29 September 2014 : Review article
Ann Transplant 2014; 19:488-493
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.
Keywords: Genome-Wide Association Study, Immunosuppression, Liver Cirrhosis, Biliary, Liver Transplantation, Recurrence
10 Nov 2023 : Original articleEffects of Preservation of Donor Liver Gastroduodenal Artery on Post-Transplant Biliary Complications in 18...
Ann Transplant In Press; DOI: 10.12659/AOT.941699
07 Nov 2023 : Original articleAllogeneic Hematopoietic Stem Cell Transplantation Can Improve Prognosis of Extramedullary Infiltration Pos...
Ann Transplant In Press; DOI: 10.12659/AOT.942197
06 Nov 2023 : Original articleClinical Outcomes of Administration of Rituximab for Desensitization in Liver Transplant Patients with Pref...
Ann Transplant In Press; DOI: 10.12659/AOT.941456
Most Viewed Current Articles
24 Aug 2021 : Review articleNormothermic Machine Perfusion (NMP) of the Liver – Current Status and Future Perspectives
Ann Transplant 2021; 26:e931664
26 Jan 2022 : Review articleRecurrence of Hepatocellular Carcinoma After Liver Transplantation: Risk Factors and Predictive Models
Ann Transplant 2022; 27:e934924
29 Dec 2021 : Original articleEfficacy and Safety of Tacrolimus-Based Maintenance Regimens in De Novo Kidney Transplant Recipients: A Sys...
Ann Transplant 2021; 26:e933588