
Health Dictionary
Reliable, easy-to-read patient information on common medical conditions β written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Primary biliary cirrhosis medical therapy
Introduction
Overview
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
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 regimenΒ : 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 regimenΒ :Budesonide 6 to 9 mg per day PO (contraindicated for patients with hypersensitivity to budesonide)
- Alternative regimen:Cyclosporine: 5-10 mg PO q24h
- 2.1 Glucocorticoid
- 3 Farnesoid-X-receptor (FXR) agonist
- 3.1 Obetocholic acid
- 4 Peroxisome proliferator-activated receptor agonist
Symptomatic Therapy
1. Pruritus
- 1st line
- Cholestyramine 4 g per day (before and after breakfast)
- 2nd line
- Rifampin 150 mg bid
- 3rd line
- 4th line
2. Supportive
- UV light, sunlight
3. Emergency
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 3β6 months
- Dental hygiene
- Dry vagina
- Vaginal lubricants
References
References
- β 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): 884β90. PMIDΒ 9287980.
- β 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: S3β12. doi:10.1016/S2210-7401(12)70015-3. PMIDΒ 23141891.
- β Kumagi T, Heathcote EJ (2008). “Primary biliary cirrhosis”. Orphanet J Rare Dis. 3: 1. doi:10.1186/1750-1172-3-1. PMCΒ 2266722. PMIDΒ 18215315.
- β 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): 318β323. doi:10.1002/hep.510310209. ISSNΒ 0270-9139.
- β 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): 235β242. doi:10.1111/j.1365-2036.2010.04512.x. ISSNΒ 0269-2813.
- β 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): 1528β1536. doi:10.1111/j.1572-0241.2007.01200.x. ISSNΒ 0002-9270.
- β 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): 291β294. doi:10.1136/gut.26.3.291. ISSNΒ 0017-5749.
- β Gluud, C; Christensen, E; Gluud, Christian (2001). “Ursodeoxycholic acid for primary biliary cirrhosis”. doi:10.1002/14651858.CD000551.
- β Gallant C, Kenny P (1986). “Oral glucocorticoids and their complications. A review”. J. Am. Acad. Dermatol. 14 (2 Pt 1): 161β77. PMIDΒ 3512634.
- β Kumagi, Teru; Heathcote, E Jenny (2008). “Primary biliary cirrhosis”. Orphanet Journal of Rare Diseases. 3 (1): 1. doi:10.1186/1750-1172-3-1. ISSNΒ 1750-1172.
- β 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): 918β25. PMIDΒ 10500075.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Dildar Hussain, MBBS [2]
