Primary biliary cirrhosis: New perspectives in diagnosis and treatment

Postgraduate Medical Journal

Volumn 76, Issue 894, 2000, Pages 199-206

  Prince, Martin I ^a   Jones, David E J ^a  

^a NEWCASTLE UNIVERSITY   (United Kingdom)

Author keywords

Liver transplantation; Primary biliary cirrhosis; Pruritus

Indexed keywords

ANTIOXIDANT; AZATHIOPRINE; BILE ACID; BISPHOSPHONIC ACID DERIVATIVE; COLCHICINE; COLESTIPOL; COLESTYRAMINE; CYCLOSPORIN; METHOTREXATE; NALTREXONE; ONDANSETRON; OPIATE ANTAGONIST; PENICILLAMINE; PHENOBARBITAL; PREDNISOLONE; RIFAMPICIN; UDCA; UNCLASSIFIED DRUG; VITAMIN D;

BREAST CARCINOMA; CELIAC DISEASE; DEATH; DOSE RESPONSE; DRUG MECHANISM; FATIGUE; GASTROINTESTINAL DISEASE; HUMAN; HYPERPIGMENTATION; IMMUNOFLUORESCENCE; JAUNDICE; LIVER BIOPSY; LIVER CARCINOMA; LIVER FAILURE; LIVER TRANSPLANTATION; OSTEOPOROSIS; PRIMARY BILIARY CIRRHOSIS; PROGNOSIS; PRURITUS; REVIEW; SJOEGREN SYNDROME; THYROID DISEASE;

HUMANS; LIVER CIRRHOSIS, BILIARY; LIVER TRANSPLANTATION; PROGNOSIS; PRURITUS;

EID: 0034068374     PISSN: 00325473     EISSN: None     Source Type: Journal    
DOI: 10.1136/pmj.76.894.199     Document Type: Review

References (113)

