Primary biliary cirrhosis medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Medical Therapy

There is no known cure, but medication may slow the progression so that a normal lifespan and quality of life may be attainable for many patients. Specific treatment for fatigue, which may be debilitating in some patients, is limited and currently undergoing trials.

  • Ursodeoxycholic acid (Ursodiol) is the most frequently used treatment. This helps reduce the cholestasis and improves blood test results (liver function tests). It has a minimal effect on symptoms and whether it improves prognosis is controversial.
  • To relieve itching caused by bile acids in circulation, which would normally be removed by the liver, cholestyramine (a bile acid sequestrant) may be prescribed to absorb bile acids in the gut and be eliminated, rather than re-enter the blood stream. Alternative agents include naltrexone and rifampicin.
  • To relieve fatigue associated with primary biliary cirrhosis, current studies indicate that Provigil (modafinil) may be effective without damaging the liver.[1] Though off-patent, the limiting factor in the use of modafinil in the U.S. is cost. The manufacturer, Cephalon, has made agreements with manufacturers of generic modafinil to provide payments in exchange for delaying their sale of modafinil.[2] The FTC has filed suit against Cephalon alleging anti-competitive behavior.[3]
  • Patients with PBC have poor lipid-dependent absorption of Vitamins A, D, E, K.[4] Appropriate supplementation is recommended when bilirubin is elevated[5]. Multivitamins (esp. Vitamin D) and calcium are also recommended.
  • Patients with PBC are at elevated risk of developing osteoporosis[6] and esophageal varices[7] as compared to the general population and others with liver disease. Screening and treatment of these complications is an important part of the management of PBC.

As in all liver diseases, excessive consumption of alcohol is contraindicated.

References

  1. Modafinil#Primary_biliary_cirrhosis
    Ian Gan S, de Jongh M, Kaplan MM (2009). "Modafinil in the treatment of debilitating fatigue in primary biliary cirrhosis: a clinical experience". Dig. Dis. Sci. 54 (10): 2242–6. doi:10.1007/s10620-008-0613-3. PMID 19082890. Unknown parameter |month= ignored (help)
    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. Ref 157 viz:
    Jones DE, Newton JL (2007). "An open study of modafinil for the treatment of daytime somnolence and fatigue in primary biliary cirrhosis". Aliment. Pharmacol. Ther. 25 (4): 471–6. doi:10.1111/j.1365-2036.2006.03223.x. PMID 17270003. Unknown parameter |month= ignored (help)
  2. Modafinil#Patent_protection_and_antitrust_litigation
    Carrier MA (2011). "Provigil: A Case Study of Anticompetitive Behavior" (PDF). Hastings Science & Technology Law Journal. 3 (2): 441–452.
  3. http://www.ftc.gov/os/caselist/0610182/080213complaint.pdf
  4. Bruce R. Bacon; John G. O'Grady (2006). Comprehensive clinical hepatology. Elsevier Health Sciences. pp. 283–. ISBN 978-0-323-03675-7. Retrieved 29 June 2010.
  5. Lindor, KD (2009 Jul). "Primary biliary cirrhosis". Hepatology (Baltimore, Md.). 50 (1): 291–308. doi:10.1002/hep.22906. PMID 19554543. Unknown parameter |coauthors= ignored (help); Check date values in: |date= (help)
  6. Collier, Jane (2002). "Guidelines on the management of osteoporosis associated with chronic liver disease". Gut. 50: i1–i9. PMC 1867644. PMID 11788576. |access-date= requires |url= (help)
  7. Ali, AH (2011 Aug). "Varices in early histological stage primary biliary cirrhosis". Journal of Clinical Gastroenterology. 45 (7): e66–71. doi:10.1097/MCG.0b013e3181f18c4e. PMID 20856137. Unknown parameter |coauthors= ignored (help); Check date values in: |date= (help)

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