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Primary biliary cholangitis

Last updated: August 2, 2021

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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; however, it primarily affects middle-aged women and is frequently associated with other autoimmune conditions. In the early stages, PBC is typically asymptomatic. Fatigue is the most common initial complaint. In advanced disease, increased fibrotic changes lead to typical signs of cholestasis (e.g., jaundice) and portal hypertension (e.g., ascites, gastrointestinal bleeding). Elevated cholestasis parameters (ALP, γ-GT, bilirubin) in addition to antimitochondrial antibodies (AMAs) help establish the diagnosis. Management consists of slowing disease progression with ursodeoxycholic acid and relieving symptoms. Liver transplantation is the only definitive treatment.

  • Sex: > (∼ 9:1) [1]
  • Age range: 30–65 years [2]
  • Miscellaneous: most common cause of vanishing bile duct syndrome (a condition in which intrahepatic ducts are progressively destroyed) [3]

Epidemiological data refers to the US, unless otherwise specified.

  • The exact cause of PBC is still unclear; however, it is considered as an autoimmune disease.
  • Associated with other autoimmune conditions (see “Clinical features” below)
  • Positive family history is a predisposing factor.

References:[4]

Patients are initially often asymptomatic. Signs and symptoms are mainly due to the resulting cholestasis, liver cirrhosis, and portal hypertension.

References:[2][7]

Histopathological stages

The differential diagnoses listed here are not exhaustive.

There is no cure for PBC. Treatment consists of slowing disease progression and alleviating symptoms. Liver transplantation is the only definitive treatment.

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

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  2. Poupon R. Clinical manifestations, diagnosis, and prognosis of primary biliary cholangitis (primary biliary cirrhosis). In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/clinical-manifestations-diagnosis-and-prognosis-of-primary-biliary-cholangitis-primary-biliary-cirrhosis.Last updated: October 16, 2015. Accessed: December 26, 2016.
  3. Ludwig J, Wiesner RH, LaRusso NF. Idiopathic adulthood ductopenia. A cause of chronic cholestatic liver disease and biliary cirrhosis.. J Hepatol. 1988; 7 (2): p.193-9. doi: 10.1016/s0168-8278(88)80482-3 . | Open in Read by QxMD
  4. 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
  5. 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
  6. 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
  7. 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
  8. Reau N. Hepatic ductopenia and vanishing bile duct syndrome. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/hepatic-ductopenia-and-vanishing-bile-duct-syndrome.Last updated: March 7, 2016. Accessed: December 26, 2016.
  9. Pyrsopoulos NT. Primary Biliary Cholangitis (Primary Biliary Cirrhosis). In: BS Anand, Primary Biliary Cholangitis (Primary Biliary Cirrhosis). New York, NY: WebMD. http://emedicine.medscape.com/article/171117. Updated: June 3, 2016. Accessed: December 26, 2016.