title: Primary biliary cirrhosis in the era of liver transplantation.
abstract: Primary biliary cirrhosis (PBC) is an autoimmune disease of the liver, characterized by the presence of antimitochondrial antibodies (AMA) and progressive immune-mediated destruction of biliary ductules, which lead to cirrhosis. Theories of the PBC etiopathogenesis assume that the disease develops secondarily as an improper immunological reaction to undefined environmental and/or infectious factors in genetically predisposed individuals. Ursodeoxycholic acid (UDCA) is the only drug recommended to treat PBC; it delays the progression of liver disease, but remains only a symptomatic treatment. In the advanced stage of PBC, the treatment of choice is liver transplantation (LTx). Nowadays, PBC is the third indication for LTx, after viral-related and alcoholic liver cirrhosis. Unfortunately, PBC recurs in 21-37% of patients at 10 years after LTx, and in 43% at 15 years after LTx, with the median time to recurrence of 3-5.5 years. Diagnosis of recurrent PBC (rPBC) is based on the liver histopathology. Although various risk factors of rPBC have been investigated, the cause of the recurrence is not clear. There is no specific treatment of rPBC. Together with immunosuppression after LTx, UDCA remains the treatment of choice. New diagnostic technologies (e.g., genomics, proteomics, cell-based therapy, and clinical study of the rPBC patients) may be helpful in understanding the pathogenesis of PBC and the development of new treatment modalities.
authors.affiliation: Department of Immunology, Transplantology and Internal Medicine, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Joanna
authors.initials: J
authors.lastname: Raczyńska
authors.affiliation: Department of Gastroenterology, Hepatology and Oncology, Medical Center for Postgraduate Education, Oncology Center, Warsaw, Poland.
authors.forename: Andrzej
authors.initials: A
authors.lastname: Habior
authors.affiliation: Department of Immunology, Transplantology and Internal Diseases, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Leszek
authors.initials: L
authors.lastname: Pączek
authors.affiliation: Department of Immunology, Transplantology and Internal Diseases, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Bartosz
authors.initials: B
authors.lastname: Foroncewicz
authors.affiliation: Department of Gastroenterology, Hepatology and Oncology, Medical Center for Postgraduate Education, Oncology Center, Warsaw, Poland.
authors.forename: Andrzej
authors.initials: A
authors.lastname: Pawełas
authors.affiliation: Department of Immunology, Transplantology and Internal Medicine, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Mucha
mesh_terms: D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D006801:Humans; D007165:Immunosuppression Therapy; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation; D012008:Recurrence; D012307:Risk Factors; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid