Primary sclerosing cholangitis

L. M. Alba, P. Angulo, Keith Lindor

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic syndrome of unknown etiology frequently associated with inflammatory bowel disease and characterized by diffuse inflammation and fibrosis of the intra and/or extrahepatic bile ducts. Recent studies seem to favor autoimmunity in the context of a genetic predisposition as the most likely underlying mechanism for the development of the disease, however our knowledge on the pathogenesis of PSC is still incomplete and further work is needed. The most common manifestations are fatigue, pruritus, jaundice and abdominal pain; however, the increasing use of invasive cholangiography has led to diagnosing this condition in a high proportion of asymptomatic patients. PSC usually follows a progressive course leading to biliary cirrhosis with complications of portal hypertension and hepatic failure. Patients with PSC also may develop a number of other complications, including bacterial cholangitis, dominant biliary strictures, conditions of chronic cholestasis, colorectal cancer and cholangiocarcinoma. Currently, no medical therapy aimed at disrupting disease progression is available, although high-dose ursode-oxycholic acid and other medicines are being evaluated in clinical trials. A better understanding of the pathogenesis of the disease will serve as a guide for evaluating new medical approaches. Liver transplantation is the only therapeutic alternative that improves survival in patients with end-stage PSC. Prognostic models are useful in determining the timing of liver transplantation.

Original languageEnglish (US)
Pages (from-to)99-113
Number of pages15
JournalMinerva Gastroenterologica e Dietologica
Volume48
Issue number2
StatePublished - 2002
Externally publishedYes

Fingerprint

Sclerosing Cholangitis
Liver Transplantation
Extrahepatic Bile Ducts
Cholangitis
Cholangiocarcinoma
Cholangiography
Biliary Liver Cirrhosis
Cholestasis
Liver Failure
Portal Hypertension
Pruritus
Genetic Predisposition to Disease
Jaundice
Autoimmunity
Inflammatory Bowel Diseases
Abdominal Pain
Fatigue
Disease Progression
Colorectal Neoplasms
Pathologic Constriction

Keywords

  • Cholangitis, sclerosing, diagnosis
  • Cholangitis, sclerosing, epidemiology
  • Cholangitis, sclerosing, etiology
  • Cholangitis, sclerosing, therapy
  • Liver transplantation

ASJC Scopus subject areas

  • Gastroenterology
  • Internal Medicine
  • Nutrition and Dietetics
  • Endocrinology, Diabetes and Metabolism

Cite this

Primary sclerosing cholangitis. / Alba, L. M.; Angulo, P.; Lindor, Keith.

In: Minerva Gastroenterologica e Dietologica, Vol. 48, No. 2, 2002, p. 99-113.

Research output: Contribution to journalArticle

Alba, LM, Angulo, P & Lindor, K 2002, 'Primary sclerosing cholangitis', Minerva Gastroenterologica e Dietologica, vol. 48, no. 2, pp. 99-113.
Alba, L. M. ; Angulo, P. ; Lindor, Keith. / Primary sclerosing cholangitis. In: Minerva Gastroenterologica e Dietologica. 2002 ; Vol. 48, No. 2. pp. 99-113.
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