肝移植治疗终末期自身免疫性肝病的临床分析

Clinical analysis of liver transplantation in end-stage autoimmune liver diseases

目的分析肝移植治疗终末期自身免疫性肝病(AILD)患者的临床特征及远期预后。方法回顾性收集2004年1月至2018年8月在天津市第一中心医院肝移植科因终末期AILD接受肝移植的147例受者的临床资料,并对受者进行随访,随访截止日期为2019年3月1日。观察受者术后生存情况,绘制Kaplan-Meier生存曲线,计算累积存活率,并分析死亡原因;统计受者术后排斥反应、AILD复发、胆道并发症和肝炎病毒感染的发生率。结果147例AILD肝移植受者中,自身免疫性肝炎(AIH)25例,原发性胆汁性胆管炎(PBC)94例,AIH重叠PBC(AIH-PBC)14例,原发性硬化性胆管炎(PSC)14例;男性22例,女性125例,男女比例1∶5.7;年龄(53.2±10.1)岁;终末期肝病模型(MELD)评分(14.6±9.0)分。截至随访终点,147例受者死亡21例,存活126例。生存曲线分析显示,AILD肝移植受者术后1、3、5、10年累积存活率分别为98.6%、94.0%、84.6%、76.9%;死亡原因分析显示,主要死亡原因为移植肝失功,其次为移植肝功能衰竭和呼吸衰竭。147例受者中,17例(11.56%)术后发生排斥反应;19例(12.93%)AILD复发,其中2例PBC受者复发AIH-PBC;37例(25.17%)发生胆道并发症;19例(12.93%)感染肝炎病毒,其中乙型肝炎病毒(HBV)14例,丙型肝炎病毒(HCV)5例。结论终末期AILD患者以中老年女性为主,PBC最为常见;肝移植是终末期AILD的有效治疗手段,且远期预后良好,应重视术后免疫抑制剂的使用和复发问题。

Objective To explore the clinical characteristics and long-term prognosis of recipients with end-stage autoimmune liver diseases(AILD)after liver transplantation.Methods A retrospective study was conducted.The data of 147 end-stage autoimmune liver diseases recipients who underwent liver transplantation admitted to department of liver transplantation of Tianjin First Center Hospital from January 2004 to August 2018 were collected.Patients were followed till March 1st,2019.The postoperative survival was analyzed,the cumulative survival rate was estimated by the Kaplan-Meier survival curve,and the cause of death was analyzed.Postoperative rejection,recurrence of AILD,biliary complications,and hepatitis virus infection were also summarized.Results Among the 147 recipients,25 were autoimmune hepatitis(AIH),94 were primary biliary cholangitis(PBC),14 were AIH overlapping PBC(AIH-PBC),14 were primary sclerosing cholangitis(PSC).The 147 recipients were consisted of 22 males and 125 females,with a ratio of 1∶5.7,with a mean age of(53.2±10.1)years old.The average model for end-stage liver disease(MELD)score was 14.6±9.0.After a median follow-up of 49.0 months,21 recipients died,and 126 recipients survived.The survival curve analysis showed that the 1-,3-,5-and 10-year cumulative survival rate was 98.6%,94.0%,84.6%,and 76.9%,respectively.The main cause of death was graft dysfunction,followed by graft failure and respiratory failure.Among the 147 recipients,17(11.56%)rejection occurred,19 recurrence(12.93%)were diagnosed with liver biopsy,and of which 2 PBC recipients overlapped de novo AIH-PBC.Biliary complications happened in 37 recipients(25.17%).De novo hepatitis virus infection was found in 19 recipients(12.93%),including 14 with hepatitis B virus(HBV)and 5 with hepatitis C virus(HCV).Conclusions The majority patients of the end-stage of AILD were middle-aged and old women,and PBC was the most common.Liver transplantation is an effective treatment for end-stage autoimmune liver diseases with excellent prognosis.Postoperative immunosuppressant usage and recurrence should be emphasized.