What is the role of primary sclerosing cholangitis (PSC) in the etiology of biliary obstruction?

Updated: Oct 16, 2019
  • Author: Jennifer Lynn Bonheur, MD; Chief Editor: Vinay Kumar Kapoor, MBBS, MS, FRCS, FAMS  more...
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PSC is most common in men aged 20-40 years, and the cause is unknown. However, PSC is commonly associated with inflammatory bowel disease (IBD), most commonly in patients with pancolitis. IBD (the vast majority being ulcerative colitis) is present in 60-80% of patients with PSC, and PSC is found in approximately 3% of patients with ulcerative colitis. PSC is characterized by diffuse inflammation of the biliary tract, causing fibrosis and stricture of the biliary system. It generally manifests as a progressive obstructive jaundice and is most readily diagnosed based on findings from endoscopic retrograde cholangiopancreatography (ERCP).

AIDS-related cholangiopathy manifests as abdominal pain and elevated liver function test results, suggesting obstruction. The etiology of this disorder in patients who are HIV-positive is thought to be infectious (cytomegalovirus, Cryptosporidium species, and microsporidia have been implicated). Direct cholangiography often reveals abnormal findings in the intrahepatic and extrahepatic ducts that may closely resemble PSC.

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