Liver transplantation for primary sclerosing cholangitis in children versus young adults.

INTRODUCTION

Primary sclerosing cholangitis is an immune-mediated cholestatic liver disease characterized by inflammation of the intra- and/or extrahepatic bile ducts and is associated with inflammatory bowel disease.

METHODS

We retrospectively compared children (<18 years) and young adults (18-25 years) undergoing first liver transplantation for primary sclerosing cholangitis using United Network for Organ Sharing data (February 2002-December 2024).

RESULTS

A total of 531 patients with primary sclerosing cholangitis underwent liver transplantation (160 children; 371 young adults). Children had lower laboratory Model for End-stage Liver Disease / Pediatric End-stage Liver Disease score than young adults (median 13.0 vs 20.0, P < .001). A smaller proportion of children had inflammatory bowel disease (58.8% vs 67.9%, P = .04), ascites (35.9% vs 47.8%, P = .01), and underwent living donor liver transplantation (18.1% vs 29.9%, P < .001) than young adults. Multivariable Cox regression showed decreased risk of patient mortality in children versus young adults (hazard ratio 0.49, 95% confidence interval 0.27-0.89, P = .02) and White, non-Hispanic patients compared with other race/ethnicity (0.44, 0.27-0.73, P = .001), when adjusting for inflammatory bowel disease, Model for End-stage Liver Disease / Pediatric End-stage Liver Disease score, intensive care unit status, graft type, and era. Multivariable Cox regression showed decreased risk of graft loss in female versus male sex (hazard ratio 0.68, 95% confidence interval 0.49-0.96, P = .03) and White, non-Hispanic patients compared with other race/ethnicity (0.71, 0.50-0.99, P = .04), when adjusting for inflammatory bowel disease, Model for End-stage Liver Disease / Pediatric End-stage Liver Disease score, intensive care unit status, graft type, and era.

CONCLUSION

Pediatric liver transplantation recipients with primary sclerosing cholangitis exhibit superior patient survival versus young adult liver transplantation recipients, whereas graft survival does not differ significantly. White, non-Hispanic race/ethnicity was independently associated with improved survival, underscoring persistent disparities in liver transplantation outcomes.