Primary biliary cirrhosis - An uncommon disease in Singapore

Abstract

Primary biliary cirrhosis (PBC) is uncommon in Singapore. Twelve consecutive patients with PBC were seen between 1987 and 1994 at the National University Hospital. Eleven were women and the mean age at presentation was 53 years. Three patients presented with pruritus and jaundice whilst three had decompensated cirrhosis. The remaining six patients had no symptoms attributed to their liver disease when first detected, three of them presented with associated conditions including sicca syndrome and interstitial lung fibrosis, lichen planus, and carcinoma of breast. All patients had elevated serum alkaline phosphatase and positive anti-mitochondrial antibodies. Liver histology (10/12) showed Stage II disease (2), Stage III (5) and Stage IV (3). Three patients also had co-existing gall bladder stones but their endoscopic retrograde cholangiograms were normal. The mean follow-up period was 32.6 months and four patients died during follow-up. The only male patient had liver transplantation, two patients had symptomatic treatment while the rest were treated with ursodeoxycholic acid. In conclusion, local patients tended to presented relatively early in the course of the disease with 50% being asymptomatic and in the precirrhotic Stages.