Abstract：
OBJECTIVE: Recurrent primary biliary cholangitis (rPBC) develops in approximately 30% of patients and negatively impacts graft and overall patient survival after liver transplantation (LT). There is a lack of data regarding the response rate to ursodeoxycholic acid (UDCA) in rPBC. We evaluated a large, international, multi-center cohort to assess the performance of PBC scores in predicting the risk of graft and overall survival after LT in patients with rPBC.
METHODS: A total of 332 patients with rPBC after LT were evaluated from 28 centers across Europe, North and South America. The median age at the time of rPBC was 58.0 years [IQR 53.2-62.6], and 298 patients (90%) were female. The biochemical response was measured with serum levels of alkaline phosphatase (ALP) and bilirubin, and Paris-2, GLOBE and UK-PBC scores at 1 year after UDCA initiation.
RESULTS: During a median follow-up of 8.7 years [IQR 4.3-12.9] after rPBC diagnosis, 52 patients (16%) had graft loss and 103 (31%) died. After 1 year of UDCA initiation the histological stage at rPBC (hazard ratio [HR] 3.97, 95% CI 1.36-11.55, p = 0.01), use of prednisone (HR 3.18, 95% CI 1.04-9.73, p = 0.04), ALP xULN (HR 1.59, 95% CI 1.26-2.01, p <0.001), Paris-2 criteria (HR 4.14, 95% CI 1.57-10.92, p = 0.004), GLOBE score (HR 2.82, 95% CI 1.71-4.66, p <0.001), and the UK-PBC score (HR 1.06, 95% CI 1.03-1.09, p <0.001) were associated with graft survival in the multivariate analysis. Similar results were observed for overall survival.
CONCLUSIONS: Patients with rPBC and disease activity, as indicated by standard PBC risk scores, have impaired outcomes, supporting efforts to treat recurrent disease in similar ways to pre-transplant PBC.
UNASSIGNED: One in three people who undergo liver transplantation for primary biliary cholangitis develop recurrent disease in their new liver. Patients with recurrent primary biliary cholangitis and incomplete response to ursodeoxycholic acid, according to conventional prognostic scores, have worse clinical outcomes, with higher risk of graft loss and mortality in similar ways to the disease before liver transplantation. Our results supportsupport efforts to treat recurrent disease in similar ways to pre-transplant primary biliary cholangitis.
METHODS: A total of 332 patients with rPBC after LT were evaluated from 28 centers across Europe, North and South America. The median age at the time of rPBC was 58.0 years [IQR 53.2-62.6], and 298 patients (90%) were female. The biochemical response was measured with serum levels of alkaline phosphatase (ALP) and bilirubin, and Paris-2, GLOBE and UK-PBC scores at 1 year after UDCA initiation.
RESULTS: During a median follow-up of 8.7 years [IQR 4.3-12.9] after rPBC diagnosis, 52 patients (16%) had graft loss and 103 (31%) died. After 1 year of UDCA initiation the histological stage at rPBC (hazard ratio [HR] 3.97, 95% CI 1.36-11.55, p = 0.01), use of prednisone (HR 3.18, 95% CI 1.04-9.73, p = 0.04), ALP xULN (HR 1.59, 95% CI 1.26-2.01, p <0.001), Paris-2 criteria (HR 4.14, 95% CI 1.57-10.92, p = 0.004), GLOBE score (HR 2.82, 95% CI 1.71-4.66, p <0.001), and the UK-PBC score (HR 1.06, 95% CI 1.03-1.09, p <0.001) were associated with graft survival in the multivariate analysis. Similar results were observed for overall survival.
CONCLUSIONS: Patients with rPBC and disease activity, as indicated by standard PBC risk scores, have impaired outcomes, supporting efforts to treat recurrent disease in similar ways to pre-transplant PBC.
UNASSIGNED: One in three people who undergo liver transplantation for primary biliary cholangitis develop recurrent disease in their new liver. Patients with recurrent primary biliary cholangitis and incomplete response to ursodeoxycholic acid, according to conventional prognostic scores, have worse clinical outcomes, with higher risk of graft loss and mortality in similar ways to the disease before liver transplantation. Our results supportsupport efforts to treat recurrent disease in similar ways to pre-transplant primary biliary cholangitis.
