Liver & intrahepatic bile ducts

Biliary tract disease

Secondary biliary cirrhosis

Editor-in-Chief: Debra L. Zynger, M.D.
Raul S. Gonzalez, M.D.

Last author update: 10 December 2020
Last staff update: 5 August 2022

Copyright: 2002-2024,, Inc.

PubMed Search: Secondary biliary cirrhosis

Raul S. Gonzalez, M.D.
Page views in 2023: 3,381
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Cite this page: Gonzalez RS. Secondary biliary cirrhosis. website. Accessed April 14th, 2024.
Definition / general
Essential features
  • Secondary, rather than primary, biliary pattern cirrhosis
  • Rare, given modern therapy for large duct obstruction
  • Not to be confused with secondary sclerosing cholangitis
ICD coding
  • ICD-10: K74.4 - secondary biliary cirrhosis
  • Now rare
  • Large bile duct injury induces cholestatic liver disease, with secondary inflammation, fibrosis, scarring and nodule formation (Methods Mol Biol 2019;1981:237)
Clinical features
  • Depending on cause of biliary obstruction, patients may not be symptomatic until cirrhosis has already developed
  • Can be complicated by hepatolithiasis or portal hypertension (Arch Surg 1989;124:1301, Arch Surg 1968;96:604)
  • No particular serologic associations
Prognostic factors
Case reports
  • Relief of the biliary obstruction can halt progression
  • End stage disease requires liver transplant
Gross description
  • Cirrhotic liver with yellow-green discoloration
Microscopic (histologic) description
  • Same as biliary pattern cirrhosis of other causes, with jigsaw architecture, ductular reaction and cholestasis (Burt: Macsween's Pathology of the Liver, 7th Edition, 2018
  • May show septal edema, feathery degeneration, bile infarcts, bile lakes
  • Should not see florid duct lesions of primary biliary cholangitis
Microscopic (histologic) images

Contributed by Raul S. Gonzalez, M.D.

Biliary type cirrhosis

Sample pathology report
  • Liver, native, orthotopic transplantation:
    • Cirrhotic liver with biliary pattern injury and focal bile lakes (see comment)
    • Comment: The patient’s reported history of chronic large duct stricture is noted. The findings are consistent with secondary biliary cirrhosis. There is no evidence of malignancy. A trichrome stain confirms cirrhosis.
Differential diagnosis
  • Primary biliary cholangitis:
    • Florid duct lesions, antimitochondrial antibody
  • Primary sclerosing cholangitis:
    • Onion skin fibrosis, characteristic biliary imaging
  • Secondary sclerosing cholangitis:
    • Findings similar to primary sclerosing cholangitis but with an identified etiology
  • Sepsis:
    • Can mimic biliary pattern injury; may show ductular cholestasis; requires clinical correlation
Board review style question #1
    What is the most common cause of end stage liver disease in so called secondary biliary cirrhosis?

  1. Large duct obstruction
  2. Liver flukes
  3. Primary biliary cholangitis
  4. Secondary sclerosing cholangitis
Board review style answer #1
A. Large duct obstruction

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Reference: Secondary biliary cirrhosis
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