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Extrahepatic bile ducts

Non tumor

Primary sclerosing cholangitis

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 15 February 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● 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
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● Rule of 70’s: 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’s thyroiditis, retroperitoneal and mediastinal fibrosis, orbital pseudotumor, Sjogren’s 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

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

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

Laboratory
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● Elevated serum alkaline phosphatase, IgM, IgG
● Variable bilirubin
● May be p-ANCA positive

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

Case reports
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● 17 year old boy (Diagn Cytopathol 2012;40:337)

Treatment
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● Liver transplant (Hum Pathol 2003;34:1127, Am J Surg Pathol 2007;31:907) since no effective medical therapy

Gross description
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● Periductal portal tract fibrosis, segmental stenosis of extrahepatic and intrahepatic bile ducts

Micro description
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● 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

Micro images
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Portal tract fibrosis


Bile duct with marked periductal sclerosis

Differential diagnosis
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● Sclerosing, well differentiated adenocarcinoma

End of Extrahepatic bile ducts > Non tumor > Primary sclerosing cholangitis

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