Primary biliary cholangitis

Last updated: September 7, 2023

Summarytoggle arrow icon

Primary biliary cholangitis (PBC; also known as primary biliary cirrhosis) is a chronic progressive liver disease of autoimmune origin that is characterized by destruction of the intralobular bile ducts. The pathogenesis of PBC is unclear. PBC is frequently associated with other autoimmune conditions and primarily affects middle-aged women. In the early stages, PBC is typically asymptomatic. Fatigue is the most common initial symptom. In advanced disease, increased fibrotic changes lead to typical signs of cholestasis (e.g., jaundice), portal hypertension (e.g., ascites, gastrointestinal bleeding), and severe hypercholesterolemia (e.g., xanthomas, xanthelasmas). Elevated alkaline phosphatase (ALP) levels, antimitochondrial antibodies (AMA), and liver biopsy findings can establish the diagnosis. Management involves supportive care, e.g., management of cholestasis-associated pruritus, and slowing disease progression with ursodeoxycholic acid. Liver transplantation is the only definitive treatment.

Epidemiologytoggle arrow icon

  • Sex: : > (∼ 9:1) [1]
  • Age range: : 30–65 years [2]

Epidemiological data refers to the US, unless otherwise specified.

Etiologytoggle arrow icon

Pathophysiologytoggle arrow icon

  • Inflammation and progressive destruction (likely due to an autoimmune reaction) of the small and medium-sized intrahepatic bile ducts (progressive ductopenia) defective bile duct regeneration chronic cholestasis secondary hepatocyte damage due to increased concentration of toxins that typically get excreted via bile gradual portal and periportal fibrotic changes → liver failure → liver cirrhosis and portal hypertension (in advanced stage) [10]

Clinical featurestoggle arrow icon

Patients with PBC are usually initially asymptomatic. [3][4]

Diagnosticstoggle arrow icon

Approach [3]

Laboratory studies [3]

The combination of hyperbilirubinemia with hypoalbuminemia, thrombocytopenia, and increased INR suggests decompensated cirrhosis, which indicates a poor prognosis. [4]

Liver biopsy [3]

  • Most patients do not require a liver biopsy to confirm PBC.
  • Obtain in certain patients to either:
    • Confirm PBC diagnosis in patients without characteristic autoantibodies
    • Evaluate for alternative diagnoses or concomitant liver disease [3]
  • Supportive findings
    • Nonsuppurative cholangitis and destruction of small and medium bile ducts [4]
    • See “Pathology” below for findings according to the histopathological stage of disease.


Imaging is not routinely indicated but may be used to exclude alternative diagnoses and/or evaluate for complications. See also “Diagnostics” in “Jaundice and cholestasis.”

Pathologytoggle arrow icon

Histopathological stages

Differential diagnosestoggle arrow icon

The differential diagnoses listed here are not exhaustive.

Treatmenttoggle arrow icon

General principles

Liver transplantation is the only definitive treatment, although most cases of PBC can be managed effectively with pharmacotherapy.

Pharmacotherapy [3][4]

Because bile acid sequestrants can interfere with UDCA absorption, these medications should not be taken at the same time. [3]

Supportive care [3][4]

Monitoring and screening [3][4]

Complicationstoggle arrow icon

We list the most important complications. The selection is not exhaustive.

