Kelly Claire, Zen Yoh, Heneghan Michael A
Institute of Liver Studies, Kings College Hospital, London, UK.
J Clin Exp Hepatol. 2023 Mar-Apr;13(2):350-359. doi: 10.1016/j.jceh.2022.07.002. Epub 2022 Jul 12.
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.
自身免疫性肝病(AILDs)是一组免疫介导的肝损伤可能导致需要进行移植的疾病。总体而言,它们占所有肝移植的近四分之一。随着患者选择、手术技术和长期护理的改善,几十年来所有肝移植的移植物和患者生存率都有显著提高,这在自身免疫性肝病患者中也有体现。目前,自身免疫性疾病患者移植后的五年和十年生存率分别高达88%和78%,非常理想。对于这些自身免疫性疾病患者,维持肝移植后良好预后的一个关键因素是免疫抑制策略。这些患者的排斥反应发生率增加,并且自身免疫性疾病在移植物中都可能复发,复发率在12%至60%之间,具体取决于所研究的人群。免疫抑制方案以钙调神经磷酸酶抑制剂为核心,通常与低剂量皮质类固醇联合使用,可加用或不加用抗代谢物治疗。对于这些疾病,目前尚无明确的循证免疫抑制方案,通常采用根据个体免疫状况与免疫抑制风险相平衡的个体化方法。为优化移植物功能,存在一些特定疾病的考虑因素,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,以及在炎症性肠病患者的原发性硬化性胆管炎中结肠切除术的作用和时机。然而,在肝移植后自身免疫性肝病的管理方面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风险方面。