Objective: To explore the clinical characteristics and long-term prognosis of recipients with end-stage autoimmune liver diseases (AILD) after liver transplantation.
Methods: A retrospective study was conducted. The data of 147 end-stage autoimmune liver diseases recipients who underwent liver transplantation admitted to department of liver transplantation of Tianjin First Center Hospital from January 2004 to August 2018 were collected. Patients were followed till March 1st, 2019. The postoperative survival was analyzed, the cumulative survival rate was estimated by the Kaplan-Meier survival curve, and the cause of death was analyzed. Postoperative rejection, recurrence of AILD, biliary complications, and hepatitis virus infection were also summarized.
Results: Among the 147 recipients, 25 were autoimmune hepatitis (AIH), 94 were primary biliary cholangitis (PBC), 14 were AIH overlapping PBC (AIH-PBC), 14 were primary sclerosing cholangitis (PSC). The 147 recipients were consisted of 22 males and 125 females, with a ratio of 1:5.7, with a mean age of (53.2±10.1) years old. The average model for end-stage liver disease (MELD) score was 14.6±9.0. After a median follow-up of 49.0 months, 21 recipients died, and 126 recipients survived. The survival curve analysis showed that the 1-, 3-, 5- and 10-year cumulative survival rate was 98.6%, 94.0%, 84.6%, and 76.9%, respectively. The main cause of death was graft dysfunction, followed by graft failure and respiratory failure. Among the 147 recipients, 17 (11.56%) rejection occurred, 19 recurrence (12.93%) were diagnosed with liver biopsy, and of which 2 PBC recipients overlapped de novo AIH-PBC. Biliary complications happened in 37 recipients (25.17%). De novo hepatitis virus infection was found in 19 recipients (12.93%), including 14 with hepatitis B virus (HBV) and 5 with hepatitis C virus (HCV).
Conclusions: The majority patients of the end-stage of AILD were middle-aged and old women, and PBC was the most common. Liver transplantation is an effective treatment for end-stage autoimmune liver diseases with excellent prognosis. Postoperative immunosuppressant usage and recurrence should be emphasized.