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__NOTOC__
__NOTOC__
{{CMG}}
{{Primary biliary cirrhosis}}
{{Primary biliary cirrhosis}}
{{CMG}}; {{AE}} {{SH}}
==Overview==
Pharmacologic medical therapies for primary biliary cirrhosis include [[Immunomodulator|immunomodulators]], antifibrotics, and anticholestatics. The anticholestatic [[ursodeoxycholic acid]] (UDCA) is recommended as the first line medical therapy for [[Primary biliary cirrhosis|PBC]].
==Medical Therapy==
==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.
*Pharmacologic medical therapy is recommended among patients with Primary biliary cirrhosis.<ref name="pmid9287980">{{cite journal |vauthors=Poupon RE, Lindor KD, Cauch-Dudek K, Dickson ER, Poupon R, Heathcote EJ |title=Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis |journal=Gastroenterology |volume=113 |issue=3 |pages=884–90 |year=1997 |pmid=9287980 |doi= |url=}}</ref><ref name="pmid23141891">{{cite journal |vauthors=Poupon R |title=Ursodeoxycholic acid and bile-acid mimetics as therapeutic agents for cholestatic liver diseases: an overview of their mechanisms of action |journal=Clin Res Hepatol Gastroenterol |volume=36 Suppl 1 |issue= |pages=S3–12 |year=2012 |pmid=23141891 |doi=10.1016/S2210-7401(12)70015-3 |url=}}</ref><ref name="pmid18215315">{{cite journal |vauthors=Kumagi T, Heathcote EJ |title=Primary biliary cirrhosis |journal=Orphanet J Rare Dis |volume=3 |issue= |pages=1 |year=2008 |pmid=18215315 |pmc=2266722 |doi=10.1186/1750-1172-3-1 |url=}}</ref><ref name="AnguloJorgensen2000">{{cite journal|last1=Angulo|first1=Paul|last2=Jorgensen|first2=Roberta A.|last3=Keach|first3=Jill C.|last4=Dickson|first4=E. Rolland|last5=Smith|first5=Coleman|last6=Lindor|first6=Keith D.|title=Oral budesonide in the treatment of patients with primary biliary cirrhosis with a suboptimal response to ursodeoxycholic acid|journal=Hepatology|volume=31|issue=2|year=2000|pages=318–323|issn=0270-9139|doi=10.1002/hep.510310209}}</ref><ref name="LevyPeter2011">{{cite journal|last1=Levy|first1=C.|last2=Peter|first2=J. A.|last3=Nelson|first3=D. R.|last4=Keach|first4=J.|last5=Petz|first5=J.|last6=Cabrera|first6=R.|last7=Clark|first7=V.|last8=Firpi|first8=R. J.|last9=Morelli|first9=G.|last10=Soldevila-Pico|first10=C.|last11=Lindor|first11=K.|title=Pilot study: fenofibrate for patients with primary biliary cirrhosis and an incomplete response to ursodeoxycholic acid|journal=Alimentary Pharmacology & Therapeutics|volume=33|issue=2|year=2011|pages=235–242|issn=02692813|doi=10.1111/j.1365-2036.2010.04512.x}}</ref><ref name="TandonRowe2007">{{cite journal|last1=Tandon|first1=Puneeta|last2=Rowe|first2=Brian H.|last3=Vandermeer|first3=Ben|last4=Bain|first4=Vincent G.|title=The Efficacy and Safety of Bile Acid Binding Agents, Opioid Antagonists, or Rifampin in the Treatment of Cholestasis-Associated Pruritus|journal=The American Journal of Gastroenterology|volume=102|issue=7|year=2007|pages=1528–1536|issn=0002-9270|doi=10.1111/j.1572-0241.2007.01200.x}}</ref><ref name="CohenAmbinder1985">{{cite journal|last1=Cohen|first1=L B|last2=Ambinder|first2=E P|last3=Wolke|first3=A M|last4=Field|first4=S P|last5=Schaffner|first5=F|title=Role of plasmapheresis in primary biliary cirrhosis.|journal=Gut|volume=26|issue=3|year=1985|pages=291–294|issn=0017-5749|doi=10.1136/gut.26.3.291}}</ref><ref name="GluudChristensen2001">{{cite journal|last1=Gluud|first1=C|last2=Christensen|first2=E|last3=Gluud|first3=Christian|title=Ursodeoxycholic acid for primary biliary cirrhosis|year=2001|doi=10.1002/14651858.CD000551}}</ref><ref name="pmid3512634">{{cite journal |vauthors=Gallant C, Kenny P |title=Oral glucocorticoids and their complications. A review |journal=J. Am. Acad. Dermatol. |volume=14 |issue=2 Pt 1 |pages=161–77 |year=1986 |pmid=3512634 |doi= |url=}}</ref><ref name="KumagiHeathcote2008">{{cite journal|last1=Kumagi|first1=Teru|last2=Heathcote|first2=E Jenny|title=Primary biliary cirrhosis|journal=Orphanet Journal of Rare Diseases|volume=3|issue=1|year=2008|pages=1|issn=1750-1172|doi=10.1186/1750-1172-3-1}}</ref><ref>{{cite journal |vauthors=Leuschner M, Maier KP, Schlichting J, Strahl S, Herrmann G, Dahm HH, Ackermann H, Happ J, Leuschner U |title=Oral budesonide and ursodeoxycholic acid for treatment of primary biliary cirrhosis: results of a prospective double-blind trial |journal=Gastroenterology |volume=117 |issue=4 |pages=918–25 |year=1999 |pmid=10500075 |doi= |url=}}</ref>


