自身免疫性肝病肝移植的临床分析。

Clinical analysis of liver transplantation in autoimmune liver diseases.

机构信息

Department of Liver Surgery, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China.

出版信息

Hepatobiliary Pancreat Dis Int. 2018 Feb;17(1):27-31. doi: 10.1016/j.hbpd.2018.01.001. Epub 2018 Jan 9.

DOI:10.1016/j.hbpd.2018.01.001

PMID:29428100

Abstract

BACKGROUND

Autoimmune liver diseases (ALDs) consist of autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), IgG4-associated cholangitis and overlap syndromes. Patients with these diseases may gradually progress to end-stage liver diseases and need liver transplantation. The present study aimed to explore the prognosis of patients with ALDs after liver transplantation.

METHODS

The clinical data of 80 patients with ALD (24 cases of AIH, 35 of PBC, 15 of PSC and 6 of AIH-PBC overlap syndromes) who underwent liver transplantation in Renji Hospital, Shanghai Jiao Tong University School of Medicine from June 2004 to September 2016 were collected retrospectively. The causes of death were analyzed and the postoperative cumulative survival rate was estimated by the Kaplan-Meier method. Recurrence and other complications were also analyzed.

RESULTS

Of the 80 patients, 18 were males and 62 were females. The average age was 50.5 years and the average Model for End-stage Liver Disease (MELD) score was 14.1. After a median follow-up of 19.8 months, 8 patients died. The 1-, 3- and 5-year cumulative survival rates were all 89.0%. Three cases of recurrent ALDs were diagnosed (3.8%) but they were not totally consistent with primary diseases. Biliary tract complication occurred in 10 patients (12.5%). The new onset of tumor was observed in 1 patient (1.3%). De novo HBV/CMV/EBV infection was found in 3, 8 and 3 patients, respectively.

CONCLUSION

Liver transplantation is an effective and safe treatment for end-stage ALD.

摘要

背景

自身免疫性肝病（ALD）包括自身免疫性肝炎（AIH）、原发性胆汁性肝硬化（PBC）、原发性硬化性胆管炎（PSC）、IgG4 相关胆管炎和重叠综合征。这些疾病的患者可能逐渐进展为终末期肝病并需要进行肝移植。本研究旨在探讨 ALD 患者肝移植后的预后。

方法

回顾性收集 2004 年 6 月至 2016 年 9 月在上海交通大学医学院附属仁济医院接受肝移植的 80 例 ALD 患者（AIH 24 例、PBC 35 例、PSC 15 例和 AIH-PBC 重叠综合征 6 例）的临床资料。分析死因并采用 Kaplan-Meier 法估计术后累积生存率。还分析了复发和其他并发症。

结果

80 例患者中，男 18 例，女 62 例。平均年龄为 50.5 岁，平均终末期肝病模型（MELD）评分 14.1。中位随访 19.8 个月后，8 例患者死亡。1、3 和 5 年累积生存率均为 89.0%。诊断出 3 例复发性 ALD（3.8%），但与原发性疾病不完全一致。10 例患者发生胆道并发症（12.5%）。1 例患者新发肿瘤（1.3%）。3、8 和 3 例患者分别新发乙型肝炎病毒/巨细胞病毒/EB 病毒感染。