1. Ahrens E, Payne M, Kunkel H, et al. Primary biliary cirrhosis. Medicne 1950;29:299-366.
2. Addison T, Gull W. On a certain affliction of the skin-vitiligoides-a planus tuberosa. Guys Hospital Review 1857;7:268.
3. Sherlock S, Scheuer P. The presentation and diagnosis of 100 patients with primary biliary cirrhosis. N Engl J Med 1973;289:674-8.
4. Walker J, Doniach D, Roitt I, et al. Serological tests in diagnosis of primary biliary cirrhosis. Lancet 1965;i:827-33.
5. Eriksson S, Lindgren S. The prevalence and clinical spectrum of primary biliary cirrhosis in a defined population. Scand J Gastroenterol 1984;19:971-6.
6. Christensen E, Crowe J, Doniach D, et al. Clinical pattern and course of disease in primary biliary cirrhosis based on an analysis of 236 patients. Gastroenterology 1980;78:236-46.
7. James O, Macklon AF, Watson AJ. Primary biliary cirrhosis - a revised clinical spectrum. Lancet 1981;i:1278-81.
8. Witt-Sullivan H, Heathcote J, Cauch K, et al. The demography of primary biliary cirrhosis in Ontario, Canada, Hepatology 1990;12:98-105.
9. Scheuer P. Primary biliary cirrhosis. Proceedings of the Royal Society of Medicine 1967;40:1257.
10. Kaplan M. Primary biliary cirrhosis. N Engl J Med 1987;316:521-8.
11. Invernizzi P, Crosignani A, Battezzati PM, et al. Comparison of the clinical features and clinical course of antimitochondrial antibody-positive and negative primary biliary cirrhosis. Hepatology 1997;25:1090-5.
12. Lacerda MA, Ludwig J, Dickson ER, et al. Antimitochondrial antibody-negative primary biliary cirrhosis. Am J Gastroenterol 1995;90:247-9.
13. Mattalia A, Quaranta S, Leung PS, et al. Characterization of antimitochondrial antibodies in health adults. Hepatology 1998;27:656-61.
14. Turchany JM, Uibo R, Kivik T, et al. A study of antimitochondrial antibodies in a random population in Estonia. Am J Gastroenterol 1997;92:124-6.
15. Berg PA, Doniach D, Roitt IM. Mitochondrial antibodies in primary biliary cirrhosis. I. Localization of the antigen to mitochondrial membranes. J Exp Med 1967;126:277-90.
16. Chazouilleres O, Wendum D, Serfaty L, et al. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology 1998;28:296-301.
17. Mitchison HC, Bassendine MF, Hendrick A, et al. Positive antimitochondrial antibody but normal alkaline phosphatase: is this primary biliary cirrhosis? Hepatology 1986;6:1279-84.
18. Metcalf J, Mitchison H, Palmer J, et al. Natural history of early primary biliary cirrhosis. Lancet 1996;348:1399-402.
19. Gershwin M, Mackay I, Sturgess A, et al. Identification of specificity of a cDNA encoding the 70kD mitochondrial antigen recognised in primary biliary cirrhosis. J Immunol 1987;138:3525-31.
20. Yeaman S, Fussey S, Danner D, et al. Primary biliary cirrhosis: identification of two common M2 mitochondrial autoanitgens. Lancet 1988;i:1067-70.
21. Michieletti P, Wanless IR, Katz A, et al. Antimitochondrial antibody negative primary biliary cirrhosis: a distinct syndrome of autoimmune cholangitis. Gut 1994;35:260-5.
22. Goodman ZD, McNally PR, Davis DR, et al. Autoimmune cholangitis: a variant of primary biliary cirrhosis. Clinicopathologic and serologic correlations in 200 cases. Dig Dis Sci 1995;40:1232-42.
23. Jorgensen RA, Dickson ER, Hofmann AF, et al. Characterisation of patients with a complete biochemical response to ursodeoxycholic acid. Gut 1995;36:935-8.
24. Melegh B, Skuta G, Pajor L, et al. Autoantibodies against subunits of pyruvate dehydrogenase and citrate synthase in a case of paediatric biliary cirrhosis. Gut 1998;42:753-6.
25. Jones D, Watt F, Metcalf J, et al. Familial primary biliary cirrhosis: a geographically based population study. J Hepatol 1999;30:402-7.
