Hübscher S G
Department of Pathology, University of Birmingham, Birmingham, UK.
Liver Transpl. 2001 Apr;7(4):285-91. doi: 10.1053/jlts.2001.23085.
Approximately 20% to 30% of patients undergoing liver transplantation for autoimmune hepatitis (AIH) develop features of recurrent disease. Diagnostic criteria for recurrent AIH are similar to those used in the nontransplanted liver and include, in varying combinations, biochemical, serological, and histological abnormalities and steroid dependency. However, these criteria are more difficult to apply in the liver allograft because of potential interactions between recurrent AIH and other complications of liver transplantation, particularly rejection, and the uncertain effects of long-term immunosuppression. In the absence of other reliable diagnostic markers, a number of studies have used the histological finding of chronic hepatitis as the main or sole criterion for diagnosing recurrent AIH. However, this also lacks diagnostic specificity because there are many other possible causes of chronic hepatitis in the liver allograft. In addition, approximately 20% to 40% of biopsies performed on patients as part of routine annual review have histological features of chronic hepatitis, for which no definite cause can be identified. Risk factors that have been associated with the development of recurrent AIH include suboptimal immunosuppression, HLA phenotype, disease type and severity in the native liver, and duration of follow up. In many cases in which recurrent AIH seems to be related to underimmunosuppression, biochemical and histological features rapidly resolve once adequate immunosuppression is restored. However, in other cases, recurrent AIH behaves more aggressively, with progression to cirrhosis and graft failure. Areas that require further study include developing uniform criteria for the diagnosis of recurrent AIH, identifying risk factors for severe recurrent disease, and determining optimal levels of immunosuppression that minimize the impact of disease recurrence without exposing patients to the risks of overimmunosuppression.
接受自身免疫性肝炎（AIH）肝移植的患者中，约20%至30%会出现疾病复发的特征。复发性AIH的诊断标准与非移植肝脏所使用的标准相似，不同组合包括生化、血清学和组织学异常以及对类固醇的依赖。然而，由于复发性AIH与肝移植的其他并发症（尤其是排斥反应）之间可能存在相互作用，以及长期免疫抑制的不确定影响，这些标准在肝移植中更难应用。在缺乏其他可靠诊断标志物的情况下，许多研究将慢性肝炎的组织学表现作为诊断复发性AIH的主要或唯一标准。然而，这也缺乏诊断特异性，因为肝移植中慢性肝炎还有许多其他可能的病因。此外，在作为常规年度复查一部分对患者进行的活检中，约20%至40%具有慢性肝炎的组织学特征，且无法确定明确病因。与复发性AIH发生相关的危险因素包括免疫抑制不足、HLA表型、原肝脏的疾病类型和严重程度以及随访时间。在许多复发性AIH似乎与免疫抑制不足相关的病例中，一旦恢复足够的免疫抑制，生化和组织学特征会迅速缓解。然而，在其他情况下，复发性AIH表现得更为侵袭性，会进展为肝硬化和移植肝衰竭。需要进一步研究的领域包括制定复发性AIH诊断的统一标准、确定严重复发性疾病的危险因素以及确定最佳免疫抑制水平，以在不使患者面临过度免疫抑制风险的情况下将疾病复发的影响降至最低。
