Characteristics of primary biliary cirrhosis in Slovenian patients. Analysis of 169 patients in the period from 1984 to 2010

Abstract

Background: The aim of our study was to determine clinical and laboratory features, symptom progression and outcome of the disease in Slovenian patients with primary biliary cirrhosis (PBC). Methods: We reviewed medical records of patients with PBC treated at the Clinical Department of Gastroenterology of the University Medical Centre Ljubljana in the period from 1984 to 2010. The diagnosis of PBC was based on the clinical criteria, which were compliant with recent recommendations of the European Association for the Study of the Liver (EASL). Apart from patients’ demographic characteristics, we examined the outcome of the disease, occurrence of symptoms and signs of cirrhosis, as well as concomitant diseases, such as metabolic bone disease, autoimmune diseases and cancer. For data collection we used methods of descriptive statistics. Results: In the group of 169 patients with PBC we found that 96.5 % of patients were females at an average age of 53 years at the time of diagnosis. While 50 % of the patients were asymptomatic, 6 % of patients had liver cirrhosis at the time of diagnosis. In the majority of patients PBC had a slow progression rate.. By the end of the follow-up period, after 8.03 years on average, , 21 % of patients developed liver cirrhosis. Liver transplantation was performed in 8 % of the patients. At the end of the observation period, 54 % were still asymptomatic. Concomitant autoimmune diseases and osteopenia/osteoporosis were found in 42 % of our PBC patients. Most frequent autoimmune diseases were autoimmune thyroid disease and Sjoegrens’ syndrome. Conclusions: In this first investigation of PBC in Slovenian patients we found that the features and course of PBC differ in some aspects from other patients’ populations in the western countries. The difference in our group of patients was an exceptionally low number of males and the high proportion of asymptomatic patients at the end of the observation period. We speculate that the aetiology of liver diseases in male patients in Slovenia is to frequently assigned to excessive alcohol consumption and that this attitude needs to be changed. The high number of asymptomatic PBC patients at the end of the observation period could be due to the consistent treatment with ursodeoxycholic acid.