Dienes H P
Institut für Pathologie des Universitätsklinikums Mainz.
Verh Dtsch Ges Pathol. 1995;79:177-85.
The morphology of autoimmune hepatitis is characterized by portal-periportal predilection of necroinflammatory lesions. In comparison to the viral type of hepatitis severe piece-meal-necroses, the collapse of periportal parenchyma, and to a higher degree acinar transformation of hepatocytes are more prominent. The autoimmune hepatitis may start with acute disease displaying unusual clinical und histopathologic features. The postinfantile giant cell hepatitis seems to constitute a variant of autoimmune hepatitis. Autoimmune hepatitis has been reproduced in animal models and it could be demonstrated in rabbits that humoral immunity plays a role in tissue damage. The importance of cellular mechanisms could be analyzed in syngenic mice showing that the CD4-positive lymphocytes play a pivotal role. The most promising candidate antigen seems to be the asialoglycoprotein-receptors including the liver specific protein (LSP). By immunohistologic analysis dense deposits of IgG could be demonstrated in sinusoids and on the membranes of hepatocytes. In accordance with in vitro data the determination of CD4 positive lymphocytes in the tissue was found to play a decisive role in cellular immune reaction. The HSP65 molecule seems to evoke mechanisms that have been shown to play a pathogenetic role in experimental arthritis.
自身免疫性肝炎的形态学特征为坏死性炎症病变以门脉周围为主。与病毒性肝炎的严重桥接坏死、门脉周围实质塌陷相比,肝细胞腺泡样转化程度更高且更为突出。自身免疫性肝炎可能起病为急性疾病,表现出不寻常的临床和组织病理学特征。婴儿期后巨细胞肝炎似乎是自身免疫性肝炎的一种变体。自身免疫性肝炎已在动物模型中再现,并且在兔子身上已证实体液免疫在组织损伤中起作用。细胞机制的重要性可在同基因小鼠中进行分析,结果表明CD4阳性淋巴细胞起关键作用。最有希望的候选抗原似乎是去唾液酸糖蛋白受体,包括肝特异性蛋白(LSP)。通过免疫组织学分析,可在肝血窦和肝细胞细胞膜上证实IgG的密集沉积。与体外数据一致,发现组织中CD4阳性淋巴细胞的测定在细胞免疫反应中起决定性作用。HSP65分子似乎引发了一些机制,这些机制已被证明在实验性关节炎中起致病作用。