Clinical Analysis of 75 Patients with Primary Biliary Cirrhosis

Abstract

Background and aims: Primary biliary cirrhosis is a chronic and slowly progressive cholestatic liver disease characterized by destruction of interlobular bile ducts, which if untreated, leads to fibrosis, cirrhosis and liver failure. It is more frequent among female patients and is usually diagnosed in the fifth decade of life. This study aims to determine the demographic, clinical, biochemical and serological characteristics and histological stage of patients with primary biliary cirrhosis. Methods: Retrospective analysis of the adult patients diagnosed with primary biliary cirrhosis at our center from January 2005 to December 2013 was performed. Data collection included demographics, clinical features, biochemical and serological markers, and histological stage. Results: 75 patients were diagnosed with primary biliary cirrhosis (mean age: 55 years, range: 19-83), of whom 92.0% were women. The most common symptoms at presentation were fatigue (40.0%), pruritus (40.0%), jaundice (28.0%) and dark urine (26.7%). 20.0% were asymptomatic at diagnosis. 48.0% of patients had cirrhosis at presentation. Positive antimitochondrial antibodies were found in 96% of cases. 34.8% of the patients were positive for antinuclear antibodies. Overlap syndromes were present in 10.6%. Liver biopsy was performed in 45.3% of the patients. Conclusions: The clinical features of primary biliary cirrhosis were similar to those reported in the international literature but with a high percentage of symptomatic and cirrhotic patients at diagnosis.