Premium
Variations in primary sclerosing cholangitis across the age spectrum
Author(s) -
Eaton John E,
McCauley Bryan M,
Atkinson Elizabeth J,
Juran Brian D,
Schlicht Erik M,
Andrade Mariza,
Lazaridis Konstantinos N
Publication year - 2017
Publication title -
journal of gastroenterology and hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.214
H-Index - 130
eISSN - 1440-1746
pISSN - 0815-9319
DOI - 10.1111/jgh.13774
Subject(s) - medicine , primary sclerosing cholangitis , hazard ratio , gastroenterology , confidence interval , retrospective cohort study , bile duct , cohort , decompensation , pediatrics , disease
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.