26. Agarwal K, Jones D, Bassendine M. Genetic susceptability to primary biliary cirrhosis. Eur J Gastroenterol Hepatol 1999 (in press).
27. Metcalf J, James O. The geoepidemiology of primary biliary cirrhosis. Semin Liv Dis 1996;17:13-22.
28. Metcalf J, Bhopal R, Gray J, et al. Incidence and prevalence of primary biliary cirrhosis in the city of Newcastle upon Tyne, England. Int J Epidemiol 1997;26:830-6.
29. Kingham JG, Parker DR. The association between primary biliary cirrhosis and coeliac disease: a study of relative prevalences. Gut 1998;42:120-2.
30. James OF, Bhopal R, Howel D, et al. Primary biliary cirrhosis once rare, now common in the United Kingdom? Hepatology 1999;30:390-4.
31. Triger D. Primary biliary cirrhosis: an epidemiological study. BMJ 1980;281:772-5.
32. Butler P, Valle F, Hamilton-Miller JM, et al. M2 mitochondrial antibodies and urinary rough mutant bacteria in patients with primary biliary cirrhosis and in patients with recurrent bacteriuria. J Hepatol 1993;17:408-14.
33. Meilof JF, Smeenk RJ. Detection of retroviral antibodies in primary biliary cirrhosis [letter]. Lancet 1998;352:739-40.
34. Mason AL, Xu L, Guo L, et al. Detection of retroviral antibodies in primary biliary cirrhosis and other idiopathic biliary disorders. Lancet 1998,351:1620-4.
35. Vilagut L, Vila J, Vinas O, et al. Cross-reactivity of anti-Mycobacterium gordonae antibodies with the major mitochondrial autoantigens in primary biliary cirrhosis [see comments]. J Hepatol 1994;21:673-7.
36. Jones DE, Palmer JM, Yeaman SJ, et al. Breakdown of tolerance to pyruvate dehydrogenase complex in experimental autoimmune cholangitis: a mouse model of primary biliary cirrhosis. Hepatology 1999;30:65-70.
37. Bassendine M, Palmer J, Decruz D, et al. Approaches to a murine model of AMA positive non-suppurative destructive cholangitis (NSDC). J Hepatol 1998;28:124.
38. Heathcote E. The clinical expression of primary biliary cirrhosis. Semin Liv Dis 1996;17:23-33.
39. Long RG, Scheuer PJ, Sherlock S. Presentation and course of asymptomatic primary biliary cirrhosis. Gastroenterology 1977;72:1204-7.
40. Prince M, James O, Jones D. Fatigue in patients with primary biliary cirrhosis: validation of fatigue severity score as a clinical and research tool. Hepatology 1998;28:542A.
41. Cauch-Dudek K, Abbey S, Stewart DE, et al. Fatigue in primary biliary cirrhosis. Gut 1998,43:705-710.
42. Cauch-Dudek K, Heathcote E, Hutere N, et al. Is disturbed sleep, based on objective polysomnographic indicators, a factor in the fatigue of patients with primary biliary cirrhosis. Hepatology 1998;28:A543.
43. Wanless I, Heathcote E, Cauch K. Detailed histological grading as a predictor of morbidity and mortality in primary biliary cirrhosis. Hepatology 1993;18:174A.
44. Jones E. Fatigue associated with chronic liver disease: wrapped inside a mystery inside an enigma. Hepalology 1995,22:1606-8.
45. Swain MG, Le T. Chronic cholestasis in rats induces anhedonia and a loss of social interest. Hepatology 1998;28:6-10.
46. Swain M, Marie M. Improvement in cholestasis-associated fatigue with a serotonin receptor agonist using a novel rat model of fatigue assessment. Hepatology 1997,25:291-4.
47. Bergasa N, Jones E. The pruritus of cholestasis: potential pathogenic and therapeutic implications of opioids. Gastroemerology 1995;108:1582-8.
48. Bergasa NV, Talbot TL, Ailing DW, et al. A controlled trial of naloxone infusions for the pruritus of chronic cholestasis. Gastroenterology 1992,102:544-9.
49. Wolfhagen FH, Sternieri E, Hop WC, et al. Oral naltrexone treatment for cholestatic pruritus: a double-blind, placebo-controlled study. Gastroenterology 1997;113:1264-9.
