Hildebrand Tatiana, Pannicke Nadine, Dechene Alexander, Gotthardt Daniel N, Kirchner Gabriele, Reiter Florian P, Sterneck Martina, Herzer Kerstin, Lenzen Henrike, Rupp Christian, Barg-Hock Hannelore, de Leuw Philipp, Teufel Andreas, Zimmer Vincent, Lammert Frank, Sarrazin Christoph, Spengler Ulrich, Rust Christian, Manns Michael P, Strassburg Christian P, Schramm Christoph, Weismüller Tobias J
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hannover, Germany.
1st Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Liver Transpl. 2016 Jan;22(1):42-52. doi: 10.1002/lt.24350.
Liver transplantation (LT) is the only definitive treatment for patients with end-stage liver disease due to primary sclerosing cholangitis (PSC), but a high rate of biliary strictures (BSs) and of recurrent primary sclerosing cholangitis (recPSC) has been reported. In this multicenter study, we analyzed a large patient cohort with a long follow-up in order to evaluate the incidence of BS and recPSC, to assess the impact on survival after LT, and to identify risk factors. We collected clinical, surgical, and laboratory data and records on inflammatory bowel disease (IBD), immunosuppression, recipient and graft outcome, and biliary complications (based on cholangiography and histology) of all patients who underwent LT for PSC in 10 German transplant centers between January 1990 and December 2006; 335 patients (68.4% men; mean age, 38.9 years; 73.5% with IBD) underwent transplantation 8.8 years after PSC diagnosis with follow-up for 98.8 months. The 1-, 5-, and 10-year recipient and graft survival was 90.7%, 84.8%, 79.4% and 79.1%, 69.0%, 62.4%, respectively. BS was diagnosed in 36.1% after a mean time of 3.9 years, and recPSC was diagnosed in 20.3% after 4.6 years. Both entities had a significant impact on longterm graft and recipient survival. Independent risk factors for BS were donor age, ulcerative colitis, chronic ductopenic rejection, bilirubin, and international normalized ratio (INR) at LT. Independent risk factors for recPSC were donor age, IBD, and INR at LT. These variables were able to categorize patients into risk groups for BS and recPSC. In conclusion, BS and recPSC affect longterm graft and patient survival after LT for PSC. Donor age, IBD, and INR at LT are independent risk factors for BS and recPSC and allow for risk estimation depending on the recipient-donor constellation.
肝移植（LT）是原发性硬化性胆管炎（PSC）所致终末期肝病患者的唯一确定性治疗方法，但据报道，胆管狭窄（BS）和复发性原发性硬化性胆管炎（recPSC）的发生率较高。在这项多中心研究中，我们分析了一个长期随访的大型患者队列，以评估BS和recPSC的发生率，评估其对肝移植后生存的影响，并确定危险因素。我们收集了1990年1月至2006年12月期间在10个德国移植中心接受PSC肝移植的所有患者的临床、手术和实验室数据以及关于炎症性肠病（IBD）、免疫抑制、受者和移植物结局以及胆道并发症（基于胆管造影和组织学）的记录；335例患者（68.4%为男性；平均年龄38.9岁；73.5%患有IBD）在PSC诊断后8.8年接受移植，随访98.8个月。受者和移植物的1年、5年和10年生存率分别为90.7%、84.8%、79.4%和79.1%、69.0%、62.4%。平均3.9年后，36.1%的患者被诊断为BS，4.6年后，20.3%的患者被诊断为recPSC。这两种情况均对移植物和受者的长期生存有显著影响。BS的独立危险因素包括供体年龄、溃疡性结肠炎、慢性胆管开放性排斥反应、胆红素以及肝移植时的国际标准化比值（INR）。recPSC的独立危险因素包括供体年龄、IBD以及肝移植时的INR。这些变量能够将患者分为BS和recPSC的风险组。总之，BS和recPSC影响PSC肝移植后移植物和患者的长期生存。供体年龄、IBD以及肝移植时的INR是BS和recPSC的独立危险因素，并可根据受者-供体情况进行风险评估。
