctive (but not exclusive) markers for auto-immune CAH include: a negative test for HBsAg; female; Northern European ethnic background; multisystem disease expression; histological CAH with large areas of periportal piecemeal necrosis and plasmacytosis; pronounced hypergammaglobulinaemia; serum auto-antibodies the HLA B8-DR3 phenotype; responsiveness to corticosteroid therapy; and rarity of supervening hepatocellular carcinoma. Much weight is attached to the serological marker auto-antibodies to nuclear or smooth muscle (actin) antigens (ANA, SMA). However, these auto-antibodies do not have an absolute association with auto-immune CAH: the serological reactions are not yet standardized; titres decrease with remission of disease; and other auto-antibodies mark variant forms of auto-immune hepatitis. A more confident acceptance of auto-immune hepatitis as an entity requires detection of a liver-specific antigen, a valid experimental disease model in animals, and a better understanding of immune-mediated damage to liver cells.
自身免疫性肝炎作为一种疾病实体的概念是从20世纪50年代对“慢性活动性肝炎”（CAH）的描述发展而来的。几种类型的CAH通过疾病特异性特征加以区分。自身免疫性CAH的独特（但并非唯一）标志物包括：乙肝表面抗原检测阴性；女性；北欧种族背景；多系统疾病表现；组织学上为伴有大片汇管区碎屑样坏死和浆细胞增多的CAH；显著的高球蛋白血症；血清自身抗体、HLA B8 - DR3表型；对皮质类固醇治疗有反应；以及极少并发肝细胞癌。血清学标志物，即针对核抗原或平滑肌（肌动蛋白）抗原的自身抗体（抗核抗体、平滑肌抗体）受到很大重视。然而，这些自身抗体与自身免疫性CAH并非绝对相关：血清学反应尚未标准化；滴度随疾病缓解而降低；并且其他自身抗体标志着自身免疫性肝炎的不同变体形式。要更有把握地将自身免疫性肝炎认定为一种实体疾病，需要检测肝脏特异性抗原、建立有效的动物实验疾病模型，以及更好地理解免疫介导的肝细胞损伤。
