Variations in primary sclerosing cholangitis across the age spectrum

John E. Eaton, Bryan M. McCauley, Elizabeth J. Atkinson, Brian D. Juran, Erik M. Schlicht, Mariza de Andrade, Konstantinos N. Lazaridis

Research output: Contribution to journalArticlepeer-review

9 Scopus citations


Background and Aim: 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.

Original languageEnglish (US)
Pages (from-to)1763-1768
Number of pages6
JournalJournal of Gastroenterology and Hepatology (Australia)
Issue number10
StatePublished - Oct 2017


  • cholangiocarcinoma
  • inflammatory bowel disease
  • liver transplantation
  • primary sclerosing cholangitis

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology


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