Primary Biliary Cirrhosis
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  to over 10 years currently .
KeywordsPortal Hypertension Primary Biliary Cirrhosis Serum Bilirubin Prognostic Index Graft Versus Host Disease
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- 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.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
- 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
- 6.James OFW, Macklon AF, Watson AT (1981) Primary biliary cirrhosis: a revised clinical spectrum. Lancet i:1278Google Scholar
- 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.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
- 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
- 15.Stellon AJ (personal communication)Google Scholar