PDF(Pubmed)
Abstract：
Autoimmune liver diseases (AILDs) are a group of conditions where immune-mediated liver damage can lead to the need for transplantation. Collectively, they account for almost a quarter of all liver transplants. Outcomes in terms of graft and patient survival for all liver transplants have improved markedly over decades with improvements in patient selection, surgical techniques and longer-term care and this is also seen in patients with AILDs. The current five- and ten-year survival rates post-transplant in autoimmune disease are excellent, at 88% and 78%, respectively. A key factor in maintaining good outcomes post liver transplant for these autoimmune conditions is the immunosuppression strategy. These patients have increased the rates of rejection, and autoimmune conditions can all recur in the graft ranging from 12 to 60% depending on the population studied. Immunosuppressive regimens are centred on calcineurin inhibitors, often combined with low dose corticosteroids, with or without the addition of antimetabolite therapy. There is no clear evidence-based immunosuppressive regimen for these conditions, and a tailored approach balancing the individuals\' immunological profile against the risks of immunosuppression is often used. There are disease-specific considerations to optimised graft function including the role of ursodeoxycholic acid in both primary biliary cholangitis and primary sclerosing cholangitis and the role and timing of colectomy in primary sclerosing cholangitis in inflammatory bowel disease patients. However, unmet needs still exist in the management of AILDs post liver transplantation particularly in building the evidence base for optimal immunosuppression as well as mitigating the risk of recurrent disease.
摘要：
自身免疫性肝病（AILD）是一组免疫介导的肝损伤可能导致需要移植的疾病。总的来说，它们几乎占所有肝移植手术的四分之一。几十年来，随着患者选择的改善，所有肝移植的移植物和患者存活率方面的结果都有了显着改善。手术技术和长期护理，这也见于AILDs患者。目前自身免疫性疾病移植后5年和10年生存率非常好，88%和78%，分别。在这些自身免疫性疾病的肝移植后保持良好结果的关键因素是免疫抑制策略。这些患者的排斥反应率增加,和自身免疫性疾病都可以在移植物中复发，范围为12％至60％，具体取决于所研究的人群。免疫抑制方案以钙调磷酸酶抑制剂为中心，经常与低剂量皮质类固醇联合使用，有或没有添加抗代谢药物治疗。对于这些情况，没有明确的基于证据的免疫抑制方案，并且经常使用量身定制的方法来平衡个体的免疫学特征与免疫抑制的风险。优化移植物功能需要考虑特定疾病，包括熊去氧胆酸在原发性胆汁性胆管炎和原发性硬化性胆管炎中的作用，以及结肠切除术在炎症性肠病患者原发性硬化性胆管炎中的作用和时机。然而,肝移植后AILDs的管理仍存在未满足的需求,特别是在建立最佳免疫抑制的证据基础以及降低疾病复发的风险方面.
