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Abstract Details
Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis
Samantha Sarcognato1, Diana Sacchi1, Federica Grillo2, Nora Cazzagon3, Luca Fabris4, Massimiliano Cadamuro4, Ivana Cataldo1, Claudia Covelli5, Alessandra Mangia6, Maria Guido17
Author information
1Department of Pathology, Azienda ULSS2 Marca Trevigiana, Treviso, Italy.
2Anatomic Pathology Unit, University of Genova and Policlinico San Martino Hospital, Genova, Italy.
3Department of Surgery, Oncology and Gastroenterology - DISCOG, University of Padova, Padova, Italy.
4Department of Molecular Medicine - DMM, University of Padova, Padova, Italy.
5Pathology Unit, Fondazione IRCCS "Casa Sollievo della Sofferenza", San Giovanni Rotondo, Italy.
6Liver Unit, Fondazione IRCCS "Casa Sollievo della Sofferenza", San Giovanni Rotondo, Italy.
7Department of Medicine - DIMED, University of Padova, Padova, Italy.
Abstract
Autoimmune cholestatic liver diseases are rare hepato-biliary disorders characterized by a progressive, inflammatory destruction of bile ducts. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the main autoimmune cholestatic liver diseases. Both may evolve into secondary biliary cirrhosis and its complications. Therapeutic options are limited and liver transplantation remains the only definitive treatment for PBC and PSC.
Most PBC and PSC patients have a typical presentation, which does not require liver biopsy. However, in routine clinical practice, important variants or specific subgroups that benefit from liver biopsy for proper management may be observed. Herein, we provide a general overview of clinical and pathological characteristic of PBC and PSC, highlighting the most important features for routine diagnostic practice.