Primary biliary cirrhosis medical therapy

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Primary Biliary Cirrhosis Microchapters


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dildar Hussain, MBBS [2]


Pharmacologic medical therapies for primary biliary cirrhosis include immunomodulators, antifibrotics, and anticholestatics. The anticholestatic ursodeoxycholic acid (UDCA) is recommended as the first line medical therapy for PBC.

Medical Therapy

Primary biliary cirrhosis

  • 1 Anticholestatics
    • 1.1 Preferred regimen : Ursodeoxycholic acid (UDCA) 13-15 mg PO q12h for 10-21 days (contraindicated for patients who are allergic to bile acids)
  • 2 Immunomodulator
    • 2.1 Glucocorticoid
      • Preferred regimen :Budesonide 6 to 9 mg per day PO (contraindicated for patients with hypersensitivity to budesonide)
      • Alternative regimen:Cyclosporine: 5-10 mg PO q24h
  • 3 Farnesoid-X-receptor (FXR) agonist
    • 3.1 Obetocholic acid
  • 4 Peroxisome proliferator-activated receptor agonist

Symptomatic Therapy

1. Pruritus

2. Supportive

  • UV light, sunlight

3. Emergency

4. Raynauds

5. Sicca syndrome

  • Dry eyes
    • Artificial tears
  • Dry mouth
    • Dental hygiene
      • Dental visit every 3–6 months
  • Dry vagina
    • Vaginal lubricants


  1. Poupon RE, Lindor KD, Cauch-Dudek K, Dickson ER, Poupon R, Heathcote EJ (1997). "Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis". Gastroenterology. 113 (3): 884–90. PMID 9287980.
  2. Poupon R (2012). "Ursodeoxycholic acid and bile-acid mimetics as therapeutic agents for cholestatic liver diseases: an overview of their mechanisms of action". Clin Res Hepatol Gastroenterol. 36 Suppl 1: S3–12. doi:10.1016/S2210-7401(12)70015-3. PMID 23141891.
  3. Kumagi T, Heathcote EJ (2008). "Primary biliary cirrhosis". Orphanet J Rare Dis. 3: 1. doi:10.1186/1750-1172-3-1. PMC 2266722. PMID 18215315.
  4. Angulo, Paul; Jorgensen, Roberta A.; Keach, Jill C.; Dickson, E. Rolland; Smith, Coleman; Lindor, Keith D. (2000). "Oral budesonide in the treatment of patients with primary biliary cirrhosis with a suboptimal response to ursodeoxycholic acid". Hepatology. 31 (2): 318–323. doi:10.1002/hep.510310209. ISSN 0270-9139.
  5. Levy, C.; Peter, J. A.; Nelson, D. R.; Keach, J.; Petz, J.; Cabrera, R.; Clark, V.; Firpi, R. J.; Morelli, G.; Soldevila-Pico, C.; Lindor, K. (2011). "Pilot study: fenofibrate for patients with primary biliary cirrhosis and an incomplete response to ursodeoxycholic acid". Alimentary Pharmacology & Therapeutics. 33 (2): 235–242. doi:10.1111/j.1365-2036.2010.04512.x. ISSN 0269-2813.
  6. Tandon, Puneeta; Rowe, Brian H.; Vandermeer, Ben; Bain, Vincent G. (2007). "The Efficacy and Safety of Bile Acid Binding Agents, Opioid Antagonists, or Rifampin in the Treatment of Cholestasis-Associated Pruritus". The American Journal of Gastroenterology. 102 (7): 1528–1536. doi:10.1111/j.1572-0241.2007.01200.x. ISSN 0002-9270.
  7. Cohen, L B; Ambinder, E P; Wolke, A M; Field, S P; Schaffner, F (1985). "Role of plasmapheresis in primary biliary cirrhosis". Gut. 26 (3): 291–294. doi:10.1136/gut.26.3.291. ISSN 0017-5749.
  8. Gluud, C; Christensen, E; Gluud, Christian (2001). "Ursodeoxycholic acid for primary biliary cirrhosis". doi:10.1002/14651858.CD000551.
  9. Gallant C, Kenny P (1986). "Oral glucocorticoids and their complications. A review". J. Am. Acad. Dermatol. 14 (2 Pt 1): 161–77. PMID 3512634.
  10. Kumagi, Teru; Heathcote, E Jenny (2008). "Primary biliary cirrhosis". Orphanet Journal of Rare Diseases. 3 (1): 1. doi:10.1186/1750-1172-3-1. ISSN 1750-1172.
  11. Leuschner M, Maier KP, Schlichting J, Strahl S, Herrmann G, Dahm HH, Ackermann H, Happ J, Leuschner U (1999). "Oral budesonide and ursodeoxycholic acid for treatment of primary biliary cirrhosis: results of a prospective double-blind trial". Gastroenterology. 117 (4): 918–25. PMID 10500075.

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