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Liver Transplantation for Children With Primary Sclerosing Cholangitis and Autoimmune Hepatitis: UNOS Database
Jacqueline Jossen1, Rachel Annunziato, Hee-Sung Kim
1*Department of Pediatrics and RMTI, Mount Sinai Medical Center, New York †Fordham University, Bronx, NY.
Insights
Pediatric liver transplant outcomes for autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) show similar patient and graft survival rates. However, AIH patients with fulminant hepatic failure had lower survival.
Area of Science:
- Hepatology
- Transplant Surgery
- Pediatric Gastroenterology
Background:
- Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are progressive immune-mediated liver diseases.
- Liver transplantation (LT) is a potential treatment for end-stage liver disease in children with AIH and PSC.
- Outcomes of pediatric LT for AIH and PSC are not well-characterized.
Purpose of the Study:
- To analyze and compare patient and graft survival after liver transplantation in children with AIH versus PSC.
- To evaluate the impact of transplant status (e.g., status 1 for fulminant hepatic failure) on outcomes in pediatric AIH patients.
Main Methods:
- Retrospective analysis of the United Network for Organ Sharing database.
- Inclusion of children (≤18 years) undergoing first, isolated LT for AIH or PSC between 2002 and 2012.
- Comparison of one- and five-year patient and graft survival rates between AIH and PSC groups, and within the AIH group based on transplant status.
Main Results:
- A total of 174 children with AIH and 113 with PSC underwent LT.
- One- and five-year patient survival rates were high and not significantly different between AIH (91.4% at 5 years) and PSC (92.9% at 5 years) groups.
- AIH patients listed as status 1 (fulminant hepatic failure) had significantly lower patient survival compared to those with end-stage liver disease, but similar graft survival.
Conclusions:
- Pediatric liver transplantation for AIH and PSC demonstrates comparable patient and graft survival rates at one and five years.
- Urgent transplantation (status 1) in pediatric AIH patients is associated with reduced patient survival but not graft survival.
- These findings contribute to understanding pediatric end-stage liver disease management and LT outcomes.
Objectives:
Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are progressive immune-mediated inflammatory diseases that may require liver transplant (LT). Outcomes in children undergoing LT for these diseases are poorly studied in the pediatric end-stage liver disease era. We aimed to characterize the outcome of LT in children with AIH and PSC.
Methods:
Children 18 years or younger with PSC or AIH who had a first, isolated LT from 2002 to 2012 were identified from the United Network for Organ Sharing database. Graft and patient outcomes were studied.
Results:
A total of 174 children with AIH and 113 with PSC were transplanted in the study period. One-year patient survival was 95.4% for AIH and 97.3% for PSC. Five-year patient survival was 91.4% for AIH and 92.9% for PSC. Patient survival was not significantly different between the 2 groups. Forty-four (25.2%) children with AIH were listed as status 1 for transplant (fulminant hepatic failure at presentation or acute-on-chronic disease). Patients transplanted as status 1 had significantly lower patient survival compared with patients transplanted with AIH and end-stage liver disease. The one- and five-year graft survival rates were not significantly different between patients with AIH and PSC.
Conclusion:
Children with AIH transplanted as status 1 had significantly lower patient survival rates but similar graft survival rates to children with chronic AIH. Children transplanted for AIH versus PSC showed no significant differences in patient or graft survival at both 1 and 5 years.