50. Crippin JS, Lindor KD, Jorgensen R, et al. Hypercholesterolemia and atherosclerosis in primary biliary cirrhosis: what is the risk? Hepatology 1992;15:858-62.
51. Zukowski TH, Jorgensen RA, Dickson ER, et al. Autoimmune conditions associated with primary biliary cirrhosis: response to ursodeoxycholic acid therapy. Am J Gastroenterol 1998;93:958-61.
52. Eastell R, Dickson ER, Hodgson SF, et al. Rates of vertebral bone loss before and after liver transplantation in women with primary biliary cirrhosis. Hepatology 1991;14:296-300.
53. Matloff DS, Kaplan MM, Neer RM, et al. Osteoporosis in primary biliary cirrhosis: effects of 25-hydroxyvitamin D3 treatment. Gastroenterology 1982;83(1 pt 1):97-102.
54. Wolke AM, Schaffner F, Kapelman B, et al. Malignancy in primary biliary cirrhosis. High incidence of breast cancer in affected women. Am J Med 1984;76:1075-8.
55. Goudie BM, Burt AD, Boyle P, et al. Breast cancer in women with primary biliary cirrhosis. BMJ 1985;291:1597-8.
56. Howel D, Metcalf J, James O. Is cancer commoner in primary biliary cirrhosis? Hepatology 1998;28:540A.
57. Loof L, Adami HO, Sparen P, et al. Cancer risk in primary biliary cirrhosis: a population-based study from Sweden. Hepatology 1994;20(1 pt 1):101-4.
58. Jones DE, Metcalf JV, Collier JD, et al. Hepatocellular carcinoma in primary biliary cirrhosis and its impact on outcomes. Hepatology 1997;26:1138-42.
59. Shapiro JM, Smith H, Schaffner F. Serum bilirubin: a prognostic factor in primary biliary cirrhosis. Gut 1979;20:137-40.
60. Christensen E, Neuberger J, Crowe J, et al. Beneficial effect of azathioprine and prediction of prognosis in primary biliary cirrhosis. Final results of an international trial. Gastroenterology 1985;89:1084-91.
61. Christensen E, Altman DG, Neuberger J, et al. Updating prognosis in primary biliary cirrhosis using a time-dependent Cox regression model. PBC1 and PBC2 trial groups. Gastroenterology 1993;105:1865-76.
62. Dickson ER, Grambsch PM, Fleming TR, et al. Prognosis in primary biliary cirrhosis: model for decision making. Hepatology 1989;10:1-7.
63. Inoue K, Hirohara J, Nakano T, et al. Prediction of prognosis of primary biliary cirrhosis in Japan. Liver 1995; 15:70-7.
64. Wiesner RH, Porayko MK, Dickson ER, et al. Selection and timing of liver transplantation in primary biliary cirrhosis and primary sclerosing cholangitis. Hepatology 1992;16: 1290-9.
65. Murtaugh PA, Dickson ER, Van Dam GM, et al. Primary biliary cirrhosis: prediction of short-term survival based on repeated patient visits. Hepatology 1994;20(1 pt 1):126-34.
66. Bonsel GJ, Klompmaker IJ, van't Veer F, et al. Use of prognostic models for assessment of value of liver transplantation in primary biliary cirrhosis. Lancet 1990;335:493-7.
67. Grambsch PM, Dickson ER, Kaplan M, et al. Extramural cross-validation of the Mayo primary biliary cirrhosis survival model establishes its generalizability. Hepatology 1989;10:846-50.
68. van Dam GM, Verbaan BW, Therneau TM, et al. Primary biliary cirrhosis: Dutch application of the Mayo model before and after orthotopic liver transplantation. Hepatogastroenterology 1997;44:732-43.
69. Combes B, Carithers RL Jr, Maddrey WC, et al. A randomized, double-blind, placebo-controlled trial of ursodeoxycholic acid in primary biliary cirrhosis. Hepatology 1995;22:759-66.
70. Eriksson LS, Olsson R, Glauman H, et al. Ursodeoxycholic acid treatment in patients with primary biliary cirrhosis. A Swedish multicentre, double-blind, randomized controlled study. Scand J Gastroenterol 1997;32:179-86.
71. Lindor KD, Therneau TM, Jorgensen RA, et al. Effects of ursodeoxycholic acid on survival in patients with primary biliary cirrhosis. Gastroenterology 1996;110:1515-8.
