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


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


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==Overview==
The mainstay of treatment for primary biliary cirrhosis is medical therapy. Surgery is usually reserved for patients with either decompensated [[cirrhosis]] and endstage [[liver failure]] who do not show any improvement with medical therapy.


==Overview==
==Indications==
In advanced cases, a [[Liver transplantation|liver transplant]], if successful, results in a favorable prognosis. After liver transplant, the recurrence rate may be as high as 18% at 5 years, and up to 30% at 10 years. There is no consensus on risk factors for recurrence of the disease. <ref>Medical care of the Liver Trasplant Patient, 3rd Edition published 2006, editied by Paul G. Killenberg, page 429</ref>
Indications for liver transplantation include:<ref name="pmid21459072">{{cite journal |vauthors=Carbone M, Neuberger J |title=Liver transplantation in PBC and PSC: indications and disease recurrence |journal=Clin Res Hepatol Gastroenterol |volume=35 |issue=6-7 |pages=446–54 |year=2011 |pmid=21459072 |doi=10.1016/j.clinre.2011.02.007 |url=}}</ref><ref name="Mucha2014">{{cite journal|last1=Mucha|first1=Krzysztof|title=Primary Biliary Cirrhosis in the Era of Liver Transplantation|journal=Annals of Transplantation|volume=19|year=2014|pages=488–493|issn=2329-0358|doi=10.12659/AOT.890753}}</ref><ref name="pmid23721009">{{cite journal |vauthors=Taniguchi M |title=Liver transplantation in the MELD era--analysis of the OPTN/UNOS registry |journal=Clin Transpl |volume= |issue= |pages=41–65 |year=2012 |pmid=23721009 |doi= |url=}}</ref><ref name="MazzaferroBhoori2011">{{cite journal|last1=Mazzaferro|first1=Vincenzo|last2=Bhoori|first2=Sherrie|last3=Sposito|first3=Carlo|last4=Bongini|first4=Marco|last5=Langer|first5=Martin|last6=Miceli|first6=Rosalba|last7=Mariani|first7=Luigi|title=Milan criteria in liver transplantation for hepatocellular carcinoma: An evidence-based analysis of 15 years of experience|journal=Liver Transplantation|volume=17|issue=S2|year=2011|pages=S44–S57|issn=15276465|doi=10.1002/lt.22365}}</ref><ref name="Purohit2015">{{cite journal|last1=Purohit|first1=Treta|title=Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy|journal=World Journal of Hepatology|volume=7|issue=7|year=2015|pages=926|issn=1948-5182|doi=10.4254/wjh.v7.i7.926}}</ref>
*Decompensated [[cirrhosis]]
*Endstage [[liver failure]]
*Indications specific to endstage [[liver failure]] include:
**Refractory [[ascites]]
**Recurrent [[spontaneous bacterial peritonitis]]
**Recurrent [[Variceal bleed|variceal hemorrhage]]
**[[Hepatic encephalopathy]]
**[[Hepatorenal syndrome|Hepatorenal syndrome type I]]
**[[Hepatocellular carcinoma]]
*Indications specific to primary biliary cirrhosis include:
**Refractory [[pruritus]]
**Chronic [[fatigue]]
 
== Surgery ==
*The mainstay of treatment for [[Primary biliary cirrhosis|primarybiliary cirrhosis]] is medical therapy. [[Liver transplantation]] is usually reserved for patients with either:<ref name="Mucha2014" /><ref name="Purohit20152">{{cite journal|last1=Purohit|first1=Treta|title=Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy|journal=World Journal of Hepatology|volume=7|issue=7|year=2015|pages=926|issn=1948-5182|doi=10.4254/wjh.v7.i7.926}}</ref><ref name="pmid14594139">{{cite journal |vauthors=MacQuillan GC, Neuberger J |title=Liver transplantation for primary biliary cirrhosis |journal=Clin Liver Dis |volume=7 |issue=4 |pages=941–56, ix |year=2003 |pmid=14594139 |doi= |url=}}</ref><ref name="pmid11821734">{{cite journal |vauthors=Tinmouth J, Tomlinson G, Heathcote EJ, Lilly L |title=Benefit of transplantation in primary biliary cirrhosis between 1985-1997 |journal=Transplantation |volume=73 |issue=2 |pages=224–7 |year=2002 |pmid=11821734 |doi= |url=}}</ref><ref name="pmid25262831">{{cite journal |vauthors=Raczyńska J, Habior A, Pączek L, Foroncewicz B, Pawełas A, Mucha K |title=Primary biliary cirrhosis in the era of liver transplantation |journal=Ann. Transplant. |volume=19 |issue= |pages=488–93 |year=2014 |pmid=25262831 |doi=10.12659/AOT.890753 |url=}}</ref>
**Decompensated [[cirrhosis]]
 
