Primary Biliary Cirrhosis (PBC) is a relatively rare cholestatic liver disease. The first case was described by Addison and Gull in 1851. The name PBC is generally accepted, however in fact this is a misnomer since cirrhosis is found in a minority of patients. PBC is one of the most frequent causes of the “vanishing bile duct syndrome”, characterized by cholestasis due to destruction and progressive disappearance of small intrahepatic bile ducts. Until a few decades ago no effective medical treatment was available and the prognosis for patients with symptomatic disease was poor, with reported survival less than 10 years. The pathogenesis of PBC has still not been resolved, but nowadays an effective therapy is available and this has significantly improved the prospects for patients with this condition.

Additional Metadata
Keywords gastroenterology, hepatology, liver diseases, primary biliary cirrhosis
Promotor H.L.A. Janssen (Harry)
Publisher Erasmus University Rotterdam
Sponsor Zambon Nederland, Erasmus University Medical Center, J.E. Jurriaanse Stichting, Nederlandse vereniging voor Gastroenterologie, Tramedico, Genzyme Europe, Dr. Falk Ph. Benelux, Novartis Pharma, Ferring Pharmaceuticals
ISBN 978-90-8559-106-1
Persistent URL
Kuiper, E.M.M. (2010, November 24). Primary biliary cirrhosis: Diagnostic and therapeutic aspects. Erasmus University Rotterdam. Retrieved from