72. Poupon RE, Balkau B, Eschwege E, et al. A multicenter, controlled trial of ursodiol for the treatment of primary biliary cirrhosis. UDCA-PBC Study Group. N Engl J Med 1991;324:1548-54.
73. Turner IB, Myszor M, Mitchison HC, et al. A two year controlled trial examining the effectiveness of ursodeoxycholic acid in primary biliary cirrhosis. J Gastroenterol Hepatol 1994;9:162-8.
74. Kilmurry MR, Heathcote EJ, Cauch-Dudek K, et al. Is the Mayo model for predicting survival useful after the introduction of ursodeoxycholic acid treatment for primary biliary cirrhosis? Hepatology 1996;23:1148-53.
75. Bonnard A, Heathcote E, Lindor K, et al. Clinical significance of bilirubin levels under ursodeoxycholic acid therapy in patients with primary biliary cirrhosis. Hepatology 1999;29;39-43.
76. Beswick DR, Klatskin G, Boyer JL. Asymptomatic primary biliary cirrhosis. A progress report on long-term follow-up and natural history. Gastroenterology 1985;89:267-71.
77. Mahl TC, Shockcor W, Boyer JL. Primary biliary cirrhosis: survival of a large cohort of symptomatic and asymptomatic patients followed for 24 years. J Hepatol 1994;20:707-13.
78. Roll J, Boyer JL, Barry D, et al. The prognostic importance of clinical and histologic features in asymptomatic and symptomatic primary biliary cirrhosis. N Engl J Med 1983;308:1-7.
79. Balasubramaniam K, Grambsch PM, Wiesner RH, et al. Diminished survival in asymptomatic primary biliary cirrhosis. A prospective study. Gastroenterology 1990;98: 1567-71.
80. Nyberg A, Loof L. Primary biliary cirrhosis: clinical features and outcome, with special reference to asymptomatic disease. Scand J Gastroenterol 1989;24:57-64.
81. Klein R, Pointner H, Zilly W, et al. Antimitochondrial antibody profiles in primary biliary cirrhosis distinguish at early stages between a benign and a progressive course: a prospective study on 200 patients followed for 10 years. Liver 1997;17:119-28.
82. Jones D, Watt F, Grove J, et al. Tumour necrosis factor-alpha promoter polymorphism in primary biliary cirrhosis. J Hepatol 1999;30:232-36.
83. Nyberg A, Engstrom-Laurent A, Loof L. Serum hyaluronate in primary biliary cirrhosis - a biochemical marker for progressive liver damage. Hepatology 1988;8:142-6.
84. Eriksson S, Zettervall O. The N-terminal propeptide of collagen type III in serum as a prognostic indicator in primary biliary cirrhosis. J Hepatol 1986;2:370-8.
85. Reichen J, Widmer T, Cotting J. Accurate prediction of death by serial determination of galactose elimination capacity in primary biliary cirrhosis: a comparison with the Mayo model. Hepatology 1991;14:504-10.
86. Beukers R, van Zanten RA, Schalm SW. Serial determination of type III procollagen amino propeptide serum levels in patients with histologically progressive and non-progressive primary biliary cirrhosis. J Hepatol 1992;14:22-9.
87. Clements D, Elias E, McMaster P. Preliminary study of indocyanine green clearance in primary biliary cirrhosis. Scand J Gastroenterol 1991;26:119-23.
88. Lindor KD, Lacerda MA, Jorgensen RA, et al. Relationship between biliary and serum bile acids and response to ursodeoxycholic acid in patients with primary biliary cirrhosis. Am J Gastroenterol 1998;93:1498-504.
89. Poupon RE, Lindor KD, Cauch-Dudek K, et al. Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis. Gastroenterology 1997;113: 884-90.
90. Poupon RE, Chretien Y, Poupon R, et al. Serum bile acids in primary biliary cirrhosis: effect of ursodeoxycholic acid therapy. Hepatology 1993;17:599-604.
91. Terasaki S, Nakanuma Y, Ogino H, et al. Hepatocellular and biliary expression of HLA antigens in primary biliary cirrhosis before and after ursodeoxycholic acid therapy. Am J Gastroenterol 1991;86:1194-9.
