Cited by 119Liver Diseases and ImmunityPediatric Hepatobiliary Diseases and TreatmentsInflammatory Bowel Disease

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Primary Sclerosing Cholangitis: Diagnosis, Prognosis, and Management

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Singh, Siddharth, Talwalkar, Jayant A.·2013·Clinical Gastroenterology and Hepatology

DOI 10.1016/j.cgh.2013.02.016PMC PMC3692584

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Abstract

Primary sclerosing cholangitis (PSC) is a chronic immune-mediated disease of the liver of unclear etiology, characterized by chronic inflammation and fibrosis of bile ducts. It primarily affects middle-aged men and is associated with 4-fold increased mortality as compared with an age- and sex-matched population. Progressive biliary and hepatic damage results in portal hypertension and hepatic failure in a significant majority of patients over a 10- to 15-year period from the initial diagnosis. In addition, PSC confers a markedly increased risk of hepatobiliary cancer, including cholangiocarcinoma and gallbladder cancer, as compared with the general population, and cancer is the leading cause of mortality in patients with PSC. It is associated with inflammatory bowel disease in 70% of patients and increases the risk of colorectal cancer almost 10-fold. Despite significant research efforts in this field, the pathogenic mechanisms of PSC are still incompletely understood, although growing evidence supports the role of genetic and immunologic factors. There are no proven medical therapies that alter the natural course of the disease. Thus, liver transplantation is the only available treatment for patients with advanced PSC, with excellent outcomes in this population.

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Authors: Singh, S., & Talwalkar, J. A.
Affiliations: Mayo Clinic (US)
Corresponding author: Talwalkar, Jayant A.
ORCID: Singh, Siddharth
Venue: Clinical Gastroenterology and Hepatology
Publisher: Elsevier BV
Published: 2013-08
Volume: 11
Issue: 8
Pages: 898-907
ISSN: 1542-3565 (ISSN-L)
DOI: 10.1016/j.cgh.2013.02.016
PMC: PMC3692584
OpenAlex: W2076789082
Cited by: 119
References: 114
Citations by year: 2026: 6 · 2025: 8 · 2024: 6 · 2023: 9 · 2022: 8 · 2021: 5 · 2020: 8 · 2019: 7 · 2018: 10 · 2017: 11 · 2016: 9 · 2015: 13 · 2014: 13 · 2013: 6
FWCI: 1.74
Type: journal article
Language: en
Open access: bronze
doi.org
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
www.elsevier.com/legal/tdmrep-license (tdm)
Locations: Clinical Gastroenterology and Hepatology (publishedVersion)
PubMed (publishedVersion)
PubMed Central (acceptedVersion)
Funders: National Institute of Diabetes and Digestive and Kidney Diseases (R01DK056924)
Topics: Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
Health Sciences › Medicine › Gastroenterology › Inflammatory Bowel Disease
Keywords: primary sclerosing cholangitis, bile duct inflammation, liver fibrosis, portal hypertension, hepatobiliary cancer, cholangiocarcinoma, gallbladder cancer, inflammatory bowel disease, colorectal cancer risk, liver transplantation, prognosis, pathogenesis, immune-mediated liver disease
MeSH terms: Bile Ducts, Gallbladder Neoplasms, Humans, Hypertension, Portal, Liver Neoplasms, Prognosis, Cholangitis, Sclerosing, Liver Transplantation, Liver Failure, Cholangiocarcinoma

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Primary Sclerosing Cholangitis: Diagnosis, Prognosis, and Management

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