**Endstage [[liver failure]]
*[[Liver transplantation]] is considered to be the only definitive treatment for [[Primary biliary cirrhosis|PBC]] with decompensated [[cirrhosis]] and endstage [[liver failure]].
*The 5 year survival rate following [[liver transplantation]] is up to 85%, which is the highest in any liver disease.
*'''"Model for end-stage liver disease” (MELD)''' score is used to govern priority for [[liver transplantation]].<ref name="pmid12512033">{{cite journal| author=Wiesner R, Edwards E, Freeman R, Harper A, Kim R, Kamath P et al.| title=Model for end-stage liver disease (MELD) and allocation of donor livers. | journal=Gastroenterology | year= 2003 | volume= 124 | issue= 1 | pages= 91-6 | pmid=12512033 | doi=10.1053/gast.2003.50016 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12512033  }} </ref>
*The score range between 6 and 40 and is calculated using a logarithmic assessment of three objective and [[reproducible]] variables including:
**[[Total serum bilirubin]]
**[[Creatinine]] concentrations
**[[International normalized ratio]]
* As per European Association for the Study of the Liver (EASL) guidelines the MELD score of over 12 should be evaluated for potential liver transplantation in [[Primary biliary cirrhosis|PBC]].


==Surgery==
== Recurrence ==
*The recurrence rates of primary biliary cirrhosis following [[Liver transplantation|liver transplantation]] are:<ref name="pmid21459072" /><ref name="pmid11084062">{{cite journal |vauthors=Gilroy RK, Lynch SV, Strong RW, Kerlin P, Balderson GA, Stuart KA, Crawford DH |title=Confirmation of the role of the Mayo Risk Score as a predictor of resource utilization after orthotopic liver transplantation for primary biliary cirrhosis |journal=Liver Transpl. |volume=6 |issue=6 |pages=749–52 |year=2000 |pmid=11084062 |doi=10.1053/jlts.2000.9746 |url=}}</ref><ref name="pmid8750166">{{cite journal |vauthors=Dubel L, Farges O, Bismuth H, Sebagh M, Homberg JC, Johanet C |title=Kinetics of anti-M2 antibodies after liver transplantation for primary biliary cirrhosis |journal=J. Hepatol. |volume=23 |issue=6 |pages=674–80 |year=1995 |pmid=8750166 |doi= |url=}}</ref><ref name="pmid20526013">{{cite journal |vauthors=Ciesek S, Becker T, Manns MP, Strassburg CP |title=Anti-parietal cell autoantibodies (PCA) in primary biliary cirrhosis: a putative marker for recurrence after orthotopic liver transplantation? |journal=Ann Hepatol |volume=9 |issue=2 |pages=181–5 |year=2010 |pmid=20526013 |doi= |url=}}</ref><ref name="pmid16926769">{{cite journal |vauthors=Schreibman I, Regev A |title=Recurrent primary biliary cirrhosis after liver transplantation--the disease and its management |journal=MedGenMed |volume=8 |issue=2 |pages=30 |year=2006 |pmid=16926769 |pmc=1785210 |doi= |url=}}</ref>
**About 20% after 5 years
**About 30% at 10 years
**About 40% at 15 years


==References==
==References==
{{reflist|2}}
{{Reflist|2}}
 
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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

The mainstay of treatment for primary biliary cirrhosis is medical therapy. Surgery is usually reserved for patients with either decompensated cirrhosis and endstage liver failure who do not show any improvement with medical therapy.

