Variations in primary sclerosing cholangitis across the age spectrum

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.