Autoimmune hepatitis--an update.
Autoimmune hepatitis--an update.
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发表时间：
1992-04
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通讯作者：
K. M. Z. Büschenfelde;T. Poralla
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作者：
K. M. Z. Büschenfelde;T. Poralla
自身免疫性慢性活动性肝炎（ai-CAH）是一种独特的形式的进行性炎症性肝病，迄今病因不明。如果不治疗，预后不良，因此应尽快诊断。通过建立一组标记自身抗体来定义该疾病的几个亚组，极大地促进了诊断方法。此外，这些自身抗体的靶点的表征为疾病的发病机制提供了新的见解，即使触发疾病的主要事件仍有待确定。虽然上述标记自身抗体针对细胞内结构，但可以检测到对肝细胞表面的器官特异性组分，特别是脱唾液酸糖蛋白受体（ASGPR）的额外免疫反应（细胞和体液）。可以假定这些后一种反应在疾病的开始和/或持续中起作用。基于联合免疫抑制的治疗策略可以显著改善症状并延长预期寿命。如果他们失败了，肝移植提供了一个非常有希望的前景。
Autoimmune chronic active hepatitis (ai-CAH) is a distinct form of progressive inflammatory liver diseases of so far unknown etiology. If untreated it carries an unfavourable prognosis, therefore the diagnosis should be made as soon as possible. The diagnostic approach has been greatly facilitated by the establishment of a panel of marker autoantibodies that define several subgroups of the disease. Moreover the characterization of the targets of these autoantibodies shed new insight into the pathogenesis of the disease, even though the primary event triggering the disease remains to be defined. While the marker autoantibodies mentioned above are directed to intracellular structures additional immune reactions (both cellular and humoral) could be detected to organ specific components of the liver cell surface especially the asialoglycoprotein receptor (ASGPR). These latter reactions can be assumed to play a role in the initiation and/or perpetuation of the disease. Therapeutic strategies based on combined immunosuppression can considerably improve symptoms and prolong life expectancy. If they fail liver transplantation offers a very promising perspective.