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Joseph Junewick, MD FACR
over 11 years ago
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Primary Sclerosing Cholangitis

Case Detail

Anatomy: Gastrointestinal
Joseph Junewick, MD FACR
Diagnostic Category: Infectious-Inflammatory
Created: over 8 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: FL US
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Teenager with Crohn disease and jaundice.

Case Images


Primary Sclerosing Cholangitis


FL – Irregularly narrowed intrahepatic and extrahepatic bile ducts.

US – Mural thickening of the bile ducts.


Hepatobiliary diseases are common in IBD patients. Primary sclerosing cholangitis, a chronic cholestatic disorder characterized by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts, is the most common. Other associated abnormalities include hepatomegaly (related to steatosis), cholelithiasis (altered bile salt resorption related to ileal disease), hepatic abscesses (portal bacteremia from bowel inflammation), and portal thrombosis (prothrombotic effects of inflammation.

Primary sclerosing cholangitis is more frequent in males and patients with ulcerative colitis. Symptoms of primary sclerosis cholangitis are fatigue, pruritus, jaundice, and abdominal discomfort but it is not rare that the isolate finding of abnormalities in liver biochemical markers. Biopsy or cholangiography is often necessary for the diagnosis.

Anti-colonic mucosa auto-antibodies can cross react with biliary epithelium has been identified[. Colonic epithelial protein and the human tropomyosin isoform 5 are expressed in the colon and also in the biliary tract, skin, eyes, and joints which may may be implicated in the autoimmune attack in extraintestinal organs of IBD patients. Patients with sclerosing cholangitis are at much higher risk for colonic and biliary cancer, metabolic bone disease, and folate deficiency. Median survival time is between 9-12 years from the time of diagnosis.


Danese S, Semeraro S, Papa A, et al. Extraintestinal manifestations in inflammatory bowel disease. World J Gastroenterol (2005); 14:7227-7236.

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