原位肝移植治疗终末期自身免疫性肝病

Orthotopic liver transplantation for the treatment of end-stage autoimmune liver disease

目的评价肝移植治疗终末期自身免疫性肝病的疗效并总结临床经验。方法回顾性分析2003年9月至2009年7月问因终末期自身免疫性肝病接受肝移植手术的11例患者的临床资料，其中8例为原发性胆汁性肝硬化，2例为自身免疫性肝炎，1例为原发性硬化性胆管炎。平均年龄为（44．2±8．7）岁。手术方式均采用附加腔静脉整形的改良背驮式肝移植术。术后免疫抑制治疗采用他克莫司或环孢素A联合激素的二联免疫抑制方案，部分患者使用骁悉和熊去氧胆酸。结果本组11例患者中2例原发性胆汁性肝硬化患者死亡，其中1例于术后第5天死于肺部感染和多器官功能衰竭，另1例于术后964d死于脓毒症和移植肝失功。5例患者术后1个月内出现急性排斥反应，加强免疫抑制治疗后痊愈。9例患者生存良好并存活至今，随访期7-62个月，中位随访时间为38个月。受体1年存活率为9l％，3年存活率为82％，最长存活期5年。随访期间未发现复发病例。结论肝移植是治疗终末期自身免疫性肝病的惟一有效手段，手术时机的正确把握和有效的免疫抑制治疗是减少肝移植术后并发症的关键。

Objective To evaluate the effect of liver transplantation for end-stage autoimmune liver disease （ESALD） and summarize the clinical experience of liver transplantation in the treatment of ESALD. Methods The clinical data of 11 ESALD cases who underwent liver transplantation from September 2003 to July 2009 were analyzed retrospectively. There were 2 males and 9 females （ median age, 44. 2 ±8.7 years）. The indication of liver transplantation was end stage of primary biliary cirrhrosis （8 cases）, autoimmune hepatitis （2 cases）, and primary sclerosing cholangitis （1 case）. In all cases, modified piggyback liver transplantation with venacavaplasty was carried out. Postoperatively all patients were treated with immunosuppressive agents including tacrolimus （or eyclosporine A） and prednisone, some patients were treated additionally with mycophenolate mofetil and ursodeoxycholie acid. Results Postoperatively 2 patients of primary biliary cirrhosis died, one of lung infection and multiple organ failure on the 5th postoperative day, the other dying of sepsis and graft dysfunction on the 964th postoperative day. Five cases suffered from episodes of acute cellular rejection within 1 month after transplantation and was successfully reversed by strengthened immunosuppressive therapy. Nine patients recovered satisfactorily and with excellent life quality until now. Patients were followed up from 7 months to 62 months with the median follow-up time of 38 months. The recipient survival rate at 1 year and 3 years was 91% and 82%, respectively. One patient has now survived for 5 years. No recurrent ALD case was found during follow up. Conclusions Orthotopic liver transplantation is an exclusive treatment for ESALD. Optimum operation timing and effective immunosuppressive treatment are very important for decreasing occurrence of complications.