Primary sclerosing cholangitis: diagnosis, prognosis, and management
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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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In a small percentage of patients, clinically significant portal hypertension in PSC may occur early in the disease course, even in people with PSC with F2 or F3 fibrosis in the absence of full-blown histologic cirrhosis.
“In a small percentage of patients, CSPH in PSC (in analogy to PBC) may occur early in the disease course, i.e. even in people with PSC with F2 or F3 fibrosis in the absence of full-blown histologic cirrhosis.250”
From EASL Clinical Practice Guidelines on sclerosing cholangitis (2022) · cites this paper as [250]
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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)
- 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
- 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
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › 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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