Gohlke F, Lohse A W, Dienes H P, Löhr H, Märker-Hermann E, Gerken G, Meyer zum Büschenfelde K H
1st Department of Medicine, Johannes Gutenberg University, Mainz, Germany.
J Hepatol. 1996 Jun;24(6):699-705. doi: 10.1016/s0168-8278(96)80266-2.
BACKGROUND/AIMS: Autoimmune hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis are chronic liver diseases with probable autoimmune background. Overlapping features have been described for primary biliary cirrhosis and autoimmune hepatitis. In contrast, there have been only a few case reports on an overlap of autoimmune hepatitis and primary sclerosing cholangitis.
We describe three male patients with clinical and histological overlapping features of primary sclerosing cholangitis and autoimmune hepatitis.
All initially asymptomatic patients had elevated levels of aminotransferases, alkaline phosphatase, gamma-glutamyltranspeptidase and IgG. Anti-nuclear antibodies and/or smooth muscle antibodies were positive and anti-neutrophil cytoplasmic antibodies were detected in all patients. Retrograde endoscopic cholangiography showed bile-duct strictures characteristic for primary sclerosing cholangitis. Histopathology showed necro-inflammatory activity of portal tracts with bridging necrosis in all patients at the time of first diagnosis. Aminotransferase levels and the necro-inflammatory activity responded well to immunosuppressive treatment. Predominant periductular fibrosis as a typical histopathological feature of primary sclerosing cirrhosis was seen to develop in all patients. Cholestatic serum parameters remained elevated and periductular fibrosis as endoscopic bile duct changes progressed despite immunosuppression.
We suggest that these patients present an overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis as they fulfill the diagnostic criteria for both conditions.
背景/目的：自身免疫性肝炎、原发性胆汁性肝硬化和原发性硬化性胆管炎是具有自身免疫背景的慢性肝病。原发性胆汁性肝硬化和自身免疫性肝炎存在重叠特征。相比之下，关于自身免疫性肝炎和原发性硬化性胆管炎重叠的病例报告较少。
我们描述了3例具有原发性硬化性胆管炎和自身免疫性肝炎临床及组织学重叠特征的男性患者。
所有最初无症状的患者转氨酶、碱性磷酸酶、γ-谷氨酰转肽酶和IgG水平均升高。所有患者抗核抗体和/或平滑肌抗体呈阳性，且均检测到抗中性粒细胞胞浆抗体。逆行内镜胆管造影显示原发性硬化性胆管炎特征性的胆管狭窄。首次诊断时，所有患者的组织病理学均显示门管区坏死性炎症活动伴桥接坏死。转氨酶水平和坏死性炎症活动对免疫抑制治疗反应良好。所有患者均出现了作为原发性硬化性胆管炎典型组织病理学特征的主要的胆管周围纤维化。尽管进行了免疫抑制治疗，但胆汁淤积血清参数仍升高，且随着内镜下胆管改变进展，胆管周围纤维化仍在发展。
我们认为这些患者符合自身免疫性肝炎和原发性硬化性胆管炎的诊断标准，表现为这两种疾病的重叠综合征。
