Foroncewicz B, Mucha K, Paczek L, Oldakowska-Jedynak U, Górnicka B, Zieniewicz K, Nyckowski P, Krawczyk M
Department of Immunology, Transplant Medicine, and Internal Diseases, Medical University of Warsaw, Poland.
Transplant Proc. 2003 Sep;35(6):2310-2. doi: 10.1016/s0041-1345(03)00835-2.
Primary biliary cirrhosis (PBC) is an immune-mediated disorder of unknown cause characterized by progressive destruction of intrahepatic bile ducts and the presence of antimitochondrial antibodies. There is no known cure for PBC, and treatment generally includes various combinations of ursodeoxycholic acid and immunosuppressive agents. However, in most patients with end-stage PBC, liver transplantation offers a good quality of life. Recurrent PBC after transplantation is controversial, because most patients with suspected recurrent disease are asymptomatic. Antimitochondrial antibodies frequently persist and do not correlate with disease recurrence. However, most studies support disease recurrence within the graft. The effects of immunosuppression may modify or delay disease expression within the graft. If PBC recurs, intermediate-term patient and graft survivals are excellent, but the long-term outcome remains unknown. Many immunosuppressive agents have been studied with regard to their anti-recurrence properties; however, no standard therapy has been established for this group of patients. In this study we present two patients transplanted for PBC who displayed early recurrence of disease confirmed by liver biopsy and elevated serum AMA. Both individuals received the same immunosuppressive regimen. The data suggest that two doses of daclizumab and tacrolimus monotherapy in the early posttransplant period is insufficient to prevent recurrence of PBC. Addition of glucocorticoids may have beneficial effects in these patients.
原发性胆汁性肝硬化（PBC）是一种病因不明的免疫介导性疾病，其特征为肝内胆管进行性破坏以及存在抗线粒体抗体。目前尚无已知的PBC治愈方法，治疗通常包括熊去氧胆酸和免疫抑制剂的各种联合应用。然而，对于大多数终末期PBC患者而言，肝移植可提供良好的生活质量。移植后复发性PBC存在争议，因为大多数疑似疾病复发的患者并无症状。抗线粒体抗体常常持续存在，且与疾病复发无关。然而，大多数研究支持移植物内疾病复发。免疫抑制的效果可能会改变或延迟移植物内疾病的表现。如果PBC复发，中期患者和移植物存活率良好，但长期结局仍未知。许多免疫抑制剂已就其抗复发特性进行了研究；然而，尚未为这类患者确立标准治疗方案。在本研究中，我们报告了两名因PBC接受移植的患者，他们经肝活检和血清AMA升高证实出现了疾病早期复发。两人均接受了相同的免疫抑制方案。数据表明，移植后早期给予两剂达利珠单抗和他克莫司单药治疗不足以预防PBC复发。添加糖皮质激素可能对这些患者有有益作用。
