Home   Chapter Home   Jobs   Conferences   Fellowships   Books



Advertisement

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.

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)
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)

Laboratory
=========================================================================

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

Treatment
=========================================================================

● 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

Staging
=========================================================================

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


This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).