Variations in Primary Sclerosing Cholangitis Across the Age Spectrum.

Eaton JE, McCauley BM, Atkinson EJ et al.

Division of Gastroenterology, Hepatology Mayo Clinic, Rochester, MN.

Journal of gastroenterology and hepatology. Feb 2017.

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.We performed a multicenter retrospective review to compare the features of PSC among those diagnosed between 1-19 years (yrs) (n = 95), 20-59 yrs (n = 662), and 60-79 yrs (n = 102).Those with an early diagnosis (ED) PSC were more likely to have small duct PSC (13%) versus 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 vs. MD: HR, 0.25; 95% CI 0.06-1.03 and ED vs LD: HR, 0.07; 95% CI 0.01-0.62. Cholangiocarcinoma (CCA) was diagnosed in 78 subjects (ED n = 0; MD n = 66, 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), LD 6% (6/102), p = 0.02. Similarly, hepatic decompensation was more common among those with LD PSC versus younger individuals: LD vs. MD: HR,1.64; 95% CI 0.98-2.70 and LD vs ED: HR, 2.26; 95% CI 1.02-5.05.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 CCA among those with PSC diagnosed late in life. Pubmed