Variations in primary sclerosing cholangitis across the age spectrum

• Published in: Journal of gastroenterology and hepatology Vol. 32; no. 10; pp. 1763 - 1768
• Main Authors: Eaton, John E, McCauley, Bryan M, Atkinson, Elizabeth J, Juran, Brian D, Schlicht, Erik M, Andrade, Mariza, Lazaridis, Konstantinos N
• Format: Journal Article
• Language: English
• Published: Australia Wiley Subscription Services, Inc 01.10.2017
• Subjects: Adolescent; Adult; Age; Age Distribution; Aged; Bile Duct Neoplasms - epidemiology; Bile Duct Neoplasms - etiology; Child; Child, Preschool; Cholangiocarcinoma; Cholangiocarcinoma - epidemiology; Cholangiocarcinoma - etiology; Cholangitis; Cholangitis, Sclerosing - complications; Cholangitis, Sclerosing - diagnosis; Cholangitis, Sclerosing - pathology; Cohort Studies; Early Diagnosis; Female; Humans; Infant; inflammatory bowel disease; liver transplantation; Male; Middle age; Middle Aged; Multicenter Studies as Topic; primary sclerosing cholangitis; Retrospective Studies; Risk; Time Factors; Young Adult; cholangiocarcinoma; inflammatory bowel disease; liver transplantation; primary sclerosing cholangitis
• DOI: 10.1111/jgh.13774
• ISSN: 0815-9319; 1440-1746

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.