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==Medical Therapy==
==Medical Therapy==
*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>
*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>{{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=}}


===Primary biliary cirrhosis===
===Primary biliary cirrhosis===

Revision as of 21:54, 5 February 2018

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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

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

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 who have 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

  • 1st line:Calcium channel blockers
  • 2nd line:Alternative: 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

  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.

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