* 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.
===Primary biliary cirrhosis===


* 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]].
* '''1''' '''Anticholestatics'''
** 1.1 Preferred regimen : [[Ursodeoxycholic acid|Ursodeoxycholic acid (UDCA)]] 13-15 mg PO q12h for 10-21 days '''(contraindicated for patients who are allergic to bile acids)''' 


* To relieve fatigue associated with primary biliary cirrhosis, current studies indicate that Provigil (modafinil) may be effective without damaging the liver.<ref>[[Modafinil#Primary_biliary_cirrhosis]]<br/>{{cite journal |author=Ian Gan S, de Jongh M, Kaplan MM |title=Modafinil in the treatment of debilitating fatigue in primary biliary cirrhosis: a clinical experience |journal=Dig. Dis. Sci. |volume=54 |issue=10 |pages=2242–6 |year=2009 |month=October |pmid=19082890 |doi=10.1007/s10620-008-0613-3 |url=http://www.springerlink.com/content/f0207x6110847113/}}<br/>{{cite journal |author=Kumagi T, Heathcote EJ |title=Primary biliary cirrhosis |journal=Orphanet J Rare Dis |volume=3 |pages=1 |year=2008 |pmid=18215315 |pmc=2266722 |doi=10.1186/1750-1172-3-1 |url=http://www.ojrd.com/content/3//1 |quote=[http://www.ojrd.com/content/3/1/1#B157 Ref 157 viz:]}}<br/>{{cite journal |author=Jones DE, Newton JL |title=An open study of modafinil for the treatment of daytime somnolence and fatigue in primary biliary cirrhosis |journal=Aliment. Pharmacol. Ther. |volume=25 |issue=4 |pages=471–6 |year=2007 |month=February |pmid=17270003 |doi=10.1111/j.1365-2036.2006.03223.x |url=http://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0269-2813&date=2007&volume=25&issue=4&spage=471}}</ref>  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.<ref>[[Modafinil#Patent_protection_and_antitrust_litigation]]<br/>{{cite journal |author=Carrier MA |title=Provigil: A Case Study of Anticompetitive Behavior |journal=Hastings Science & Technology Law Journal |volume=3 |issue=2 |pages=441–452 |year=2011 |url=http://hstlj.org/content/vol3/iss2/v3i2carrier.pdf |format=PDF}}</ref>  The FTC has filed suit against Cephalon alleging anti-competitive behavior.<ref>http://www.ftc.gov/os/caselist/0610182/080213complaint.pdf</ref>
* '''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]]


* Patients with PBC have poor lipid-dependent absorption of Vitamins A, D, E, K.<ref name="BaconO'Grady2006">{{cite book|author1=Bruce R. Bacon|author2=John G. O'Grady|title=Comprehensive clinical hepatology|url=http://books.google.com/?id=ec0G9HGiR8MC&pg=PA283|accessdate=29 June 2010|year=2006|publisher=Elsevier Health Sciences|isbn=978-0-323-03675-7|pages=283–}}</ref> Appropriate supplementation is recommended when bilirubin is elevated<ref>{{cite journal|last=Lindor|first=KD|coauthors=Gershwin, ME; Poupon, R; Kaplan, M; Bergasa, NV; Heathcote, EJ; American Association for Study of Liver, Diseases|title=Primary biliary cirrhosis.|journal=Hepatology (Baltimore, Md.)|date=2009 Jul|volume=50|issue=1|pages=291-308|pmid=19554543|doi=10.1002/hep.22906}}</ref>. Multivitamins (esp. Vitamin D) and calcium are also recommended.
===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]]


* Patients with PBC are at elevated risk of developing [[osteoporosis]]<ref>{{cite journal|last=Collier|first=Jane|title=Guidelines on the management of osteoporosis associated with chronic liver disease|journal=Gut|year=2002|volume=50|pages=i1-i9|pmid=11788576|accessdate=14 June 2012|pmc=1867644}}</ref> and [[esophageal varices]]<ref>{{cite journal|last=Ali|first=AH|coauthors=Sinakos, E; Silveira, MG; Jorgensen, RA; Angulo, P; Lindor, KD|title=Varices in early histological stage primary biliary cirrhosis.|journal=Journal of Clinical Gastroenterology|date=2011 Aug|volume=45|issue=7|pages=e66-71|pmid=20856137|doi=10.1097/MCG.0b013e3181f18c4e}}</ref>  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.
'''2'''. '''Supportive '''
*UV light, sunlight


*As in all liver diseases, [[alcoholic beverage]]s are contraindicated.
'''3'''. '''Emergency'''
*[[Plasmapheresis]]
'''4'''. '''Raynauds'''
*1st line: [[calcium channel blockers]]
*2nd line: [[prostacyclin]] and its derivatives, [[Endothelin receptor antagonist|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


As in all liver diseases, excessive consumption of [[alcohol]] is contraindicated.
==References==
==References==
{{reflist|2}}
{{Reflist|2}}


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[[Category:Gastroenterology]]
[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Hepatology]]
[[Category:Autoimmune diseases]]
[[Category:Disease]]
[[Category:Disease]]
 
[[Category:Rheumatology]]
[[Category:Needs overview]]
[[Category:Medicine]]
[[Category:Up-To-Date]]

Latest revision as of 23:49, 29 July 2020

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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

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

References

  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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