肝移植治疗终末期自身免疫性肝病的预后分析

A retrospective analysis of liver transplantation in treating endstage autoimmune liver disease

目的研究终末期自身免疫性肝病患者肝移植预后情况。方法收集、整理及分析2001年4月至2015年3月45例因终末期自身免疫性肝病接受肝移植的受者临床资料，分析术后受者的生存情况及死亡原因，总结术后排斥反应发生情况、自身免疫性肝病复发情况及新发乙型肝炎病毒（HBV）感染情况。结果随访期间，45例受者中死亡12例，存活33例，术后1、3、5年受者累积存活率分别为86．3％、78．8％和78．8％。12例死亡受者的死亡原因包括移植肝功能衰竭4例，多器官功能衰竭5例，出血2例，肺部感染1例。45例受者中，术后共发生排斥反应6例，发生率为13．3％，1例发生急性排斥反应经激素冲击治疗后肝功能恢复正常，其余5例均为慢性排斥反应，均给予了保守治疗，3例治疗无效后死亡。1例在术后8年后出现新发HBV感染，予以积极抗病毒治疗后病情稳定。术后出现自身免疫性肝病复发2例，1例为PBC，另1例为考虑重叠综合征，2例受者均通过保守治疗后长期存活。结论肝移植是治疗终末期自身免疫性肝病有效的治疗手段，预后良好；需关注术后免疫抑制方案的应用，预防移植后并发症的出现，监测自身免疫性肝病的复发。

Objective Investigate the prognosis of patients undergoing liver transplantation （LT） for end-stage autoimmune liver disease （ALD）. Method The clinical data of 45 patients with endstage ALD undergoing LT from April 2001 to March 2015 in the first affiliated hospital of Zhejiang University were analyzed retrospectively. The postoperative cumulative survival rate of the recipients was calculated, and the causes of death were analyzed. The postoperative rejections, new onset viral hepatitis and ALD recurrence were also analyzed. Result In 45 ALD recipients, 33 cases survived and the postoperative 5-year cumulative survival rate was 78. 8%. Causes for 12 dead cases were multiple organ failure, liver graft failure, respiratory complications, hemorrhage and hepatic artery embolization. In 45 ALD recipients, 6 cases suffered rejection after operation with the incidence bing 13. 3%. One case suffered new onset hepatitis B infection 8 years after operation. One recipient suffered primary disease （primary biliary cirrhosis） recurrence 2 years after operation, and 1 recipient with primary disease （primary sclerosing cholangitis） developed into overlap syndrome. They all survived for a long term after active treatments. Conclusion Most LT recipients with endstage ALD can obtain a long term survival. Attentions should he paid to the immunosuppressive regimens in early period after LT, prevention of infection, rejection and postoperative new onset viral hepatitis, and timely diagnosis of primary disease recurrence.