Liver & intrahepatic bile ducts
Biliary tract disease
Secondary biliary cirrhosis
Author: Raul S. Gonzalez, M.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Last author update: 10 December 2020
Last staff update: 5 August 2022
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Secondary biliary cirrhosis
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Pathophysiology | Etiology | Clinical features | Laboratory | Prognostic factors | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1Cite this page: Gonzalez RS. Secondary biliary cirrhosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/liversecBC.html. Accessed December 26th, 2024.
Definition / general
- Biliary pattern cirrhosis secondary to chronic obstruction of large bile ducts
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
- Potential causes of large duct obstruction include biliary atresia, choledochal cysts, cystic fibrosis, Alagille syndrome, gallstones, postsurgical duct damage/stricture, portal cavernoma cholangiopathy and carcinoma of pancreatic head or biliary tree (Bol Asoc Med P R 2012;104:51, Arq Gastroenterol 2019;56:300, J Clin Exp Hepatol 2014;4:S62)
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
- Length of time for development depends on specific etiology (Am J Dig Dis 1965;10:135)
Case reports
- 9 year old girl with graft versus host disease (Gastroenterology 1990;98:223)
- 13 year old girl with systemic phaeohyphomycosis (Trop Gastroenterol 2004;25:172)
- 38 with old man with cirrhosis secondary to ascending cholangitis and bile duct stone (Gastrointest Endosc 2016;84:198)
- 48 year old woman with emphysematous cholecystitis (ACG Case Rep J 2013;1:51)
- 65 year old woman with hepatoliths and hereditary hemorrhagic telangiectasia (Eur J Gastroenterol Hepatol 1995;7:999)
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.
- Cirrhotic liver with biliary pattern injury and focal bile lakes (see comment)
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?
- Large duct obstruction
- Liver flukes
- Primary biliary cholangitis
- Secondary sclerosing cholangitis
Board review style answer #1
