Cirrhosis, Primary Biliary
- PBC primarily affects women; female:male ratio of 8 to 10:1 (1,2)
- Peak age: 40 to 60 years; rare in children (2)
- Rare, present in all parts of the world and all races and nationalities; more common in Northern European countries and in the Northern United States
- Annual incidence: 0.7 to 49/1,000,000
- Prevalence: 4 to 14/100,000 (2)
Etiology and Pathophysiology
- PBC is caused by a T-cell–mediated attack on biliary epithelial cells primarily small, intrahepatic bile ducts.
- Destruction causes cholestasis, which can lead to cirrhosis and liver failure.
- Bile duct destruction leads to retained bile acids and foamy hepatocyte degeneration (2).
- PBC primarily occurs in genetically susceptible people exposed to specific environmental triggers (1,2,3).
- Some studies have suggested that infection (bacterial and viral) may be a trigger.
- Clusters of cases suggest a possible role of a range of environmental toxins.
- Smoking increases risk of progression of fibrosis.
- PBC risk is increased following pregnancy-related pruritus or cholestasis in genetically susceptible individuals (1).
Commonly Associated Conditions
- PBC associated with: Sjögren syndrome, scleroderma, rheumatoid arthritis, lupus, autoimmune thyroiditis, Raynaud phenomenon, sarcoidosis, pulmonary fibrosis, polymyositis, celiac disease, and ulcerative colitis (2)
- 53% have one or more concurrent autoimmune conditions (1,2).
- Overlap syndrome with autoimmune hepatitis in 5–19%. Diagnosis is difficult and prognosis worse (1).
- Cirrhotic PBC increases relative risk of hepatocellular carcinoma (HCC). HCC occurs 1–6% of patients with PBC per year (1).
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Domino, Frank J., et al., editors. "Cirrhosis, Primary Biliary." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2020. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117415/all/Cirrhosis__Primary_Biliary.
Cirrhosis, Primary Biliary. In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters Kluwer; 2020. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117415/all/Cirrhosis__Primary_Biliary. Accessed May 31, 2023.
Cirrhosis, Primary Biliary. (2020). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5-Minute Clinical Consult (27th ed.). Wolters Kluwer. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117415/all/Cirrhosis__Primary_Biliary
Cirrhosis, Primary Biliary [Internet]. In: Domino FJF, Baldor RAR, Golding JJ, Stephens MBM, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2020. [cited 2023 May 31]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117415/all/Cirrhosis__Primary_Biliary.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Cirrhosis, Primary Biliary ID - 117415 ED - Domino,Frank J, ED - Baldor,Robert A, ED - Golding,Jeremy, ED - Stephens,Mark B, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117415/all/Cirrhosis__Primary_Biliary PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -