Liver and intrahepatic bile ducts - nontumor
Biliary tract disease
Primary sclerosing cholangitis

Topic Completed: 1 May 2012

Revised: 14 November 2019

Copyright: 2002-2019,, Inc.

PubMed Search: Primary sclerosing cholangitis[TI] liver[TI] free full text[sb]

Komal Arora, M.D.
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Cite this page: Arora K. Primary sclerosing cholangitis. website. Accessed December 5th, 2019.
Definition / general
  • 65% men, usually under 45 years
  • Possibly autoimmune, 50 - 70% also have inflammatory bowel disease (particularly ulcerative colitis, although only 4% with ulcerative colitis have primary sclerosing cholangitis)
  • Secondary sclerosing cholangitis: due to stones, prior surgery
  • Symptoms: fatigue, pruritis, jaundice, right upper quadrant pain / tenderness
  • Increased risk for cholangiocarcinoma
  • 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, Am J Surg Pathol 2010;34:27)
  • PSC with tissue IgG4 positivity has a more aggressive clinical course (shorter time to transplant, higher likelihood of recurrence) than IgG4 negative PSC (Am J Surg Pathol 2010;34:88)
Staging / staging classifications
  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 alkaline phosphatase, IgM, IgG
  • Variable bilirubin
  • 2/3 are pANCA positive (also positive in ulcerative colitis without PSC)
  • Negative antimitochondrial antibody
Radiology description
  • Xray: beading of barium column in cholangiogram due to irregular strictures and dilations of affected bile ducts
  • Liver transplant since no effective medical therapy (associated with autoimmune liver disease in 42% and recurrence in 33%, Hum Pathol 2003;34:1127)
Gross description
  • Periductal portal tract fibrosis, segmental stenosis of extrahepatic and intrahepatic bile ducts
Gross images

Images hosted on other servers:

Liver with white fibrous cords

Microscopic (histologic) description
  • Fibrosing cholangitis of intra and extrahepatic bile ducts (large and small) 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, often elongated; variable portal eosinophils
  • Cirrhotic nodules, when present, have "jigsaw" pattern due to portal to portal nature of fibrosis; also sclerosis and prominent lymphoid aggregates in gallbladder
  • Posttransplant recurrences exhibit bile duct structuring and nonspecific autoimmune hepatitis with variable fibrosis
Microscopic (histologic) images

Images hosted on other servers:

Bile duct with marked periductal sclerosis

Differential diagnosis
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