Primary Sclerosing Cholangitis

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What is Primary Sclerosing Cholangitis?

Primary Sclerosing Cholangitis is a disease affecting the bile ducts within and outside the liver.

There is inflammation, fibrosis and strictures of the intra and extra-hepatic bile ducts.

Who gets Primary Sclerosing Cholangitis?

This disease is not very common -7/100,000 is estimated to be the prevalence. The disease is seven times more common in men than women (70% of patients affected are men).

The usual age of presentation is around 40 years of age.

Predisposing Factors

Predisposing factors include being of the male sex, having a history of inflammatory bowel disease, and AIDS.


Progression

This condition is a progressive liver failure that leads to biliary cirrhosis and ultimately liver failure. The survival of patients whether symptomatic or asymptomatic is reported to be below the normal population. There are currently no treatments that halt disease progression.

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