title: Variations in primary sclerosing cholangitis across the age spectrum.
abstract: OBJECTIVE
  Primary sclerosing cholangitis (PSC) typically develops in middle-age adults. Little is known about phenotypic differences when PSC is diagnosed at various ages. Therefore, we sought to compare the clinical characteristics of a large PSC cohort based on the age when PSC was diagnosed.
  
  
  METHODS
  We performed a multicenter retrospective review to compare the features of PSC among those diagnosed between 1-19 (n = 95), 20-59 (n = 662), and 60-79 years (n = 102).
  
  
  RESULTS
  Those with an early diagnosis (ED) of PSC were more likely to have small-duct PSC (13%) than those with a middle-age diagnosis (MD) (5%) and late diagnosis (LD) groups (2%), P < 0.01, and appeared to have a decrease risk of hepatobiliary malignancies: ED versus MD: hazard ratio (HR), 0.25; 95% confidence interval (CI) 0.06-1.03, and ED versus LD: HR, 0.07; 95% CI 0.01-0.62. Cholangiocarcinoma was diagnosed in 78 subjects (ED n = 0, MD n = 66, and LD n = 12) and was more likely to be diagnosed within a year after the PSC diagnosis among those found to have PSC late in life: ED 0% (0/95), MD 2% (14/662), and LD 6% (6/102), P = 0.02. Similarly, hepatic decompensation was more common among those with LD-PSC versus younger individuals: LD versus MD: HR, 1.64; 95% CI 0.98-2.70, and LD versus ED: HR, 2.26; 95% CI 1.02-5.05.
  
  
  CONCLUSIONS
  Those diagnosed with PSC early in life are more likely to have small-duct PSC and less likely to have disease-related complications. Clinicians should be vigilant for underlying cholangiocarcinoma among those with PSC diagnosed late in life.
authors.affiliation: Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine and Sciences, Rochester, Minnesota, USA.
authors.forename: John E
authors.initials: JE
authors.lastname: Eaton
authors.affiliation: Division of Biomedical Statistics and Informatics, Mayo Clinic College of Medicine and Sciences, Rochester, Minnesota, USA.
authors.forename: Bryan M
authors.initials: BM
authors.lastname: McCauley
authors.affiliation: Division of Biomedical Statistics and Informatics, Mayo Clinic College of Medicine and Sciences, Rochester, Minnesota, USA.
authors.forename: Elizabeth J
authors.initials: EJ
authors.lastname: Atkinson
authors.affiliation: Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine and Sciences, Rochester, Minnesota, USA.
authors.forename: Brian D
authors.initials: BD
authors.lastname: Juran
authors.affiliation: Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine and Sciences, Rochester, Minnesota, USA.
authors.forename: Erik M
authors.initials: EM
authors.lastname: Schlicht
authors.affiliation: Division of Biomedical Statistics and Informatics, Mayo Clinic College of Medicine and Sciences, Rochester, Minnesota, USA.
authors.forename: Mariza
authors.initials: M
authors.lastname: de Andrade
authors.affiliation: Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine and Sciences, Rochester, Minnesota, USA.|Division of Biomedical Statistics and Informatics, Mayo Clinic College of Medicine and Sciences, Rochester, Minnesota, USA.
authors.forename: Konstantinos N
authors.initials: KN
authors.lastname: Lazaridis
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D001650:Bile Duct Neoplasms / Q000453:epidemiology / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D018281:Cholangiocarcinoma / Q000453:epidemiology / Q000209:etiology; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D015331:Cohort Studies; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D015337:Multicenter Studies as Topic; D012189:Retrospective Studies; D012306:Risk; D013997:Time Factors; D055815:Young Adult
keywords: cholangiocarcinoma; inflammatory bowel disease; liver transplantation; primary sclerosing cholangitis