PSC is a cholestatic liver disease that results in chronic inflammation, fibrosis, and structuring of the intra- and extrahepatic biliary tree. It can ultimately lead to cirrhosis and hepatic failure in some patients. PSC is frequently seen in patients with inflammatory bowel disease (ulcerative colitis and Crohns disease). Figure 1 shows diffuse bilateral intrahepatic strictures and loss of high intrahepatic ducts (pruning). These strictures cause episodes of cholangitis with fever. In PBC, the cholangiogram is usually normal. Choledocholithiasis would show stones in the common bile duct (CBD) on ERCP, which are absent in this case. SSC often presents as diffuse intra- and extrahepatic stone disease or develops following a bile duct injury, causing a biliary stricture. A patient with hepatitis A would likely have a normal cholangiogram.
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