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Primary sclerosing cholangitis.

Worthington J, Chapman R - Orphanet J Rare Dis (2006)

Bottom Line: PSC is thought to be immune mediated and is often associated with inflammatory bowel disease, especially ulcerative colitis.Patients who are asymptomatic at diagnosis, the majority of whom will develop progressive disease, have a survival rate greater than 70% at 16 years after diagnosis.Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from PSC, although high dose ursodeoxycholic acid may have a beneficial effect.

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Affiliation: Department of Gastroenterology, John Radcliffe Hospital, Headington, Oxford, OX3 9DU, UK. joyworthington@hotmail.com

ABSTRACT
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown aetiology characterised by inflammation and fibrosis of the biliary tree. The mean age at diagnosis is 40 years and men are affected twice as often as women. There is a reported annual incidence of PSC of 0.9-1.31/100,000 and point prevalence of 8.5-13.6/100,000. The onset of PSC is usually insidious and many patients are asymptomatic at diagnosis or have mild symptoms only such as fatigue, abdominal discomfort and pruritus In late stages, splenomegaly and jaundice may be a feature. In most, the disease progresses to cirrhosis and liver failure. Cholangiocarcinoma develops in 8-30% of patients. PSC is thought to be immune mediated and is often associated with inflammatory bowel disease, especially ulcerative colitis. The disease is diagnosed on typical cholangiographic and histological findings and after exclusion of secondary sclerosing cholangitis. Median survival has been estimated to be 12 years from diagnosis in symptomatic patients. Patients who are asymptomatic at diagnosis, the majority of whom will develop progressive disease, have a survival rate greater than 70% at 16 years after diagnosis. Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from PSC, although high dose ursodeoxycholic acid may have a beneficial effect.

No MeSH data available.


Related in: MedlinePlus

Histological features of primary sclerosing cholangitis.
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Figure 2: Histological features of primary sclerosing cholangitis.

Mentions: In early stages, changes associated with PSC can be focal and may be missed on liver biopsy or the changes may be non-specific. The characteristic finding is of concentric "onion-skin" fibrosis surrounding the bile ducts (See Figure 2). Other bile duct abnormalities may include necrosis of epithelial cells, inflammatory infiltrates and fibrosis. There may be intrahepatic bile duct proliferation with ductopaenia or oedema in some portal tracts. In advanced cases, loss of bile ducts can be a feature (vanishing bile duct syndrome). The parenchyma usually shows non-specific changes [30]. Copper storage protein may be seen in advanced cases.


Primary sclerosing cholangitis.

Worthington J, Chapman R - Orphanet J Rare Dis (2006)

Histological features of primary sclerosing cholangitis.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC1636629&req=5

Figure 2: Histological features of primary sclerosing cholangitis.
Mentions: In early stages, changes associated with PSC can be focal and may be missed on liver biopsy or the changes may be non-specific. The characteristic finding is of concentric "onion-skin" fibrosis surrounding the bile ducts (See Figure 2). Other bile duct abnormalities may include necrosis of epithelial cells, inflammatory infiltrates and fibrosis. There may be intrahepatic bile duct proliferation with ductopaenia or oedema in some portal tracts. In advanced cases, loss of bile ducts can be a feature (vanishing bile duct syndrome). The parenchyma usually shows non-specific changes [30]. Copper storage protein may be seen in advanced cases.

Bottom Line: PSC is thought to be immune mediated and is often associated with inflammatory bowel disease, especially ulcerative colitis.Patients who are asymptomatic at diagnosis, the majority of whom will develop progressive disease, have a survival rate greater than 70% at 16 years after diagnosis.Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from PSC, although high dose ursodeoxycholic acid may have a beneficial effect.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Gastroenterology, John Radcliffe Hospital, Headington, Oxford, OX3 9DU, UK. joyworthington@hotmail.com

ABSTRACT
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown aetiology characterised by inflammation and fibrosis of the biliary tree. The mean age at diagnosis is 40 years and men are affected twice as often as women. There is a reported annual incidence of PSC of 0.9-1.31/100,000 and point prevalence of 8.5-13.6/100,000. The onset of PSC is usually insidious and many patients are asymptomatic at diagnosis or have mild symptoms only such as fatigue, abdominal discomfort and pruritus In late stages, splenomegaly and jaundice may be a feature. In most, the disease progresses to cirrhosis and liver failure. Cholangiocarcinoma develops in 8-30% of patients. PSC is thought to be immune mediated and is often associated with inflammatory bowel disease, especially ulcerative colitis. The disease is diagnosed on typical cholangiographic and histological findings and after exclusion of secondary sclerosing cholangitis. Median survival has been estimated to be 12 years from diagnosis in symptomatic patients. Patients who are asymptomatic at diagnosis, the majority of whom will develop progressive disease, have a survival rate greater than 70% at 16 years after diagnosis. Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from PSC, although high dose ursodeoxycholic acid may have a beneficial effect.

No MeSH data available.


Related in: MedlinePlus