Abstract
Primary biliary cirrhosis (PBC) is one of the most common indications for adult liver transplantation in both Western countries and Japan. Recently, the number of liver transplantations for PBC has shown a decreasing trend in Europe and the United States, likely due to advances in medical therapies using ursodeoxycholic acid. However, liver transplantation remains the sole life-saving treatment method for PBC that has progressed to end-stage cirrhosis. Additionally, liver transplantation is occasionally indicated due to declines in the quality of life arising from severe cutaneous pruritus or chronic fatigue in PBC patients. The appropriate timing of the transplantation is calculated using several different prognosis prediction models. The results of liver transplantations for PBC have been excellent compared with those for other diseases, with all studies reporting 5-year survival rates higher than 70 %. Moreover, differences have not been observed between the results of transplants from living donors and those from deceased donors. PBC can recur in the graft liver after transplantation, but this phenomenon is poorly understood, including its frequency, risk factors, and long-term prognosis.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Prince M, Chetwynd A, Newman W et al (2002) Survival and symptom progression in a geographically based cohort of patients with primary biliary cirrhosis: follow-up for up to 28 years. Gastroenterology 123:1044–1051
Springer J, Cauch-Dudek K, O’Rourke K et al (1999) Asymptomatic primary biliary cirrhosis: a study of its natural history and prognosis. Am J Gastroenterol 94:47–53
Abe M, Onji M (2008) Natural history of primary biliary cirrhosis. Hepatol Res 38:639–645
Cavazza A, Caballeria L, Floreani A et al (2009) Incidence, risk factors, and survival of hepatocellular carcinoma in primary biliary cirrhosis: comparative analysis from two centers. Hepatology 50:1162–1168
Poupon RE, Poupon R, Balkau B, The UDCA-PBC Study Group (1994) Ursodiol for the long-term treatment of primary biliary cirrhosis. N Engl J Med 330:1342–1347
Corpechot C, Carrat F, Bonnand AM et al (2000) The effect of ursodeoxycholic acid therapy on liver fibrosis progression in primary biliary cirrhosis. Hepatology 32:1196–1199
Corpechot C, Carrat F, Bahr A et al (2005) The effect of ursodeoxycholic acid therapy on the natural course of primary biliary cirrhosis. Gastroenterology 128:297–303
Corpechot C, Abenavoli L, Rabahi N et al (2008) Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatology 48:871–877
Iwatsuki S, Starzl TE, Todo S et al (1988) Experience in 1,000 liver transplants under cyclosporine-steroid therapy: a survival report. Transplant Proc 20(Suppl 1):498–504
Adam R, McMaster P, O’Grady JG et al (2003) Evolution of liver transplantation in Europe: report of the European Liver Transplant Registry. Liver Transpl 9:1231–1243
Department of Health and Human Services, Health Resources and Services Administration, Healthcare Systems Bureau, Division of Transplantation (2011) OPTN/SRTR 2010 Annual Data Report. http://www.srtr.org/annual_reports/2010/. Accessed 3 Sept 2012
Lee J, Belanger A, Doucette JT et al (2007) Transplantation trends in primary biliary cirrhosis. Clin Gastroenterol Hepatol 5:1313–1315
Liermann Garcia RF, Evangelista Garcia C, McMaster P et al (2001) Transplantation for primary biliary cirrhosis: retrospective analysis of 400 patients in a single center. Hepatology 33:22–27
Kuiper EM, Hansen BE, Metselaar HJ et al (2010) Trends in liver transplantation for primary biliary cirrhosis in the Netherlands 1988–2008. BMC Gastroenterol 10:144
Prince MI, James OF (2003) The epidemiology of primary biliary cirrhosis. Clin Liver Dis 7:795–819
Hashikura Y, Makuuchi M, Kawasaki S et al (1994) Successful living-related partial liver transplantation to an adult patient. Lancet 343:1233–1234
The Japan Liver Transplant Society (2012) Liver transplantation in Japan. Registry by the Japanese Liver Transplantation Society. Jpn J Transplant (Ishoku) 46:416–428, in Japanese
