Differences in Phenotypes and Liver Transplantation Outcomes by Age Group in Patients with Primary Sclerosing Cholangitis

Dig Dis Sci. 2017 Nov;62(11):3200-3209. doi: 10.1007/s10620-017-4559-1. Epub 2017 Apr 8.

Abstract

Background: There is increasing evidence for a heterogeneity of phenotypes in primary sclerosing cholangitis (PSC), but differences across the age spectrum in adults with PSC have not been well characterized.

Aims: To characterize phenotypic variations and liver transplantation outcomes by age group in adults with PSC.

Methods: The United Network for Organ Sharing database was used to identify waitlist registrations for primary liver transplantation in adults with PSC. Patients were split into three age groups: 18-39 (young), 40-59 (middle-aged), and ≥60 (older). Their clinical characteristics and outcomes on the waitlist and post-transplant were compared.

Results: Overall, 8272 adults with PSC were listed for liver transplantation during the study period, of which 28.9% were young, 52.0% were middle-aged, and 19.1% were older. The young age group had the greatest male predominance (70.0 vs. 66.2 vs. 65.1%, p = 0.001), the highest proportion of black individuals (20.0 vs. 11.0 vs. 5.5%, p < 0.001), and the most patients listed with concomitant autoimmune hepatitis (2.2 vs. 1.0 vs. 0.8%, p < 0.001). Older patients experienced the greatest waitlist and post-transplant mortality. Graft survival was greatest in the middle-aged group. Young patients were most likely to experience acute rejection (31 vs. 22.8 vs. 18.0%, p < 0.001) and have graft failure due to chronic rejection or PSC recurrence (47.8 vs. 42.3 vs. 17.9%, p < 0.001).

Conclusions: Age-related differences exist among adults with PSC and are associated with outcomes pre- and post-transplant. Young patients may have a more robust immune-related phenotype that is associated with poorer graft survival. Future studies are needed to further investigate these findings.

Keywords: Age factors; Liver transplantation; Phenotype; Sclerosing cholangitis; Treatment outcome.

Publication types

  • Comparative Study
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Cholangitis, Sclerosing / diagnosis
  • Cholangitis, Sclerosing / ethnology
  • Cholangitis, Sclerosing / mortality
  • Cholangitis, Sclerosing / surgery*
  • Databases, Factual
  • Female
  • Graft Rejection / ethnology
  • Graft Survival
  • Humans
  • Kaplan-Meier Estimate
  • Liver Transplantation* / adverse effects
  • Liver Transplantation* / mortality
  • Male
  • Middle Aged
  • Phenotype
  • Recurrence
  • Retrospective Studies
  • Risk Factors
  • Sex Distribution
  • Time Factors
  • Tissue and Organ Procurement
  • Treatment Outcome
  • United States / epidemiology
  • Waiting Lists / mortality
  • Young Adult