Primary Sclerosing Cholangitis (PSC)
| Overview |
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- Primary Sclerosing Cholangitis (PSC) is a disease of unknown etiology and pathogenesis which leads to inflammatory obliteration of both the intra-hepatic and extra-hepatic biliary tree.
| Morphology |
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- A primarily lymphocytic infiltrate is observed around both extra-hepatic and intra-hepatic bile ducts. Over time, a progressive ringing fibrosis surrounds the atrophying bile ducts, termed "Onion-skin Fibrosis", leading to their narrowing and ultimately total obliteration. These morphological changes occur in a segmental pattern, leaving areas of intervening normal bile duct which then undergo morphological changes associated with cholestasis due to intra-luminal build up of bile (See: cholestasis). Eventually a picture of cirrhosis similar to that of secondary biliary cirrhosis emerges.
| Clinical Consequences |
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- Patients will display the general clinical features of cholestasis: Pruritis, xanthomas, jaundice, steatorrhea, and deficiencies of fat soluble vitamins A, D, E, and K. Eventually, signs and symptoms of cirrhosis and hepatic failure will supervene.
| Incidence |
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- Over 50% of patients with PSC also possess Ulcerative Colitis. Note however that only 4% of patients with Ulcerative Colitis possess PSC.