Published: 2 February 2024
Author(s): M.C. van Hooff, E. Werner, A.J. van der Meer
Issue: May 2024
Section: Review Article

Primary biliary cholangitis (PBC), which was formerly called primary biliary cirrhosis, is a rare chronic immune-mediated liver disease [1,2]. A recent meta-analysis indicated that the pooled global incidence and prevalence of PBC were 1.76 and 14.60 per 100 000 persons, respectively, with clear regional and temporal differences [3]. Approximately 90 % of patients are female [4], mostly diagnosed between 50 and 60 years of age [1,5].The disease is histologically characterized by a non-suppurative destructive cholangitis of the small intrahepatic bile ducts [6].

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