Primary sclerosing cholangitis: Diagnosis, prognosis, and management

Siddharth Singh, Jayant A. Talwalkar

Research output: Contribution to journalArticle

55 Citations (Scopus)

Abstract

Primary sclerosing cholangitis (PSC) is a chronic immunemediated 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.

Original languageEnglish (US)
Pages (from-to)898-907
Number of pages10
JournalClinical Gastroenterology and Hepatology
Volume11
Issue number8
DOIs
StatePublished - Aug 2013

Fingerprint

Sclerosing Cholangitis
Population
Gallbladder Neoplasms
Cholangiocarcinoma
Mortality
Liver
Liver Failure
Immunologic Factors
Portal Hypertension
Bile Ducts
Inflammatory Bowel Diseases
Liver Transplantation
Colorectal Neoplasms
Neoplasms
Fibrosis
Chronic Disease
Inflammation
Therapeutics
Research

Keywords

  • Primary sclerosing cholangitis (PSC)

ASJC Scopus subject areas

  • Gastroenterology
  • Hepatology

Cite this

Primary sclerosing cholangitis : Diagnosis, prognosis, and management. / Singh, Siddharth; Talwalkar, Jayant A.

In: Clinical Gastroenterology and Hepatology, Vol. 11, No. 8, 08.2013, p. 898-907.

Research output: Contribution to journalArticle

Singh, Siddharth ; Talwalkar, Jayant A. / Primary sclerosing cholangitis : Diagnosis, prognosis, and management. In: Clinical Gastroenterology and Hepatology. 2013 ; Vol. 11, No. 8. pp. 898-907.
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