Ben-Ari Z, Dhillon A P, Sherlock S
Department of Medicine, Royal Free Hospital School of Medicine, London, United Kingdom.
Hepatology. 1993 Jul;18(1):10-5.
We describe four patients with features overlapping those of primary biliary cirrhosis and autoimmune chronic active hepatitis. Three were female and one was male; only one was symptomatic. Serum biochemical study showed increases in alkaline phosphatase and alpha-glutamyltranspeptidase levels. Markers of hepatitis B and C viruses were absent. In all four patients, serum mitochondrial antibodies could not be detected on immunofluorescence study and serum M2 antibodies were absent. All four patients had high titers of serum antinuclear antibody of diffuse type. Serum actin antibodies were detected in all four patients. Liver biopsy specimens showed histological features of primary biliary cirrhosis, with marked cellular infiltration of the portal areas and bile duct damage. Intralobular inflammation and piecemeal necrosis were mild. Three patients were treated with prednisolone and showed rapid clinical and biochemical remission. Serial liver biopsy specimens showed reduced inflammation, but bile duct lesions persisted. These patients probably form a subgroup of autoimmune chronic active type 1 with predominant bile duct damage. The subgroup might be termed autoimmune cholangiopathy.
我们描述了四名患者，其特征与原发性胆汁性肝硬化和自身免疫性慢性活动性肝炎重叠。三名女性，一名男性；仅一名有症状。血清生化研究显示碱性磷酸酶和α-谷氨酰转肽酶水平升高。未检测到乙型和丙型肝炎病毒标志物。在所有四名患者中，免疫荧光研究未检测到血清线粒体抗体，且无血清M2抗体。所有四名患者均有高滴度的弥漫型血清抗核抗体。所有四名患者均检测到血清肌动蛋白抗体。肝活检标本显示原发性胆汁性肝硬化的组织学特征，门脉区有明显的细胞浸润和胆管损伤。小叶内炎症和桥接坏死较轻。三名患者接受泼尼松龙治疗，临床和生化指标迅速缓解。系列肝活检标本显示炎症减轻，但胆管病变持续存在。这些患者可能构成以胆管损伤为主的自身免疫性慢性活动性1型的一个亚组。该亚组可称为自身免疫性胆管病。