摘要：
目的：大约30%的患者会发生复发性原发性胆汁性胆管炎（rPBC），并对肝移植（LT）后移植物和患者的总体生存率产生负面影响。缺乏有关rPBC对熊去氧胆酸（UDCA）的反应率的数据。我们评估了一个大的，国际,多中心队列评估PBC评分的性能，以预测rPBC患者肝移植后的移植物风险和总体生存率。
方法：对来自欧洲28个中心的332名接受LT术后rPBC的患者进行了评估，北美和南美。rPBC时的平均年龄为58.0岁[IQR53.2-62.6]，298例患者（90%）为女性。用血清碱性磷酸酶（ALP）和胆红素水平测量生化反应，在UDCA开始后1年，Paris-2，GLOBE和UK-PBC得分。
结果：在rPBC诊断后8.7年[IQR4.3-12.9]的中位随访期间，52例患者（16％）有移植物丢失，103例（31％）死亡。UDCA开始1年后，rPBC的组织学阶段(HR，3.97，95CI1.36-11.55，P=0.01），使用泼尼松（HR3.18，95CI1.04-9.73，P=0.04），ALPxULN（HR1.59，95CI1.26-2.01，P<0.001），巴黎-2标准（HR4.14，95CI1.57-10.92，P=0.004），GLOBE评分（HR2.82，95CI1.71-4.66，P<0.001），在多变量分析中,UK-PBC评分(HR1.06,95CI1.03-1.09,P<0.001)与移植物存活相关。在总体生存分析中发现了类似的结果。
结论：标准PBC风险评分显示的rPBC和疾病活动的患者结局受损，支持以与移植前PBC相似的方式治疗复发性疾病的努力。
■因原发性胆汁性胆管炎而接受肝移植的人中，有三分之一的人在他们的新肝脏中出现复发性疾病。根据常规预后评分，复发性原发性胆汁性胆管炎和熊去氧胆酸反应不完全的患者临床结局较差，与肝移植前的疾病相似，移植物丢失和死亡的风险更高。我们的结果强调支持以与移植前原发性胆汁性胆管炎相似的方式治疗复发性疾病的努力。
方法：对来自欧洲28个中心的332名接受LT术后rPBC的患者进行了评估，北美和南美。rPBC时的平均年龄为58.0岁[IQR53.2-62.6]，298例患者（90%）为女性。用血清碱性磷酸酶（ALP）和胆红素水平测量生化反应，在UDCA开始后1年，Paris-2，GLOBE和UK-PBC得分。
结果：在rPBC诊断后8.7年[IQR4.3-12.9]的中位随访期间，52例患者（16％）有移植物丢失，103例（31％）死亡。UDCA开始1年后，rPBC的组织学阶段(HR，3.97，95CI1.36-11.55，P=0.01），使用泼尼松（HR3.18，95CI1.04-9.73，P=0.04），ALPxULN（HR1.59，95CI1.26-2.01，P<0.001），巴黎-2标准（HR4.14，95CI1.57-10.92，P=0.004），GLOBE评分（HR2.82，95CI1.71-4.66，P<0.001），在多变量分析中,UK-PBC评分(HR1.06,95CI1.03-1.09,P<0.001)与移植物存活相关。在总体生存分析中发现了类似的结果。
结论：标准PBC风险评分显示的rPBC和疾病活动的患者结局受损，支持以与移植前PBC相似的方式治疗复发性疾病的努力。
■因原发性胆汁性胆管炎而接受肝移植的人中，有三分之一的人在他们的新肝脏中出现复发性疾病。根据常规预后评分，复发性原发性胆汁性胆管炎和熊去氧胆酸反应不完全的患者临床结局较差，与肝移植前的疾病相似，移植物丢失和死亡的风险更高。我们的结果强调支持以与移植前原发性胆汁性胆管炎相似的方式治疗复发性疾病的努力。
