Primary biliary cirrhosis medical therapy

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

Overview

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

Pharmacologic medical therapy is recommended among patients with Primary biliary cirrhosis.^[1]^[2]^[3]^[4]^[5]^[6]^[7]^[8]^[9]^[10]^[11]

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
- Fibrates

Symptomatic Therapy

1. Pruritus

1st line
- Cholestyramine 4 g per day (before and after breakfast)
2nd line
- Rifampin 150 mg bid
3rd line
- Sertraline
4th line
- Naloxone

2. Supportive

UV light, sunlight

3. Emergency

Plasmapheresis

4. Raynauds

1st line: calcium channel blockers
2nd line: prostacyclin and its derivatives, endothelin receptor antagonists, and phosphodiesterase inhibitors

5. Sicca syndrome

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

References

↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ Gluud, C; Christensen, E; Gluud, Christian (2001). “Ursodeoxycholic acid for primary biliary cirrhosis”. doi:10.1002/14651858.CD000551.
↑ Gallant C, Kenny P (1986). “Oral glucocorticoids and their complications. A review”. J. Am. Acad. Dermatol. 14 (2 Pt 1): 161–77. PMID 3512634.
↑ 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.
↑ 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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[pmid9287980-1] 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.

[pmid23141891-2] 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.

[pmid18215315-3] 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.

[AnguloJorgensen2000-4] 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.

[LevyPeter2011-5] 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.

[TandonRowe2007-6] 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.

[CohenAmbinder1985-7] 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.

[GluudChristensen2001-8] Gluud, C; Christensen, E; Gluud, Christian (2001). “Ursodeoxycholic acid for primary biliary cirrhosis”. doi:10.1002/14651858.CD000551.

[pmid3512634-9] Gallant C, Kenny P (1986). “Oral glucocorticoids and their complications. A review”. J. Am. Acad. Dermatol. 14 (2 Pt 1): 161–77. PMID 3512634.

[KumagiHeathcote2008-10] 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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