Knowledge of living donor liver transplantation (LDLT) for autoimmune liver diseases (AILDs) is scarce. This study analyzed survival in LDLT recipients registered in the European Liver Transplant Registry with autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis (PSC) and the non-autoimmune disorder alcohol-related cirrhosis. In total, 29 902 individuals enrolled between 1998 and 2017 were analyzed, including 1003 with LDLT. Survival from >90 days after LDLT for AILDs in adults was 85.5%, 74.2%, and 58.0% after 5, 10, and 15 years. Adjusted for recipient age, sex, and liver transplantation era, adult PSC patients receiving LDLT showed increased mortality compared to donation after brain death (DBD) (hazard ratio [HR] = 1.95, 95% confidence interval [CI] = 1.36–2.80, p < .001). Pediatric PSC patients showed also increased mortality >90 days after LDLT compared to DBD (HR = 3.00, 95% CI 1.04–8.70, p = .043). Multivariate analysis identified several risk factors for death in adult PSC patients receiving LDLT including a male donor (HR = 2.49, p = .025). Adult PSC patients with LDLT versus DBD conferred increased mortality from disease recurrence (subdistribution hazard ratio [subHR] = 5.36, p = .001) and biliary complications (subHR = 4.40, p = .006) in multivariate analysis. While long-term outcome following LDLT for AILD is generally favorable, PSC patients with LDLT compared to DBD might be at increased risk of death.

中文翻译：

---

与自身免疫性肝病脑死亡后捐献相比，活体肝移植后的长期结果：欧洲肝移植登记处的经验

自身免疫性肝病 (AILD) 的活体肝移植 (LDLT) 知识很少。这项研究分析了在欧洲肝移植登记处登记的患有自身免疫性肝炎、原发性胆汁性胆管炎、原发性硬化性胆管炎 (PSC) 和非自身免疫性疾病酒精相关性肝硬化的 LDLT 接受者的生存率。总共分析了 1998 年至 2017 年间登记的 29902 人，其中 1003 人患有 LDLT。成人 AILD 在 LDLT 后 >90 天的生存率在 5、10 和 15 年后分别为 85.5%、74.2% 和 58.0%。根据受体年龄、性别和肝移植时代进行调整后，接受 LDLT 的成年 PSC 患者与脑死亡后捐献 (DBD) 相比死亡率增加（风险比 [HR] = 1.95，95% 置信区间 [CI] = 1.36–2.80，p <.001）。与 DBD 相比，儿科 PSC 患者在 LDLT 后 >90 天的死亡率也有所增加（HR = 3.00，95% CI 1.04–8.70，p  = .043）。多变量分析确定了接受 LDLT 的成年 PSC 患者死亡的几个危险因素，包括一名男性供体（HR = 2.49，p  = .025）。在多变量分析中，与 DBD 相比，接受 LDLT 的成年 PSC 患者因疾病复发（亚分布风险比 [subHR] = 5.36，p  = .001）和胆道并发症（subHR = 4.40，p  = .006）死亡率增加。虽然 AILD 的 LDLT 后的长期结果通常是有利的，但与 DBD 相比，接受 LDLT 的 PSC 患者的死亡风险可能更高。