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Primary Biliary Cirrhosis

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

Abstract

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].

Keywords

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.

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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

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