Primary sclerosing cholangitis: In primary sclerosing cholangitis (PSC), the bile ducts inside and outside the liver become inflamed and scarred. As the scarring increases, the ducts ... more about Primary sclerosing cholangitis.
Primary sclerosing cholangitis: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology that is recognized increasingly in children. More detailed information about the symptoms, causes, and treatments of Primary sclerosing cholangitis is available below.
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Chronic liver disease often undiagnosed: One study reported that 50% of patients with a chronic liver disease remain undiagnosed by their primary physician. The reasons are multifactorial. Possible conditions include chronic hepatitis, cirrhosis of the liver, hemochromatosis, or other types of liver condition. See introduction to liver disease or misdiagnosis of liver disease....read more »
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Prognosis for Primary sclerosing cholangitis: PSC is characterized by a slow insidious progression to cirrhosis. In adult patients, the median period of survival from the time of diagnosis is 9-11 years. The median period of survival is shorter for patients who are symptomatic at the time of diagnosis.
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In primary sclerosing cholangitis (PSC), the bile ducts inside and outside the liver become inflamed and scarred. As the scarring increases, the ducts become blocked. The ducts are important because they carry bile out of the liver. Bile is a liquid that helps break down fat in food. If the ducts are blocked, bile builds up in the liver and damages liver cells. Eventually, PSC can cause liver failure. (Source: excerpt from Primary Sclerosing Cholangitis: NIDDK)
Chronic inflammatory disease of the BILIARY TRACT. It is characterized by fibrosis and hardening of the intrahepatic and extrahepatic biliary ductal systems leading to bile duct strictures, CHOLESTASIS, and eventual BILIARY CIRRHOSIS. - (Source - Diseases Database)
Primary sclerosing cholangitis is listed as a "rare disease" by the Office of
Rare Diseases (ORD) of the National Institutes of Health
(NIH). This means that Primary sclerosing cholangitis, or a subtype of Primary sclerosing cholangitis,
affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)
Ophanet, a consortium of European partners,
currently defines a condition rare when it affects 1 person per 2,000.
They list Primary sclerosing cholangitis as a "rare disease".
Source - Orphanet
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