title: Differences in Phenotypes and Liver Transplantation Outcomes by Age Group in Patients with Primary Sclerosing Cholangitis.
abstract: 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.
  
  
  
  To characterize phenotypic variations and liver transplantation outcomes by age group in adults with PSC.
  
  
  
  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.
  
  
  
  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).
  
  
  
  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.
authors.affiliation: Duke University School of Medicine, Durham, NC, USA.
authors.forename: Jacqueline B
authors.initials: JB
authors.lastname: Henson
authors.affiliation: Division of Gastroenterology, Department of Medicine, Duke University School of Medicine, Durham, NC, USA.
authors.forename: Yuval A
authors.initials: YA
authors.lastname: Patel
authors.affiliation: Division of Gastroenterology, Department of Medicine, Duke University School of Medicine, Durham, NC, USA.|Duke Clinical Research Institute, Durham, NC, USA.
authors.forename: Julius M
authors.initials: JM
authors.lastname: Wilder
authors.affiliation: Department of Biostatistics, Duke University School of Medicine, Durham, NC, USA.
authors.forename: Jiayin
authors.initials: J
authors.lastname: Zheng
authors.affiliation: Department of Biostatistics, Duke University School of Medicine, Durham, NC, USA.|Duke Clinical Research Institute, Durham, NC, USA.
authors.forename: Shein-Chung
authors.initials: SC
authors.lastname: Chow
authors.affiliation: Division of Gastroenterology, Department of Medicine, Duke University School of Medicine, Durham, NC, USA.
authors.forename: Lindsay Y
authors.initials: LY
authors.lastname: King
authors.affiliation: Division of Gastroenterology, Department of Medicine, Duke University School of Medicine, Durham, NC, USA. andrew.muir@duke.edu.|Duke Clinical Research Institute, Durham, NC, USA. andrew.muir@duke.edu.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Muir
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000208:ethnology / Q000401:mortality / Q000601:surgery*; D016208:Databases, Factual; D005260:Female; D006084:Graft Rejection / Q000208:ethnology; D006085:Graft Survival; D006801:Humans; D053208:Kaplan-Meier Estimate; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D017678:Sex Distribution; D013997:Time Factors; D009927:Tissue and Organ Procurement; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology; D014850:Waiting Lists / Q000401:mortality; D055815:Young Adult
keywords: Age factors; Liver transplantation; Phenotype; Sclerosing cholangitis; Treatment outcome