I. Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Hepato-Biliary Center, AP-HP Paul Brousse Hospital, University of Paris-Sud, Villejuif, France.
Liver Transpl. 2020 Jul;26(7):866-877. doi: 10.1002/lt.25739. Epub 2020 May 1.
The aim of this study was to analyze longterm patient and graft survival after liver transplantation for autoimmune hepatitis (AIH-LT) from the prospective multicenter European Liver Transplant Registry. Patient and liver graft survival between 1998 and 2017 were analyzed. Patients after AIH-LT (n = 2515) were compared with patients receiving LT for primary biliary cholangitis (PBC-LT; n = 3733), primary sclerosing cholangitis (PSC-LT; n = 5155), and alcohol-related cirrhosis (AC-LT; n = 19,567). After AIH-LT, patient survival was 79.4%, 70.8%, and 60.3% and graft survival was 73.2%, 63.4%, and 50.9% after 5, 10, and 15 years of follow-up. Overall patient survival was similar to patients after AC-LT (P = 0.44), but worse than after PBC-LT (hazard ratio [HR], 1.48; P < 0.001) and PSC-LT (HR, 1.19; P = 0.002). AIH-LT patients were at increased risk for death (HR, 1.37-1.84; P < 0.001) and graft loss (HR, 1.35-1.80; P < 0.001) from infections compared with all other groups and had a particularly increased risk for lethal fungal infections (HR, 3.38-4.20; P ≤ 0.004). Excluding patients who died within 90 days after LT, risk of death after AIH-LT was superior compared with AC-LT (HR, 0.84; P = 0.004), worse compared with PBC-LT (HR, 1.38; P < 0.001) and similar compared with PSC-LT (P = 0.93). Autoimmune hepatitis (AIH) patients with living donor liver transplantation (LDLT) showed reduced survival compared with patients receiving donation after brain death (HR, 1.96; P < 0.001). In AIH-LT patients, overall survival is inferior to PBC-LT and PSC-LT. The high risk of death after AIH-LT is caused mainly by early fatal infections, including fungal infections. Patients with LDLT for AIH show reduced survival.
本研究旨在通过前瞻性的欧洲肝移植注册中心分析，研究自身免疫性肝炎（AIH）患者接受肝移植（AIH-LT）后的长期患者和移植物存活率。分析了 1998 年至 2017 年期间的患者和肝移植物存活率。将 AIH-LT 患者（n=2515）与原发性胆汁性胆管炎（PBC-LT；n=3733）、原发性硬化性胆管炎（PSC-LT；n=5155）和酒精相关肝硬化（AC-LT；n=19567）接受 LT 的患者进行比较。在 AIH-LT 后，5、10 和 15 年随访时，患者存活率分别为 79.4%、70.8%和 60.3%，移植物存活率分别为 73.2%、63.4%和 50.9%。总体患者存活率与 AC-LT 患者相似（P=0.44），但比 PBC-LT（危险比[HR]，1.48；P<0.001）和 PSC-LT（HR，1.19；P=0.002）患者差。与所有其他组相比，AIH-LT 患者因感染导致死亡（HR，1.37-1.84；P<0.001）和移植物丧失（HR，1.35-1.80；P<0.001）的风险增加，并且特别容易发生致命的真菌感染（HR，3.38-4.20；P≤0.004）。排除 LT 后 90 天内死亡的患者后，AIH-LT 患者的死亡风险优于 AC-LT（HR，0.84；P=0.004），劣于 PBC-LT（HR，1.38；P<0.001），与 PSC-LT 相似（P=0.93）。与接受脑死亡后捐献（HR，1.96；P<0.001）的患者相比，接受活体供肝移植（LDLT）的 AIH 患者的存活率降低。在 AIH-LT 患者中，总生存率低于 PBC-LT 和 PSC-LT。AIH-LT 后高死亡率主要是由早期致命感染引起的，包括真菌感染。接受 LDLT 治疗 AIH 的患者存活率降低。
