Luettig B, Boeker K H, Schoessler W, Will H, Loges S, Schmidt E, Worman H J, Gershwin M E, Manns M P
Department of Gastroenterology and Hepatology, Hannover Medical School, Germany.
J Hepatol. 1998 May;28(5):824-8. doi: 10.1016/s0168-8278(98)80233-x.
BACKGROUND/AIMS: Primary biliary cirrhosis is an autoimmune liver disease which is characterized by the presence of autoantibodies directed against mitochondrial components which belong to the pyruvate dehydrogenase enzyme complex. Apart from antibodies against mitochondrial components, primary biliary cirrhosis patients often show antibodies against nuclear components, of which anti-Sp100 and anti-gp210 are considered to be disease specific. We investigated the incidence and course of antibodies against nuclear components in primary biliary cirrhosis patients before and after liver transplantation.
Sera from 42 primary biliary cirrhosis patients were studied using indirect immunofluorescence to detect antibodies against mitochondrial components and antibodies against nuclear components, ELISA to detect anti-Sp100, and immunoblot analysis to detect anti-gp210 and antibodies against nuclear components subtypes.
Ninety-three percent of primary biliary cirrhosis patients in our study were antimitochondrial antibody positive. Forty-three percent of the patients were antinuclear antibody positive. Of these, 35% had antibodies against Sp100 and 36% were positive for anti-gp210. After transplantation, antimitochondrial antibody titers as well as antinuclear antibody titers decreased in all patients. Autoantibodies in low titer persisted for up to 13 years. The pattern of nuclear autoantigens recognized by patient sera was unchanged after liver transplantation. However, the antinuclear antibody pattern was very different between the individual patients. Anti-Sp100 and anti-gp210 were not detected in sera of patients with autoimmune hepatitis, hepatitis C infection, inflammatory bowel disease, connective tissue diseases, or primary sclerosing cholangitis. The serum alkaline phosphatase level was not different in antinuclear antibody negative or positive patients before or after transplantation.
We conclude that the persistence of antibodies against mitochondrial components, and anti-Sp100 and anti-gp210 in primary biliary cirrhosis patients after liver transplantation is disease specific, but that this does not reflect recurrent disease activity in the graft.
背景/目的：原发性胆汁性肝硬化是一种自身免疫性肝病，其特征是存在针对属于丙酮酸脱氢酶复合体的线粒体成分的自身抗体。除了抗线粒体成分抗体外，原发性胆汁性肝硬化患者常表现出抗核成分抗体，其中抗Sp100和抗gp210被认为是疾病特异性的。我们研究了肝移植前后原发性胆汁性肝硬化患者抗核成分抗体的发生率及病程。
采用间接免疫荧光法检测42例原发性胆汁性肝硬化患者血清中的抗线粒体成分抗体和抗核成分抗体，酶联免疫吸附测定法检测抗Sp100，免疫印迹分析法检测抗gp210及抗核成分亚型抗体。
本研究中93%的原发性胆汁性肝硬化患者抗线粒体抗体呈阳性。43%的患者抗核抗体呈阳性。其中，35%有抗Sp100抗体，36%抗gp210呈阳性。移植后，所有患者的抗线粒体抗体滴度和抗核抗体滴度均下降。低滴度自身抗体可持续长达13年。肝移植后患者血清识别的核自身抗原模式未改变。然而，个体患者之间的抗核抗体模式差异很大。在自身免疫性肝炎、丙型肝炎感染、炎症性肠病、结缔组织病或原发性硬化性胆管炎患者的血清中未检测到抗Sp100和抗gp210。移植前后抗核抗体阴性或阳性患者的血清碱性磷酸酶水平无差异。
我们得出结论，原发性胆汁性肝硬化患者肝移植后抗线粒体成分抗体以及抗Sp100和抗gp210的持续存在具有疾病特异性，但这并不反映移植肝的复发疾病活动。
