Liver & intrahepatic bile ducts
Biliary tract disease
Secondary biliary cirrhosis


Topic Completed: 6 January 2020

Minor changes: 24 September 2020

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PubMed Search: Secondary biliary cirrhosis[TI]


Raul S. Gonzalez, M.D.
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Cite this page: Gonzalez RS. Secondary biliary cirrhosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/liversecBC.html. Accessed November 26th, 2020.
Definition / general
Essential features
  • Secondary, rather than primary, biliary pattern cirrhosis
  • Rare, given modern therapy for large duct obstruction
Terminology
  • Not to be confused with secondary sclerosing cholangitis
ICD coding
  • ICD-10: K74.4 - secondary biliary cirrhosis
Epidemiology
  • Now rare
Pathophysiology
  • Large bile duct injury induces cholestatic liver disease, with secondary inflammation, fibrosis, scarring and nodule formation (Methods Mol Biol 2019;1981:237)
Etiology
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)
Laboratory
  • No particular serologic associations
Prognostic factors
Case reports
Treatment
  • 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
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 answer #1
A. Large duct obstruction

Reference: Secondary biliary cirrhosis

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