Primary Sclerosing Cholangitis (PSC)

1. Pathogenesis:

• Diffuse inflammatory process of biliary tract.
   
• Unknown etiology.
   
• 70% of patients are male; 70% are younger than 45 when diagnosed; 70% have ulcerative colitis.
   
• Median survival of 12 years.
   
• Treatment: liver transplant.
   
• Complications: cholangiocarcinoma (in 15%).
   

2. Radiographic findings:

• Both intra and extrahepatic ducts affected in 85-90%.
   
• Early findings: segmental strictures without prestenotic dilation.
   
• Late findings: irregular ducts, strictures, bands, beading, filling defects, diverticula, and prunned tree appearance due to diminished aborization of the intrahepatic ducts.
   
• Figures: (A) Diagram of a typical appearance of PSC on ERCP. (B) Actual ERCP demonstrating primary sclerosing cholangitis. 30 y.o. male with elevated bilirubin and alkaline phosphatase. Intrahepatic and extrahepatic ducts show irregular contours with segmental strictures and beading. Note similar although milder changes in main pancreatic duct.

            
  A                                                                    B                                               

• Figures: PSC in 64 y.o. female with ulcerative cholangitis. (A) T-tube cholangiogram shows typical changes of PSC in both intrahepatic and extrahepatic ducts. (B) Single-contrast barium enema shows diffuse narrowing of transverse colon and loss of haustration, typical of ulcerative colitis.
   

             
A                                                           B