Department of Physiology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
Division of Liver Diseases, Department of Medicine, Icahn School of Medicine at Mount Sinai, Recanati-Miller Transplantation Institute, New York, NY, USA.
Clin Transplant. 2020 May;34(5):e13841. doi: 10.1111/ctr.13841. Epub 2020 Mar 6.
There are little data on the pre- and post-liver transplantation (LT) outcomes of patients having autoimmune hepatitis-primary biliary cholangitis (AIH-PBC), AIH-primary sclerosing cholangitis (AIH-PSC), and AIH-small-duct PSC (AIH-SDPSC). The aim of this study was to analyze pre- and post-LT outcomes and survival of patients having different overlap syndromes (OS) undergoing LT.
Patients with compatible clinical and pathologic features of AIH-PBC (n = 86), AIH-PSC (n = 22), and AIH-SDPSC (n = 9) were included in the study. Demographic, laboratory, clinical, and survival data were analyzed. Multivariable analyses were performed to determine factors predicting transplant-free survival.
AIH-primary sclerosing cholangitis patients were less treatment-responsive and were more likely to undergo LT than other OS. No survival difference was noted among the 3 groups. Liver decompensation was independently associated with higher mortality (HR 21.78; 95% CI 2.50-190.01). Thirteen patients with OS underwent LT. One-year survival post-LT was 91.7%. Overall recurrence rate for OS post-LT was 8%.
AIH-primary sclerosing cholangitis patients were more likely to require LT compared with patients having AIH-PBC. Transplant-free survival was similar among the three AIH-overlap syndromes. Allograft recurrence of OS occurred in about 10% of cases. Patients with OS appear to have good short- and medium-term post-LT outcomes in terms of graft function and overall survival.
关于自身免疫性肝炎-原发性胆汁性胆管炎（AIH-PBC）、自身免疫性肝炎-原发性硬化性胆管炎（AIH-PSC）和自身免疫性肝炎-小胆管PSC（AIH-SDPSC）患者在肝移植（LT）前后的结果，数据很少。本研究旨在分析不同重叠综合征（OS）患者接受 LT 前后的结果和生存情况。
研究纳入了符合 AIH-PBC（n=86）、AIH-PSC（n=22）和 AIH-SDPSC（n=9）临床和病理特征的患者。分析了人口统计学、实验室、临床和生存数据。进行了多变量分析以确定预测无移植生存的因素。
AIH-PSC 患者的治疗反应较差，比其他 OS 更有可能接受 LT。3 组之间没有生存差异。肝功能失代偿与更高的死亡率独立相关（HR 21.78；95%CI 2.50-190.01）。13 例 OS 患者接受了 LT。LT 后 1 年生存率为 91.7%。OS 患者 LT 后总体复发率为 8%。
与 AIH-PBC 患者相比，AIH-PSC 患者更有可能需要 LT。三种 AIH 重叠综合征之间的无移植生存率相似。OS 的同种异体移植复发发生在大约 10%的病例中。在移植物功能和总体生存率方面，OS 患者在 LT 后似乎具有良好的短期和中期结果。
