Primary biliary cirrhosis is an uncommon disease amongst Malaysians. Over a 12-year period, between 1979 and 1991, only seven patients with clinical, biochemical and histologic evidence of primary biliary cirrhosis were identified in University Hospital Kuala Lumpur. All were Chinese females between the ages of 30 to 55 years. The presenting complaint was pruritus in 5 patients. All except one patient was jaundiced when the diagnosis was made. These patients were followed up from 1 to 11 years. Three deaths were reported, one from massive hemetemesis and two from liver failure.
A 43-year-old lady with long standing non-insulin dependent diabetes mellitus on glibenclamide presented with cholestatic liver disease. Initially she was thought to have developed primary biliary cirrhosis (PBC). When she made a spontaneous recovery following the withdrawal of glibenclamide, it became obvious that the patient had been suffering from drug-induced chronic cholestasis (DICC). The subtle differences between PBC and DICC are highlighted.
Primary Biliary Cirrhosis (PBC) and Auto Immune Hepatitis (AIH) are autoimmune diseases of the liver which highlighted with slow destructive process of intra hepatic small bile ducts. As a result of these damages cholestasis and over the time tissue damage will happen, which leads to scarring, fibrosis and finally cirrhosis. Some patients may present with clinical and biochemical features of both conditions, which is called "Overlap syndrome". Here we are reporting a case of PBC-AIH overlap syndrome that primarily diagnosed as gallbladder carcinoma and went under operation and finally histological examination revealed to be PBC.
Primary biliary cirrhosis (PBC) is uncommonly described from Asia and it is an extremely rare cause of chronic liver disease in India. Six first generation migrant Asian patients with PBC were seen at the Liver Unit, Queen Elizabeth Hospital, Birmingham during the period 1982-94. All were women and their ages at presentation ranged from 31 to 63 (median 40) years. All were symptomatic for a median of 6 months prior to referral to the unit for transplantation. Itching with or without jaundice was a common presenting feature. Diagnosis was based on raised serum IgM levels, presence of antimitochondrial antibody (titres 100-400) and diagnostic histology. Only one patients had an associated autoimmune disease (coeliac disease). Serum bilirubin level was above 100 mumol/l at the time of presentation in four patients. Four of these patients with end-stage PBC are first generation migrants from south Asia, who have been resident in the West Midlands for the past 10 to 34 years. The total south Asian population of the West Midlands is 276,754; thus, from these four patients alone the estimated prevalence of PBC in the migrant south Asian population is at least 14 per million. However, such data cannot be used to give any accurate assessment of prevalence, for which a population screening programme is required. A higher incidence in the migrant population than in their countries of origin is compatible with an environmental aetiology.
A prospective descriptive study of Primary Biliary Cirrhosis at Hospital Kuala Lumpur was undertaken from January 1992 to December 1999. A total of 17 patients were seen with a female to male ratio of 3.25:1. The mean age at presentation was 45.9 years (range: 14 years to 67 years) with a mean follow-up of 33.4 months (range: 3 months to 95 months). Fatigue was the most common clinical symptom at presentation. Alanine transaminase and alkaline phosphatase levels were elevated in 93% of patients at presentation. The antimitochondrial antibody was positive in 87% of patients. Ursodeoxycholic acid therapy resulted in significant symptomatic relief and biochemical improvement in all those who were treated.