92. Heathcote EJ, Cauch-Dudek K, Walker V, et al. The Canadian multicenter double-blind randomized controlled trial of ursodeoxycholic acid in primary biliary cirrhosis. Hepatology 1994;19:1149-56.
93. Bateson MC, Gedling P. Ursodeoxycholic acid therapy for primary biliary cirrhosis. A 10-year British single-centre population-based audit of efficacy and survival. Postgrad Med J 1998;74:482-5.
94. Goulis J, Lerandro G, Burroughs A. No evidence for ursodeoxycholic acid therapy in primary biliary cirrhosis: a meta-analysis. Journal of Hepatology 1999;30(suppl 1):57.
95. Lindor KD, Dickson ER, Baldus WP, et al. Ursodeoxycholic acid in the treatment of primary biliary cirrhosis. Gastroenterology 1994;106:1284-90.
96. Bodenheimer HC Jr, Schaffner F, Sternlieb I, et al. A prospective clinical trial of D-penicillamine in the treatment of primary biliary cirrhosis. Hepatology 1985;5:1139-42.
97. Neuberger J, Christensen E, Portmann B, et al. Double blind controlled trial of d-penicillamine in patients with primary biliary cirrhosis. Gut 1985;26:114-9.
98. Kaplan MM, Knox TA. Treatment of primary biliary cirrhosis with low-dose weekly methotrexate. Gastroenterology 1991;101:1332-8.
99. Kaplan MM, DeLellis RA, Wolfe HJ. Sustained biochemical and histologic remission of primary biliary cirrhosis in response to medical treatment [see comments]. Ann Intern Med 1997;126:682-8.
100. Lombard M, Portmann B, Neuberger J, et al. Cyclosporin A treatment in primary biliary cirrhosis: results of a long-term placebo controlled trial. Gastroenterology 1993; 104:519-26.
101. Poupon RE, Huet PM, Poupon R, et al. A randomized trial comparing colchicine and ursodeoxycholic acid combination to ursodeoxycholic acid in primary biliary cirrhosis. UDCA-PBC Study Group. Hepatology 1996;24:1098-103.
102. Kaplan MM, Ailing DW, Zimmerman HJ, et al. A prospective trial of colchicine for primary biliary cirrhosis. N Engl J Med 1986;315:1448-54.
103. Bodenheimer H Jr, Schaffner F, Pezzullo J. Evaluation of colchicine therapy in primary biliary cirrhosis. Gastroenterology 1988;95:124-9.
104. Mitchison HC, Palmer JM, Bassendine MF, et al. A controlled trial of prednisolone treatment in primary biliary cirrhosis. Three-year results. J Hepatol 1992;15:336-44.
105. Ghent CN, Carruthers SG. Treatment of pruritus in primary biliary cirrhosis with rifampin. Results of a double-blind, crossover, randomized trial. Gastroenterology 1988;94: 488-93.
106. Bachs L, Pares A, Elena M, et al. Comparison of rifampicin with phenobarbitone for treatment of pruritus in biliary cirrhosis. Lancet 1989;i:574-6.
107. Horsmans Y, Geubel A. Pruritus associated with chronic liver disease. Ann Intern Med 1996;125:701.
108. O'Donohoue J, Haigh C, Williams R. Ondansetron in the treatment of the pruritus of cholestasis: a randomised controlled trial. Gut 1997;40(S1):A12.
109. Watson J, Jones D, James O, et al. Oral antioxidant therapy for the treatment of primary biliary cirrhosis: a pilot study. J Gastroenterol Hepatol 1999 (in press).
110. Castedal M, Olsson R, Mattson L-A, et al. Estrogengestagen therapy for low bone mineral density in primary biliary cirrhosis. Hepatology 1998;28(4 suppl):544A.
111. Neuberger J. Transplantation for primary biliary cirrhosis. Semin Liv Dis 1997;17:137-46.
112. Neuberger J, Altman DG, Poison R, et al. Prognosis after liver transplantation for primary biliary cirrhosis. Transplantation 1989;48:444-7.
113. Gross CR, Malinchoc M, Kim WR, et al. Quality of life before and after liver transplantation for cholestatic liver disease. Hepatology 1999;29:356-64.