Gallbladder & extrahepatic bile ducts

Primary sclerosing cholangitis

Last author update: 1 September 2012
Last staff update: 30 December 2020

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PubMed Search: Primary sclerosing cholangitis extrahepatic bile ducts[TIAB]

Hanni Gulwani, M.B.B.S.
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Cite this page: Gulwani H. Primary sclerosing cholangitis. website. Accessed June 2nd, 2023.
Definition / general
  • Chronic cholestatic disorder of unknown origin (possibly autoimmune) involving entire biliary tract from ampulla of Vater to small intrahepatic bile ducts or gallbladder
  • Much less common than secondary sclerosing cholangitis
Clinical features
  • Rule of 70s: 70% men, 70% have chronic inflammatory bowel disease (particularly ulcerative colitis which is usually detected first; only 4% with ulcerative colitis have primary sclerosing cholangitis, which is unaffected by colectomy), 70% younger than age 45
  • Also associated with chronic pancreatitis (15 - 25%), Riedel thyroiditis, retroperitoneal and mediastinal fibrosis, orbital pseudotumor, Sjögren syndrome, angioimmunoblastic lymphadenopathy
  • End stage disease is associated with hyperplasia of glands of extrahepatic bile ducts, with low incidence of dysplasia and adenocarcinoma (Am J Surg Pathol 2003;27:349)
  • Bile ducts in PSC show two distinct pathways of dysplasia-carcinoma, based on differences in cell morphology, growth patterns, immunophenotypes and grade of malignancy (Histopathology 2011;59:1100)
  • Symptoms: fatigue, jaundice, pruritis

  • Biliary cirrhosis and liver failure in all cases with median survival 9 - 12 years
  • Cholangiocarcinoma (10 - 43%), colon carcinoma

  1. Inflammation without expansion of portal tracts or piecemeal necrosis
  2. Piecemeal necrosis or fibrosis without bridging
  3. Bridging necrosis or fibrosis
  4. Cirrhosis
  • Elevated serum alkaline phosphatase, IgM, IgG
  • Variable bilirubin
  • May be pANCA positive
Radiology description
  • Xray: beading of barium column in cholangiogram due to irregular strictures and dilations of affected bile ducts
Case reports
Gross description
  • Periductal portal tract fibrosis, segmental stenosis of extrahepatic and intrahepatic bile ducts
Microscopic (histologic) description
  • Fibrosing cholangitis of intra and extrahepatic bile ducts with lymphocytic infiltration
  • Progressive atrophy of bile duct epithelium and obliteration of the lumen, diffuse bile ductular proliferation
  • "Onion skin" fibrosis around affected ducts, which later disappear, leaving cord-like fibrous scar
  • Remaining ducts are ectatic and inflamed
  • Mild to florid hyperplasia often noted
  • Recurrence after transplant exhibits bile duct structuring and nonspecific autoimmune hepatitis with variable fibrosis
  • Variable portal eosinophils
Microscopic (histologic) images

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Portal tract fibrosis

Bile duct with marked periductal sclerosis

Differential diagnosis
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