Semin Liver Dis 2014; 34(03): 329-333
DOI: 10.1055/s-0034-1383731
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

The Natural History of Primary Biliary Cirrhosis

Mohamad H. Imam
1   Department of Internal Medicine, University of North Dakota, Fargo, North Dakota
,
Keith D. Lindor
2   College of Health Solutions, Arizona State University, Phoenix, Arizona
› Author Affiliations
Further Information

Publication History

Publication Date:
24 July 2014 (online)

Abstract

Our understanding of the natural history of primary biliary cirrhosis (PBC) has been evolving especially following the introduction of ursodeoxycholic acid (UDCA). A clearer understanding of disease pathophysiology and earlier diagnosis with increased prevalence of the disease worldwide has led to increased interest and improved outcomes in patients with PBC. In this article, the authors touch briefly on features of the disease and describe the natural history of PBC prior to and after the introduction of UDCA.

 
  • References

  • 1 Lee J, Belanger A, Doucette JT, Stanca C, Friedman S, Bach N. Transplantation trends in primary biliary cirrhosis. Clin Gastroenterol Hepatol 2007; 5 (11) 1313-1315
  • 2 Mendes FD, Kim WR, Pedersen R, Therneau T, Lindor KD. Mortality attributable to cholestatic liver disease in the United States. Hepatology 2008; 47 (4) 1241-1247
  • 3 Poupon R, Chrétien Y, Poupon RE, Ballet F, Calmus Y, Darnis F. Is ursodeoxycholic acid an effective treatment for primary biliary cirrhosis?. Lancet 1987; 1 (8537) 834-836
  • 4 Prince MI, James OF. The epidemiology of primary biliary cirrhosis. Clin Liver Dis 2003; 7 (4) 795-819
  • 5 Matsumoto H. Mechanism of the pivot shift. J Bone Joint Surg Br 1990; 72 (5) 816-821
  • 6 Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med 2005; 353 (12) 1261-1273
  • 7 Kitic I, Boskovic A, Stankovic I, Prokic D. Twelve-year-old girl with primary biliary cirrhosis. Case Rep Pediatr 2012; 2012: 937150
  • 8 Kim WR, Lindor KD, Locke III GR , et al. Epidemiology and natural history of primary biliary cirrhosis in a US community. Gastroenterology 2000; 119 (6) 1631-1636
  • 9 James OF, Bhopal R, Howel D, Gray J, Burt AD, Metcalf JV. Primary biliary cirrhosis once rare, now common in the United Kingdom?. Hepatology 1999; 30 (2) 390-394
  • 10 Baldursdottir TR, Bergmann OM, Jonasson JG, Ludviksson BR, Axelsson TA, Björnsson ES. The epidemiology and natural history of primary biliary cirrhosis: a nationwide population-based study. Eur J Gastroenterol Hepatol 2012; 24 (7) 824-830
  • 11 Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ. American Association for Study of Liver Diseases. Primary biliary cirrhosis. Hepatology 2009; 50 (1) 291-308
  • 12 Solis Herruzo JA, Solis Munoz P, Munoz Yague T. The pathogenesis of primary biliary cirrhosis. Revista Espanola de Enfermedades Digestivas: Organo Oficial de la Sociedad Espanola de Patologia Digestiva; 2009. 101. 413-423
  • 13 McNally RJ, James PW, Ducker S, James OF. Seasonal variation in the patient diagnosis of primary biliary cirrhosis: further evidence for an environmental component to etiology. Hepatology 2011; 54 (6) 2099-2103
  • 14 Varyani FK, West J, Card TR. An increased risk of urinary tract infection precedes development of primary biliary cirrhosis. BMC Gastroenterol 2011; 11: 95
  • 15 Chen RC, Naiyanetr P, Shu SA , et al. Antimitochondrial antibody heterogeneity and the xenobiotic etiology of primary biliary cirrhosis. Hepatology 2013; 57 (4) 1498-1508
  • 16 Sasaki M, Kakuda Y, Miyakoshi M, Sato Y, Nakanuma Y. Infiltration of inflammatory cells expressing mitochondrial proteins around bile ducts and in biliary epithelial layer may be involved in the pathogenesis in primary biliary cirrhosis. J Clin Pathol 2014; 67 (6) 470-476
  • 17 Corpechot C, Gaouar F, Chrétien Y, Johanet C, Chazouillères O, Poupon R. Smoking as an independent risk factor of liver fibrosis in primary biliary cirrhosis. J Hepatol 2012; 56 (1) 218-224
  • 18 Hirschfield GM, Liu X, Xu C , et al. Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants. N Engl J Med 2009; 360 (24) 2544-2555
  • 19 Jin Q, Moritoki Y, Lleo A , et al. Comparative analysis of portal cell infiltrates in antimitochondrial autoantibody-positive versus antimitochondrial autoantibody-negative primary biliary cirrhosis. Hepatology 2012; 55 (5) 1495-1506
  • 20 Dinani AM, Fischer SE, Mosko J, Guindi M, Hirschfield GM. Patients with autoimmune hepatitis who have antimitochondrial antibodies need long-term follow-up to detect late development of primary biliary cirrhosis. Clin Gastroenterol Hepatol 2012; 10 (6) 682-684
  • 21 Mattalia A, Quaranta S, Leung PS , et al. Characterization of antimitochondrial antibodies in health adults. Hepatology 1998; 27 (3) 656-661