Indications

Indications for liver transplantation include:[1][2][3][4][5]

Surgery

Recurrence

References

  1. 1.0 1.1 Carbone M, Neuberger J (2011). "Liver transplantation in PBC and PSC: indications and disease recurrence". Clin Res Hepatol Gastroenterol. 35 (6–7): 446–54. doi:10.1016/j.clinre.2011.02.007. PMID 21459072.
  2. 2.0 2.1 Mucha, Krzysztof (2014). "Primary Biliary Cirrhosis in the Era of Liver Transplantation". Annals of Transplantation. 19: 488–493. doi:10.12659/AOT.890753. ISSN 2329-0358.
  3. Taniguchi M (2012). "Liver transplantation in the MELD era--analysis of the OPTN/UNOS registry". Clin Transpl: 41–65. PMID 23721009.
  4. Mazzaferro, Vincenzo; Bhoori, Sherrie; Sposito, Carlo; Bongini, Marco; Langer, Martin; Miceli, Rosalba; Mariani, Luigi (2011). "Milan criteria in liver transplantation for hepatocellular carcinoma: An evidence-based analysis of 15 years of experience". Liver Transplantation. 17 (S2): S44–S57. doi:10.1002/lt.22365. ISSN 1527-6465.
  5. Purohit, Treta (2015). "Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy". World Journal of Hepatology. 7 (7): 926. doi:10.4254/wjh.v7.i7.926. ISSN 1948-5182.
  6. Purohit, Treta (2015). "Primary biliary cirrhosis: Pathophysiology, clinical presentation and therapy". World Journal of Hepatology. 7 (7): 926. doi:10.4254/wjh.v7.i7.926. ISSN 1948-5182.
  7. MacQuillan GC, Neuberger J (2003). "Liver transplantation for primary biliary cirrhosis". Clin Liver Dis. 7 (4): 941–56, ix. PMID 14594139.
  8. Tinmouth J, Tomlinson G, Heathcote EJ, Lilly L (2002). "Benefit of transplantation in primary biliary cirrhosis between 1985-1997". Transplantation. 73 (2): 224–7. PMID 11821734.
  9. Raczyńska J, Habior A, Pączek L, Foroncewicz B, Pawełas A, Mucha K (2014). "Primary biliary cirrhosis in the era of liver transplantation". Ann. Transplant. 19: 488–93. doi:10.12659/AOT.890753. PMID 25262831.
  10. Wiesner R, Edwards E, Freeman R, Harper A, Kim R, Kamath P; et al. (2003). "Model for end-stage liver disease (MELD) and allocation of donor livers". Gastroenterology. 124 (1): 91–6. doi:10.1053/gast.2003.50016. PMID 12512033.
  11. Gilroy RK, Lynch SV, Strong RW, Kerlin P, Balderson GA, Stuart KA, Crawford DH (2000). "Confirmation of the role of the Mayo Risk Score as a predictor of resource utilization after orthotopic liver transplantation for primary biliary cirrhosis". Liver Transpl. 6 (6): 749–52. doi:10.1053/jlts.2000.9746. PMID 11084062.
  12. Dubel L, Farges O, Bismuth H, Sebagh M, Homberg JC, Johanet C (1995). "Kinetics of anti-M2 antibodies after liver transplantation for primary biliary cirrhosis". J. Hepatol. 23 (6): 674–80. PMID 8750166.
  13. Ciesek S, Becker T, Manns MP, Strassburg CP (2010). "Anti-parietal cell autoantibodies (PCA) in primary biliary cirrhosis: a putative marker for recurrence after orthotopic liver transplantation?". Ann Hepatol. 9 (2): 181–5. PMID 20526013.
  14. Schreibman I, Regev A (2006). "Recurrent primary biliary cirrhosis after liver transplantation--the disease and its management". MedGenMed. 8 (2): 30. PMC 1785210. PMID 16926769.

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