Primary Biliary Cholangitis in Graz: Presentation, Course, and Outcome

Abstract

The presentation, clinical course, and outcome in patients with primary biliary cholangitis (PBC) may vary in different geographic regions. Data on this disease entity in central Europe are scarce. Therefore, the aim of this work was to characterize PBC patients at the Medical University of Graz. Data at time of diagnosis and from the last visit (follow-up) were obtained retrospectively from records of patients at our institution from 2004–2015. We identified 95 patients with PBC (89 women, 6 men). Median age at time of diagnosis was 53 years. Alkaline phosphatase (AP) was elevated less than 2‑fold above the upper limit of norm in 60%. Clinical symptoms (pruritus, fatigue, upper quadrant abdominal pain, keratoconjunctivitis sicca) were present in 39% of cases. Liver histology was available in 35% (stage I, n = 28; stage II, n = 5; stage III, n = 1; stage IV, n = 1). Using FibroScan®, 69% of patients had F0 or F1 liver fibrosis. Liver cirrhosis at follow-up was present in 10 patients (11%). Two liver-related deaths were encountered. Inadequate response to therapy with ursodeoxycholic acid was found in 30% of cases. In summary, patient outcome in our population seems to be markedly better than reported before, while the demographic data are comparable with other published studies. This might be attributed to lower levels of AP at the time of diagnosis compared to other studies.