More common in males aged 20-40 years, it is an idiopathic, progressive, fibrosing inflammatory disorder of the biliary tree. Associated w...
More common in males aged 20-40 years, it is an idiopathic, progressive, fibrosing inflammatory disorder of the biliary tree. Associated with ulcerative colitis (UC), Riedel thyroiditis, Sjogren syndrome, cystic fibrosis (CF), retroperitoneal fibrosis, etc. Pathologically, there are multifocal areas of periductal fibrosis that narrow ducts with intervening normal ducts. The stenoses cause cholestasis, sepsis and biliary cirrhosis (hepatic failure). There is an 10 to 15 increased risk of cholangiocarcinoma.
US
• Areas of intrahepatic duct dilatation with strictures.
• Both intrahepatic and extrahepatic ducts are usually involved.
• Increased echogenicity of the portal triads.
• 10% have bile duct stones.
MRCP
• Irregular strictures of the intrahepatic/extrahepatic ducts.
• Classic ‘string of beads’ appearances with alternating segments of dilatation and stenosis.
• Pruned tree appearance due to obliterated peripheral ducts.
• Up to 25% have ductal diverticula.
Primary sclerosing cholangitis. Maximum intensity projection (MIP) image from an MRCP demonstrating multiple strictures of the intrahepatic ducts (white arrows).
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