Genda T, Ichida T, Sakisaka S et al (2014) Waiting-list mortality in patients with primary biliary cirrhosis in the Japanese transplant allocation system. J Gastroenterol 49:324–331
Mazzafero V, Regalia E, Doci R et al (1996) Liver transplantation for the treatment of small hepatocellular carcinoma. N Engl J Med 334:693–699
Lindor KD, Gershwin ME, Poupon R et al (2009) Primary biliary cirrhosis. AASLD practice guidelines. Hepatology 50:291–308
Poupon RE, Chretien Y, Chazouilleres O et al (2004) Quality of life in patients with primary biliary cirrhosis. Hepatology 40:489–494
Christensen E, Gunson B, Neuberger J (1999) Optimal timing of liver transplantation for patients with primary biliary cirrhosis: use of prognostic modeling. J Hepatol 30:285–292
Pugh RN, Murray-Lyon IM, Dawson JL et al (1973) Transection of the oesophagus for bleeding oesophageal varices. Br J Surg 60:646–649
Trey C, Burns DG, Saunders SJ (1966) Treatment of hepatic coma by exchange blood transfusion. New Engl J Med 274:473–481
Malinchoc M, Kamath PS, Gordon FD et al (2000) A model to predict poor survival in patients undergoing transjugular intrahepatic portosystemic shunts. Hepatology 31:864–871
Murray KF, Carithers RL Jr (2005) AASLD practice guidelines: evaluation of the patient for liver transplantation. Hepatology 41:1407–1432
Kamath PS, Wiesner R, Malinchoc M et al (2001) A model to predict survival in patients with end-stage liver disease. Hepatology 33:464–470
Forman LM, Lucey MR (2001) Predicting the prognosis of chronic liver disease: an evolution from child to MELD. Mayo end-stage liver disease. Hepatology 33:473–475
Said A, Williams J, Holden J et al (2004) Model for end-stage liver disease score predicts mortality across a broad spectrum of liver disease. J Hepatol 40:897–903
Wiesner R, Edwards E, Freeman R et al (2003) Model for end-stage liver disease (MELD) and allocation of donor livers. Gastroenterology 124:91–96
Dickson ER, Grambsch PM, Fleming TR et al (1989) Prognosis in primary biliary cirrhosis: model for decision making. Hepatology 10:1–7
Grambsch PM, Dickson ER, Kaplan M et al (1989) Extramural cross-validation of the Mayo primary biliary cirrhosis survival model establishes its generalizability. Hepatology 10:846–850
Roll J, Boyer JL, Barry D et al (1983) The prognostic importance of clinical and histologic features in asymptomatic and symptomatic primary biliary cirrhosis. N Engl J Med 308:1–7
Christensen E, Newberger J, Crowe J et al (1985) Beneficial effect of azathioprine and prediction of prognosis in primary biliary cirrhosis. Final results of an international trial. Gastroenterology 89:1084–1091
Kim WR, Wiesner RH, Therneau TM et al (1998) Optimal timing of liver transplantation for primary biliary cirrhosis. Hepatology 28:33–38
Gilroy RK, Lynch SV, Strong RW et al (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 6:749–752
Murtaugh PA, Dickson ER, Van Dam GM et al (1994) Primary biliary cirrhosis: prediction of short-term survival based on repeated patient visits. Hepatology 20:126–134
Obata Y, Hashimoto E (1991) Selection of the patients with PBC for liver transplantation and timing of surgery. In: Ichida F, Tanikawa K (eds) Indications for liver transplantation. The Japanese Liver Transplantation Society Proceedings. Arcmedia, Tokyo, pp 13–25 (in Japanese)
Neuberger J, Altman DG, Christensen E et al (1986) Use of a prognostic index in evaluation of liver transplantation for primary biliary cirrhosis. Transplantation 41:713–716
Markus BH, Dickson ER, Grambsch PM et al (1989) Efficiency of liver transplantation in patients with primary biliary cirrhosis. N Engl J Med 320:1709–1713
Organ Procurement and Transplantation Network (2012) Liver Kaplan-Meier patient survival rates for transplant performed: 1997–2004. http://optn.transplant.hrsa.gov/latestData/rptStrat.asp. Accessed 14 Oct 2012
Roberts MS, Angus DC, Bryce CL et al (2004) Survival after liver transplantation in the United States: a disease-specific analysis of the UNOS database. Liver Transpl 10:886–897
