Chronic progressive cholestatic liver disease characterized by nonsuppurative destruction of interlobular bile ducts
Leads to advanced fibrosis, cirrhosis, and liver failure
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
Other Modality Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Idilman IS et al: Magnetic resonance imaging features in 283 patients with primary biliary cholangitis. Eur Radiol. 30(9):5139-48, 2020
Mané F et al: Treatment response in primary biliary cholangitis: the role of autoimmune hepatitis features. Ann Hepatol. 18(3):488-93, 2019
Czaja AJ et al: Autoimmune hepatitis overlap syndromes and liver pathology. Gastroenterol Clin North Am. 46(2):345-64, 2017
Kovač JD et al: Primary biliary cirrhosis and primary sclerosing cholangitis: an update on MR imaging findings with recent developments. J Gastrointestin Liver Dis. 25(4):517-24, 2016
Meng Y et al: The value of MRI in the diagnosis of primary biliary cirrhosis and assessment of liver fibrosis. PLoS One. 10(3):e0120110, 2015
Karlsen TH et al: Review article: controversies in the management of primary biliary cirrhosis and primary sclerosing cholangitis. Aliment Pharmacol Ther. 39(3):282-301, 2014
Granito A et al: Antinuclear antibodies as ancillary markers in primary biliary cirrhosis. Expert Rev Mol Diagn. 12(1):65-74, 2012
Chuang N et al: Update on the epidemiology of primary biliary cirrhosis. Expert Rev Gastroenterol Hepatol. 5(5):583-90, 2011
Grattagliano I et al: Long-term ursodeoxycholate improves circulating redox changes in primary biliary cirrhotic patients. Clin Biochem. 44(17-18):1400-4, 2011
Kim KA et al: The diagnosis and treatment of primary biliary cirrhosis. Korean J Hepatol. 17(3):173-9, 2011
Mendes F et al: Recurrent and de novo autoimmune liver diseases. Clin Liver Dis. 15(4):859-78, 2011
Gonzales E et al: Liver diseases related to MDR3 (ABCB4) gene deficiency. Front Biosci. 14:4242-56, 2009
Kuiper EM et al: Improved prognosis of patients with primary biliary cirrhosis that have a biochemical response to ursodeoxycholic acid. Gastroenterology. 136(4):1281-7, 2009
Watanabe T et al: Features of hepatocellular carcinoma in cases with autoimmune hepatitis and primary biliary cirrhosis. World J Gastroenterol. 15(2):231-9, 2009
Kita H et al: Pathogenesis of primary biliary cirrhosis. Clin Liver Dis. 7(4):821-39, 2003
MacQuillan GC et al: Liver transplantation for primary biliary cirrhosis. Clin Liver Dis. 7(4):941-56, ix, 2003
Blachar A et al: Primary biliary cirrhosis: clinical, pathologic, and helical CT findings in 53 patients. Radiology. 220(2):329-36, 2001
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
Primary biliary cholangitis (PBC)
Synonyms
Primary biliary cirrhosis
Definitions
Chronic progressive cholestatic liver disease characterized by nonsuppurative destruction of interlobular bile ducts
Leads to advanced fibrosis, cirrhosis, and liver failure
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Nuclear Medicine Findings
Other Modality Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
Idilman IS et al: Magnetic resonance imaging features in 283 patients with primary biliary cholangitis. Eur Radiol. 30(9):5139-48, 2020
Mané F et al: Treatment response in primary biliary cholangitis: the role of autoimmune hepatitis features. Ann Hepatol. 18(3):488-93, 2019
Czaja AJ et al: Autoimmune hepatitis overlap syndromes and liver pathology. Gastroenterol Clin North Am. 46(2):345-64, 2017
Kovač JD et al: Primary biliary cirrhosis and primary sclerosing cholangitis: an update on MR imaging findings with recent developments. J Gastrointestin Liver Dis. 25(4):517-24, 2016
Meng Y et al: The value of MRI in the diagnosis of primary biliary cirrhosis and assessment of liver fibrosis. PLoS One. 10(3):e0120110, 2015
Karlsen TH et al: Review article: controversies in the management of primary biliary cirrhosis and primary sclerosing cholangitis. Aliment Pharmacol Ther. 39(3):282-301, 2014
Granito A et al: Antinuclear antibodies as ancillary markers in primary biliary cirrhosis. Expert Rev Mol Diagn. 12(1):65-74, 2012
Chuang N et al: Update on the epidemiology of primary biliary cirrhosis. Expert Rev Gastroenterol Hepatol. 5(5):583-90, 2011
Grattagliano I et al: Long-term ursodeoxycholate improves circulating redox changes in primary biliary cirrhotic patients. Clin Biochem. 44(17-18):1400-4, 2011
Kim KA et al: The diagnosis and treatment of primary biliary cirrhosis. Korean J Hepatol. 17(3):173-9, 2011
Mendes F et al: Recurrent and de novo autoimmune liver diseases. Clin Liver Dis. 15(4):859-78, 2011
Gonzales E et al: Liver diseases related to MDR3 (ABCB4) gene deficiency. Front Biosci. 14:4242-56, 2009
Kuiper EM et al: Improved prognosis of patients with primary biliary cirrhosis that have a biochemical response to ursodeoxycholic acid. Gastroenterology. 136(4):1281-7, 2009
Watanabe T et al: Features of hepatocellular carcinoma in cases with autoimmune hepatitis and primary biliary cirrhosis. World J Gastroenterol. 15(2):231-9, 2009
Kita H et al: Pathogenesis of primary biliary cirrhosis. Clin Liver Dis. 7(4):821-39, 2003
MacQuillan GC et al: Liver transplantation for primary biliary cirrhosis. Clin Liver Dis. 7(4):941-56, ix, 2003
Blachar A et al: Primary biliary cirrhosis: clinical, pathologic, and helical CT findings in 53 patients. Radiology. 220(2):329-36, 2001
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