https://www.ncbi.nlm.nih.gov/pubmed/27755342

Jossen J, Annunziato R, Kim HS, Chu J, Arnon R. J Pediatr Gastroenterol Nutr. 2016 Oct 13.

Abstract

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 (PELD) era. We aimed to characterize the outcome of LT in children with AIH and PSC.

METHODS:
Children ≤18 years with PSC or AIH who had a first, isolated LT from 2002-2012 were identified from the UNOS database. Graft and patient outcomes were studied.

RESULTS:
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 two 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 to patients transplanted with AIH and end stage liver disease (ESLD). The one and five year graft survival rates were not significantly different between AIH and PSC patients.

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 one and five years.