Referencestoggle arrow icon

  1. Boonstra K, Beuers U, Ponsioen CY. Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: A systematic review. J Hepatol. 2012; 56 (5): p.1181-1188.doi: 10.1016/j.jhep.2011.10.025 . | Open in Read by QxMD
  2. Poupon R. Clinical manifestations, diagnosis, and prognosis of primary biliary cholangitis (primary biliary cirrhosis). In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. Last updated: October 16, 2015. Accessed: December 26, 2016.
  3. Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases. Hepatology. 2018.doi: 10.1002/hep.30145 . | Open in Read by QxMD
  4. Younossi ZM, Bernstein D, Shiffman ML, et al. Diagnosis and Management of Primary Biliary Cholangitis. Am J Gastroenterol. 2019; 114 (1): p.48-63.doi: 10.1038/s41395-018-0390-3 . | Open in Read by QxMD
  5. Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med. 2005; 353 (12): p.1261-73.doi: 10.1056/NEJMra043898 . | Open in Read by QxMD
  6. Callichurn K, Cvetkovic L, Therrien A, Vincent C, Hétu PO, Bouin M. Prevalence of Celiac Disease in Patients with Primary Biliary Cholangitis. J Can Assoc Gastroenterol. 2020; 4 (1): p.44-47.doi: 10.1093/jcag/gwz039 . | Open in Read by QxMD
  7. Bizzaro N, Tampoia M, Villalta D, et al. Low specificity of anti-tissue transglutaminase antibodies in patients with primary biliary cirrhosis. J Clin Lab Anal. 2006; 20 (5): p.184-189.doi: 10.1002/jcla.20130 . | Open in Read by QxMD
  8. Siegel JL, Luthra H, Donlinger J, Angulo P, Lindor K. Association of Primary Biliary Cirrhosis and Rheumatoid Arthritis. J Clin Rheumatol. 2003; 9 (6): p.340-343.doi: 10.1097/01.rhu.0000099623.30805.2f . | Open in Read by QxMD
  9. Caramella C, Avouac J, Sogni P, Puéchal X, Kahan A, Allanore Y. Association between rheumatoid arthritis and primary biliary cirrhosis. Joint Bone Spine. 2007; 74 (3): p.279-281.doi: 10.1016/j.jbspin.2006.06.012 . | Open in Read by QxMD
  10. Gulamhusein AF, Hirschfield GM. Pathophysiology of primary biliary cholangitis. Best Practice & Research Clinical Gastroenterology. 2018; 34-35: p.17-25.doi: 10.1016/j.bpg.2018.05.012 . | Open in Read by QxMD
  11. Wah-Suarez MI, Danford CJ, Patwardhan VR, Jiang ZG, Bonder A. Hyperlipidaemia in primary biliary cholangitis: treatment, safety and efficacy. Frontline Gastroenterol. 2019; 10 (4): p.401-408.doi: 10.1136/flgastro-2018-101124 . | Open in Read by QxMD
  12. Sigal S, Mitchell O, Feldman D, Diakow M. The pathophysiology of thrombocytopenia in chronic liver disease. Hepat Med. 2016: p.39.doi: 10.2147/hmer.s74612 . | Open in Read by QxMD
  13. Peck-Radosavljevic M. Thrombocytopenia in chronic liver disease. Liver Int. 2016; 37 (6): p.778-793.doi: 10.1111/liv.13317 . | Open in Read by QxMD
  14. Leung KK, Deeb M, Hirschfield GM. Review article: pathophysiology and management of primary biliary cholangitis. Aliment Pharmacol Ther. 2020.doi: 10.1111/apt.16023 . | Open in Read by QxMD
  15. Paumgartner G. Ursodeoxycholic acid in cholestatic liver disease: Mechanisms of action and therapeutic use revisited. Hepatology. 2002; 36 (3): p.525-531.doi: 10.1053/jhep.2002.36088 . | Open in Read by QxMD
  16. Lindor KD, Bowlus CL, Boyer J, Levy C, Mayo M. Primary biliary cholangitis: 2021 practice guidance update from the American Association for the Study of Liver Diseases. Hepatology. 2021; 75 (4): p.1012-1013.doi: 10.1002/hep.32117 . | Open in Read by QxMD
  17. Reau N. Hepatic ductopenia and vanishing bile duct syndrome. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. Last updated: March 7, 2016. Accessed: December 26, 2016.
  18. Pyrsopoulos NT. Primary Biliary Cholangitis (Primary Biliary Cirrhosis). In: BS Anand, Primary Biliary Cholangitis (Primary Biliary Cirrhosis). New York, NY: WebMD. Updated: June 3, 2016. Accessed: December 26, 2016.

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