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Liver and intrahepatic bile ducts-nontumor

Biliary tract disease

Primary sclerosing cholangitis

Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 15 May 2012, last major update May 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.


● 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)
Xray: beading of barium column in cholangiogram due to irregular strictures and dilations of affected bile ducts
● 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)


● Elevated alkaline phosphatase, IgM, IgG
● Variable bilirubin
● 2/3 are p-ANCA positive (also positive in ulcerative colitis without PSC)
● Negative antimitochondrial antibody


● 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

Liver with white fibrous cords

Micro 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
● Post-transplant recurrences exhibit bile duct structuring and nonspecific autoimmune hepatitis with variable fibrosis


1. Inflammation without expansion of portal tracts or piecemeal necrosis
2. Piecemeal necrosis or fibrosis without bridging
3. Bridging necrosis or fibrosis
4. Cirrhosis

Micro images

Bile duct with marked periductal sclerosis

Interlobular bile duct with periductal and concentric fibrosis, scar at site of missing bile duct,
presence of copper (Orcein stain) shows chronic cholestasis

Differential diagnosis

● Primary biliary cirrhosis (florid duct lesions, not fibrous-obliterative lesions), chronic viral hepatitis (more prominent lymphocytic infiltrate and less periportal copper deposition), cholangiocarcinoma (may have similar radiologic findings)

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

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