Primary biliary cirrhosis medical therapy: Difference between revisions

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{{Primary biliary cirrhosis}}
{{Primary biliary cirrhosis}}


{{CMG}}; {{AE}}
{{CMG}}; {{AE}} {{SH}}


==Overview==
==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]].
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==
*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
*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>
*Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
 
*Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
===Primary biliary cirrhosis===
===Primary biliary cirrhosis===


* 1 '''Anticholestatics'''
* '''1''' '''Anticholestatics'''
** 1.1 Preferred regimen : Ursodeoxycholic acid (UDCA) 13-15 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)'''   
** 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)'''   


* 2 '''Antiinflammtory'''
* '''2''' '''Immunomodulator'''
** 2.1 '''Glucocorticoid '''
** 2.1 '''[[Glucocorticoid]] '''
*** Preferred regimen : Budesonide 6 to 9 mg per day PO '''contraindicated for patients who have hypersensitivity to budesonide'''
*** 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]]


* 3 '''Farnesoid-X-receptor (FXR) agonist'''
===Symptomatic Therapy===
** 3.1 Obetocholic acid
'''1'''. '''Pruritus'''
*** Preferred regimen :5-10 mg PO q24h
*1st line
**[[Cholestyramine]] 4 g per day (before and after breakfast)
*2nd line
**[[Rifampin]] 150 mg bid
*3rd line
**[[Sertraline]]
*4th line
**[[Naloxone]]


**** Parenteral regimen
'''2'''. '''Supportive '''
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
*UV light, sunlight
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
 
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
'''3'''. '''Emergency'''
**** Oral regimen
*[[Plasmapheresis]]
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
'''4'''. '''Raynauds'''
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
*1st line: [[calcium channel blockers]]
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
*2nd line: [[prostacyclin]] and its derivatives, [[Endothelin receptor antagonist|endothelin receptor antagonists]], and [[phosphodiesterase inhibitors]]
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
'''5'''. '''Sicca syndrome'''
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
*Dry eyes
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
**Artificial tears
*** 2.1.2 '''Pediatric'''
*Dry mouth
**** Parenteral regimen
**Dental hygiene
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
***Dental visit every 3–6 months
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
*Dry vagina
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '<nowiki/>'''''(Contraindications/specific instructions)''''''
**Vaginal lubricants
**** Oral regimen
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Preferred regimen (2): [[drug name]] '''(for children aged ≥ 8 years)''' 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
** 2.2  '<nowiki/>'''''Other Organ system involved 2''''''
**: '''Note (1):'''
**: '''Note (2)''':
**: '''Note (3):'''
*** 2.2.1 '''Adult'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
**** Oral regimen
***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days 
***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
*** 2.2.2 '''Pediatric'''
**** Parenteral regimen
***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
**** Oral regimen
***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)


==References==
==References==
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{{WH}}
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[[Category:Gastroenterology]]
[[Category:Hepatology]]
[[Category:Disease]]
[[Category:Rheumatology]]
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[[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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