  • 22 Gatselis NK, Zachou K, Norman GL , et al. Clinical significance of the fluctuation of primary biliary cirrhosis-related autoantibodies during the course of the disease. Autoimmunity 2013; 46 (7) 471-479
  • 23 Rudic JS, Poropat G, Krstic MN, Bjelakovic G, Gluud C. Ursodeoxycholic acid for primary biliary cirrhosis. Cochrane Database Syst Rev 2012; 12: CD000551
  • 24 Zhang Y, Lu J, Dai W , et al. Combination therapy of ursodeoxycholic acid and corticosteroids for primary biliary cirrhosis with features of autoimmune hepatitis: a meta-analysis. Gastroenterol Res Pract 2013; 2013: 490731
  • 25 Ozaslan E, Efe C, Heurgue-Berlot A , et al. Factors associated with response to therapy and outcome of patients with primary biliary cirrhosis with features of autoimmune hepatitis. Clin Gastroenterol Hepatol 2014; 12 (5) 863-869
  • 26 Levy C, Naik J, Giordano C , et al. Hispanics with primary biliary cirrhosis are more likely to have features of autoimmune hepatitis and reduced response to ursodeoxycholic acid than non-Hispanics. Clin Gastroenterol Hepatol 2013;
  • 27 Boberg KM, Wisløff T, Kjøllesdal KS, Støvring H, Kristiansen IS. Cost and health consequences of treatment of primary biliary cirrhosis with ursodeoxycholic acid. Aliment Pharmacol Ther 2013; 38 (7) 794-803
  • 28 Papastergiou V, Tsochatzis EA, Rodriquez-Peralvarez M , et al. Biochemical criteria at 1.  year are not robust indicators of response to ursodeoxycholic acid in early primary biliary cirrhosis: results from a 29-year cohort study. Aliment Pharmacol Ther 2013; 38 (11-12) 1354-1364
  • 29 Carbone M, Mells GF, Pells G , et al UK PBC Consortium. Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic acid. Gastroenterology 2013; 144 (3) 560-569 , e7, quiz e13–e14
  • 30 Kuiper EM, Hansen BE, Adang RP , et al; Dutch PBC Study Group. Relatively high risk for hepatocellular carcinoma in patients with primary biliary cirrhosis not responding to ursodeoxycholic acid. Eur J Gastroenterol Hepatol 2010; 22 (12) 1495-1502
  • 31 Myers RP, Swain MG, Lee SS, Shaheen AA, Burak KW. B-cell depletion with rituximab in patients with primary biliary cirrhosis refractory to ursodeoxycholic acid. Am J Gastroenterol 2013; 108 (6) 933-941
  • 32 Lens S, Leoz M, Nazal L , et al. Bezafibrate normalizes alkaline phosphatase in primary biliary cirrhosis patients with incomplete response to ursodeoxycholic acid. Liver Int 2014; 34 (2) 197-203
  • 33 Trivedi PJ, Kumagi T, Al-Harthy N , et al. Good maternal and fetal outcomes for pregnant women with primary biliary cirrhosis. Clin Gastroenterol Hepatol 2013;
  • 34 Neuberger J. Liver transplantation for primary biliary cirrhosis. Autoimmun Rev 2003; 2 (1) 1-7
  • 35 Carbone M, Bufton S, Monaco A, Griffiths L, Jones DE, Neuberger JM. The effect of liver transplantation on fatigue in patients with primary biliary cirrhosis: a prospective study. J Hepatol 2013; 59 (3) 490-494
  • 36 Neuberger J, Portmann B, Macdougall BR, Calne RY, Williams R. Recurrence of primary biliary cirrhosis after liver transplantation. N Engl J Med 1982; 306 (1) 1-4
  • 37 Liermann Garcia RF, Evangelista Garcia C, McMaster P, Neuberger J. Transplantation for primary biliary cirrhosis: retrospective analysis of 400 patients in a single center. Hepatology 2001; 33 (1) 22-27
  • 38 Charatcharoenwitthaya P, Pimentel S, Talwalkar JA , et al. Long-term survival and impact of ursodeoxycholic acid treatment for recurrent primary biliary cirrhosis after liver transplantation. Liver Transpl 2007; 13 (9) 1236-1245
  • 39 Sanchez EQ, Levy MF, Goldstein RM , et al. The changing clinical presentation of recurrent primary biliary cirrhosis after liver transplantation. Transplantation 2003; 76 (11) 1583-1588
  • 40 Levitsky J, Hart J, Cohen SM, Te HS. The effect of immunosuppressive regimens on the recurrence of primary biliary cirrhosis after liver transplantation. Liver Transpl 2003; 9 (7) 733-736
  • 41 Khettry U, Anand N, Faul PN , et al. Liver transplantation for primary biliary cirrhosis: a long-term pathologic study. Liver Transpl 2003; 9 (1) 87-96
  • 42 Sebagh M, Farges O, Dubel L, Samuel D, Bismuth H, Reynes M. Histological features predictive of recurrence of primary biliary cirrhosis after liver transplantation. Transplantation 1998; 65 (10) 1328-1333
  • 43 Manousou P, Arvaniti V, Tsochatzis E , et al. Primary biliary cirrhosis after liver transplantation: influence of immunosuppression and human leukocyte antigen locus disparity. Liver Transpl 2010; 16 (1) 64-73
  • 44 Silveira MG, Talwalkar JA, Lindor KD, Wiesner RH. Recurrent primary biliary cirrhosis after liver transplantation. Am J Transplant 2010; 10 (4) 720-726