Division of Gastroenterology, Hepatology and Nutrition, Golisano Childrens Hospital at Strong, University of Rochester Medical Center, 601 Elmwood Avenue, Box 667 Rochester, New York 14642 USA.
Institute of Liver Studies, Mowat Labs, King's College Hospital, London, United Kingdom.
J Autoimmun. 2018 Dec;95:26-33. doi: 10.1016/j.jaut.2018.10.023. Epub 2018 Nov 3.
De novo autoimmune hepatitis (AIH) is an unusual cause of graft dysfunction after liver transplantation. This entity was originally described in 1996 in children transplanted for conditions other than AIH, who developed biochemical and histological features similar to AIH and responded to the therapy of classical AIH with steroids and azathioprine. In the last two decades, there have been reports of occurrence of de novo AIH in pediatric and adult liver transplant recipients, in the latter often being given different nomenclature including 'plasma cell hepatitis'. Typical causes of graft dysfunction in liver transplant recipients include rejection, infection, vascular and biliary complications as well as recurrence of disease that was the indication for liver transplantation like hepatitis C and primary biliary cholangitis. While acute cellular rejection and chronic rejection are well recognized complications post liver transplantation, in the last 5 years, antibody mediated rejection has become increasingly important in liver transplantation. In 2016, in the course of developing guidelines for the diagnosis of antibody mediated rejection, it was suggested that both de novo AIH and 'plasma cell hepatitis' be categorized as 'plasma cell rejection'. This review explores the literature on de novo AIH in pediatrics and adults, sheds light on the substantive differences between these two entities and suggests that they be kept distinct from each other as the two are not the same. This difference in the cause of graft dysfunction in pediatric and adult liver transplant recipients is important as the management of the two conditions is not the same.
原发性自身免疫性肝炎(AIH)是肝移植后移植物功能障碍的一种不常见原因。这种疾病最初于 1996 年在因非 AIH 疾病而接受肝移植的儿童中被描述,这些儿童出现了类似于 AIH 的生化和组织学特征,并对类固醇和硫唑嘌呤等经典 AIH 治疗有反应。在过去的二十年中,已有关于儿童和成人肝移植受者发生原发性 AIH 的报道,后者常被给予不同的命名,包括“浆细胞肝炎”。肝移植受者移植物功能障碍的典型原因包括排斥反应、感染、血管和胆道并发症以及导致肝移植的疾病(如丙型肝炎和原发性胆汁性胆管炎)的复发。虽然急性细胞排斥反应和慢性排斥反应是肝移植后的公认并发症,但在过去 5 年中,抗体介导的排斥反应在肝移植中变得越来越重要。2016 年,在制定抗体介导排斥反应诊断指南的过程中,建议将原发性 AIH 和“浆细胞肝炎”都归类为“浆细胞排斥反应”。本综述探讨了儿科和成人原发性 AIH 的文献,阐明了这两种疾病之间的实质性差异,并建议将它们彼此区分开来,因为它们并不相同。儿科和成人肝移植受者中导致移植物功能障碍的原因不同,因为这两种情况的处理方法不同。