Primary Biliary Cirrhosis

  • Roger Williams
  • J. Neuberger
  • M. R. Lucey
Conference paper


During the last few years, understanding of the natural history of primary biliary cirrhosis (PBC) has undergone considerable revision, with realisation of the great variability of presentation and progression. The majority of patients are middle-aged women who present with a history of gradual onset of pruritus, increasing skin pigmentation and subsequently signs and symptoms of cholestatic liver disease. Others will present with a history of portal hypertension with gastrointestinal haemorrhage, or as a consequence of hypersplenism. Others, with a poorer prognosis, present with signs of decompensated liver disease. It is becoming increasingly recognised that there is a substantial pool of patients, who present either with some of the associated conditions of PBC (such as sicca syndrome, sclerodactyly or Raynaud’s phenomenon) or who are truly asymptomatic and who are detected because of abnormal liver function tests or antimitochondrial antibodies (AMA) on routine screening. Most, but not all, present with early histological disease; some, at presentation, have an established cirrhosis. In this asymptomatic group, life expectancy is probably not significantly different from that of a normal, matched population [6, 11]. Thus, despite the lack of proven effective specific treatment for the disease, due to recognition of patients at an earlier stage there has been an apparent improvement in prognosis from 5 years twenty-five years ago [14] to over 10 years currently [2].


Portal Hypertension Primary Biliary Cirrhosis Serum Bilirubin Prognostic Index Graft Versus Host Disease 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Christensen E, Neuberger J, Crowe J et al. (in press) Beneficial effect of azathioprine and prediction of prognosis in primary biliary cirrhosis. Final results of an international trial. GastroenterologyGoogle Scholar
  2. 2.
    Eggink HF, Houthoff HJ, Huitema S, Gips CH, Poppema S (1982) Cellular and humoral reactions in chronic liver disease. I. Clin Exp Immunol 50:17Google Scholar
  3. 3.
    Goldberg MJ, Kaplan MM, Anderson CL (1982) Evidence against an immune complex pathogenesis of primary biliary cirrhosis. Gastroenterology 83:677PubMedGoogle Scholar
  4. 4.
    Howat HT, Ralston AJ, Varley H, Wilson JAC (1966) The late results of long-term treatment of primary biliary cirrhosis by corticosteroids. Revue Internat. d’Hepatologie 16:227Google Scholar
  5. 5.
    James OFW (1985) D-penicillamine for primary biliary cirrhosis. Gut 26: 109PubMedCrossRefGoogle Scholar
  6. 6.
    James OFW, Macklon AF, Watson AT (1981) Primary biliary cirrhosis: a revised clinical spectrum. Lancet i:1278Google Scholar
  7. 7.
    Jones EA (1985) Primary biliary cirrhosis: potential pathogenic mechanisms and the effects of therapies on the disease process. In: Thomas HC, MacSween RNM (eds) Advances in Hepatology, vol 1. Churchill-Livingstone, Edinburgh, p 131Google Scholar
  8. 8.
    MacSween RNM, Sumithran E (1981) Histopathology of primary biliary cirrhosis. In: Jones EA (ed) Seminars in Liver Diseases, vol 1. Thieme-Stratton, New York p 282Google Scholar
  9. 9.
    Miller KB, Sepersky RA, Bronn KM, Goldberg MJ, Kaplan MM (1983) Genetic abnormalities and immunoregulation in primary biliary cirrhosis. Am J Med 75:75PubMedCrossRefGoogle Scholar
  10. 10.
    Robson S, Neuberger J, Keller HP, Abisch E, Niederberger W, Graffenried B von, Williams R (1984) Pharmacokinetic studies of Cyclosporin A ( Sandimmun) in patients with primary biliary cirrhosis. Br J Clin Pharmacol 18:627Google Scholar
  11. 11.
    Roll J, Boyer JL, Barry O, Klatskin G (1983) The prognostic importance of clinical and histological features in asymptomatic and symptomatic primary biliary cirrhosis. N Engl J Med 308:1PubMedCrossRefGoogle Scholar
  12. 12.
    Routhier G, Epstein O, Janossy G, Thomas HC, Sherlock S, Kung PC, Goldstein G (1980) Effects of cyclosporin on suppressor and inducer T lymphocytes in primary biliary cirrhosis. Lancet 2:1223PubMedCrossRefGoogle Scholar
  13. 13.
    Schultheiss HP, Berg P, Klingenberg M (1983) The mitochondrial adenine nucleotide translocator is an antigen in primary biliary cirrhosis. Clin Exp Immunol 54:648PubMedGoogle Scholar
  14. 14.
    Sherlock S (1959) Primary biliary cirrhosis (chronic intrahepatic obstructive jaundice). Gastroenterology 37:574PubMedGoogle Scholar
  15. 15.
    Stellon AJ (personal communication)Google Scholar

Copyright information

© Sandoz Ltd, Basle 1985

Authors and Affiliations

  • Roger Williams
  • J. Neuberger
  • M. R. Lucey
    • 1
  1. 1.The Liver UnitKing’s College School of Medicine and DentistryLondonUK

Personalised recommendations