Neuberger J
Liver Unit, Queen Elizabeth Hospital, Birmingham, B15 2TH, UK.
Baillieres Best Pract Res Clin Gastroenterol. 2000 Aug;14(4):669-80. doi: 10.1053/bega.2000.0111.
Transplantation has become the accepted form of therapy for patients with end-stage liver disease. The diagnosis of recurrent disease in the allograft has been a matter of controversy, partly because of the difficulties in making the diagnosis in the allograft situation. The conventional criteria for diagnosing PBC may be inappropriate and there are many causes of bile duct damage in the graft. That the PBC-specific autoantibodies [such as antimitochondrial antibody (AMA) and gp-210] persist after transplantation is universally found, and some have reported the aberrant distribution of E2 in the allograft that is typical of PBC in the native liver, whether or not there is histological evidence of PBC recurrence. Most studies now accept that histological features of PBC, such as granulomatous bile duct damage, ductopenia and biliary-type fibrosis, may be found in the allograft; the histological features of PBC are variable and do not mirror the liver tests. The rate of recurrence increases with time, so that by 10 years, recurrence may be found in 30-50% of biopsies. There are no clear factors which identify those at risk of recurrence, but the pattern and degree of immunosuppression may be implicated. Cirrhosis has only rarely been reported. In the medium term, recurrence of PBC has little clinical impact. Ursodeoxycholic acid is used in some centres but there is no clear evidence for benefit.
移植已成为终末期肝病患者公认的治疗方式。同种异体移植中复发性疾病的诊断一直存在争议，部分原因是在同种异体移植情况下进行诊断存在困难。诊断原发性胆汁性胆管炎（PBC）的传统标准可能并不适用，且移植物中胆管损伤有多种原因。移植后普遍发现PBC特异性自身抗体（如抗线粒体抗体（AMA）和gp - 210）持续存在，一些人报告了同种异体移植中E2的异常分布，这是天然肝脏中PBC的典型表现，无论是否有PBC复发的组织学证据。现在大多数研究认为，同种异体移植中可能会出现PBC的组织学特征，如肉芽肿性胆管损伤、小胆管减少和胆汁型纤维化；PBC的组织学特征多变，与肝功能检查结果并不相符。复发率随时间增加，到10年时，30% - 50%的活检可能会发现复发。目前尚无明确因素可确定哪些人有复发风险，但免疫抑制的方式和程度可能与之有关。肝硬化的报道很少。从中期来看，PBC复发的临床影响较小。一些中心使用熊去氧胆酸，但尚无明确证据表明其有益。
