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.

1 Department of Medicine University Medical Center Hamburg Eppendorf Hamburg Germany

Avicenna Center for Medicine and Organ Transplant Shiraz University of Medical Sciences Shiraz Iran

Department of General Surgery Ege University Medical School Izmir Turkey

Department of General Transplant and Liver Surgery Medical University of Warsaw Warsaw Poland

Department of General Visceral and Transplantation Surgery University of Heidelberg Heidelberg Germany

Department of Hepatobiliary and Digestive Surgery Pontchaillou University Hospital Rennes France

Department of Hepatology and Gastroenterology University Hospital of Henri Mondor Creteil France

Department of HPB Surgery Queen Elizabeth Hospital Birmingham UK

Department of Surgery and Liver Transplant Institute Inonu University Faculty of Medicine Malatya Turkey

Department of Surgery Charité Universitätsmedizin Berlin Campus Charité Mitte and Campus Virchow Klinikum Berlin Germany

Department of Transplant Surgery Institute for Clinical and Experimental Medicine Prague Czech Republic

Department of Visceral and Transplant Surgery University Hospital Essen Essen Germany

European Reference Network for Hepatological Diseases Hamburg Germany

Hepato Biliary Center AP HP Paul Brousse Hospital University of Paris Sud Villejuif France

King's Liver Transplant Unit King's College Hospital NHS Foundation Trust London United Kingdom

Liver Transplantation and Hepatology Unit La Fe University Hospital Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas University of Valencia Valencia Spain

Liver Transplantation Center Florence Nightingale Hospital Istanbul Turkey

Martin Zeitz Center for Rare Diseases University Medical Centre Hamburg Eppendorf Hamburg Germany

Organ Transplantation Center Memorial Hospital Istanbul Turkey

The Leeds Teaching Hospitals NHS Trust Leeds UK

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