| [ Summary ] |
Primary sclerosing cholangitis (PSC), a chornic cholestatic liver disease of unknown origin, is characterized by ongoing inflammation, destruction, and fibrosis of intrahepatic and extrahepatic bile ducts. Over time, bile ducts become irregularly narrowed and obliterated, and small intrahepatic ducts disappear. Focal bile-duct dilatation proximal to areas of stricture produces a characteristic beaded appearance on cholangiography. The current criteria used to diagnose PSC are based on characteristic changes in the intrahepatic and extrahepatic biliary tree seen with ERCP or PTC. Before the diagnosis of PSC can be established, disorders that causing secondary sclerosing cholangitis must be ruled out. Laboratory tests in patients with PSC usually show a cholestatic pattern, but biochemical abbnormalities alone are never diagnostic. |