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Digestive Diseases and Sciences
Published in: Digestive Diseases and Sciences 4/2024

12-02-2024 | Primary Sclerosing Cholangitis | Original Article

Primary Sclerosing Cholangitis Limited to Intrahepatic Bile Ducts Has Distinctly Better Prognosis

Authors: Vijay S. Are, Mark A. Gromski, Fatih Akisik, Eduardo Vilar-Gomez, Craig Lammert, Marwan Ghabril, Raj Vuppalanchi, Naga Chalasani

Published in: Digestive Diseases and Sciences | Issue 4/2024

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Abstract

Background

There are two sub-phenotypes of large-duct primary sclerosing cholangitis (PSC): isolated intrahepatic PSC (IIPSC) and extrahepatic disease with or without intrahepatic (extra/intrahepatic).

Aims

This study examined the differences in outcomes in patients with IIPSC compared to extra/intrahepatic and small-duct PSC.

Methods

Patients with PSC treated at our institution from 1998 to 2019 were investigated. Biochemistries, clinical events, and survival were assessed by chart review and National Death Index. Cox-proportional hazards were used to determine the risk of clinical outcomes based on biliary tract involvement.

Results

Our cohort comprised 442 patients with large-duct PSC (57 had IIPSC, 385 had extra/intrahepatic PSC) and 23 with small-duct PSC. Median follow-up in the IIPSC group was not significantly different from the extra/intrahepatic group [7 vs. 6 years, P = 0.06]. Except for lower age (mean 37.9 vs. 43.0 years, P = 0.045), the IIPSC group was not different from the extra/intrahepatic. The IIPSC group had longer transplant-free survival (log-rank P = 0.001) with a significantly lower risk for liver transplantation (12% vs. 34%, P < 0.001). The IIPSC group had a lower risk of death or transplantation than the extra/intrahepatic PSC group [HR: 0.34, 95% CI: 0.17—0.67, P < 0.001]. No bile duct or gallbladder cancers developed in patients with IIPSC, compared to 24 in the extra/intrahepatic group. The clinical characteristics and outcomes of IIPSC were similar to 23 individuals with small-duct PSC.

Conclusions

Patients with IIPSC have a favorable prognosis similar to small-duct PSC. These data are important for counseling patients and designing therapeutic trials for PSC.
Appendix
Available only for authorised users
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Metadata
Title
Primary Sclerosing Cholangitis Limited to Intrahepatic Bile Ducts Has Distinctly Better Prognosis
Authors
Vijay S. Are
Mark A. Gromski
Fatih Akisik
Eduardo Vilar-Gomez
Craig Lammert
Marwan Ghabril
Raj Vuppalanchi
Naga Chalasani
Publication date
12-02-2024
Publisher
Springer US
Published in
Digestive Diseases and Sciences / Issue 4/2024
Print ISSN: 0163-2116
Electronic ISSN: 1573-2568
DOI
https://doi.org/10.1007/s10620-023-08260-1

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