Mendes FD, Kim WR, Pedersen R et al (2008) Mortality attributable to cholestatic liver disease in the United States. Hepatology 47:1241–1247
Hasegawa K, Sugawara Y, Imamura H et al (2005) Living donor liver transplantation for primary biliary cirrhosis: retrospective analysis of 50 patients in a single center. Transplant Int 18:794–799
Gross CR, Malinchoc M, Kim WR et al (1999) Quality of life before and after liver transplantation for cholestatic liver disease. Hepatology 29:356–364
Charatcharoenwitthaya P, Pimentel S, Talwalker JA et al (2007) Long-term survival and impact of ursodeoxycholic acid treatment for recurrent primary biliary cirrhosis after liver transplantation. Liver Transplant 13:1236–1245
Milkiewicz P, Gunson B, Saksena S et al (2000) Increased incidence of chronic rejection in adult patients transplanted for autoimmune hepatitis: assessment of risk factors. Transplantation 70:477–480
Jacob DA, Neumann UP, Bahra M et al (2006) Long-term follow-up after recurrence of primary biliary cirrhosis after liver transplantation in 100 patients. Clin Transplant 20:211–220
Neuberger J, Postmann B, Macdougall BR et al (1982) Recurrence of primary biliary cirrhosis after liver transplantation. N Engl J Med 306:1–4
Dubel L, Farges O, Bismuth H et al (1995) Kinetics of anti-M2 antibodies after liver transplantation for primary biliary cirrhosis. J Hepatol 23:674–680
Sanchez EQ, Levy MF, Goldstein RM et al (2003) The changing clinical presentation of recurrent primary biliary cirrhosis after liver transplantation. Transplantation 76:1583–1588
Sylvestre PB, Batts KP, Burgart LJ et al (2003) Recurrence of primary biliary cirrhosis after liver transplantation: histologic estimate of incidence and natural history. Liver Transplant 9:1086–1093
Neuberger J, Gunson B, Hubscher S et al (2004) Immunosuppression affects the rate of recurrent primary biliary cirrhosis after liver transplantation. Liver Transplant 10:488–491
Morioka D, Egawa H, Kasahara M et al (2007) Impact of human leukocyte antigen mismatching on outcomes of living donor liver transplantation for primary biliary cirrhosis. Liver Transplant 13:80–90
Yamagiwa S, Ichida T (2007) Recurrence of primary biliary cirrhosis and primary sclerosing cholangitis after liver transplantation in Japan. Hepatol Res 37(Suppl):S449–S454
Montano-Loza AJ, Wasilenko S, Bintner J et al (2010) Cyclosporine A protects against primary biliary cirrhosis recurrence after liver transplantation. Am J Transplant 10:852–858
Monousou P, Arvaniti V, Tsochatzis E et al (2010) Primary biliary cirrhosis after liver transplantation: influence of immunosuppression and human leukocyte antigen locus disparity. Liver Transplant 16:64–73
Kaneko J, Sugawara Y, Tamura S et al (2012) Long-term outcome of living donor liver transplantation for primary biliary cirrhosis. Transplant Int 25:7–12
Gautam M, Cheruvattath R, Balan V (2006) Recurrence of autoimmune liver disease after liver transplantation: a systematic review. Liver Transplant 12:1813–1824
Guy JE, Qian P, Lowell JA et al (2005) Recurrent primary biliary cirrhosis: peritransplant factors and ursodeoxycholic acid treatment post-liver transplant. Liver Transplant 11:1252–1257
Rowe IA, Webb K, Gunson BK et al (2008) The impact of disease recurrence on graft survival following liver transplantation: a single center experience. Transplant Int 21:459–465
Conflicts of Interest
The authors of this paper report no conflicts of interest.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer Japan
About this chapter
Cite this chapter
Genda, T., Ichida, T. (2014). Liver Transplantation for Primary Biliary Cirrhosis. In: Ohira, H. (eds) Autoimmune Liver Diseases. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54789-1_21
Download citation
DOI: https://doi.org/10.1007/978-4-431-54789-1_21
Published:
Publisher Name: Springer, Tokyo
Print ISBN: 978-4-431-54788-4
Online ISBN: 978-4-431-54789-1
eBook Packages: MedicineMedicine (R0)