Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients

Primary biliary cholangitis (PBC) is an autoimmune chronic cholestatic liver disease characterized by progressive cholangiocyte and bile duct destruction leading to fibrosis and finally to liver cirrhosis. The presence of disease-specific serological antimitochondrial antibody (AMA) together with el...

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Main Authors: Maja Mijic, Ivona Saric, Bozena Delija, Milos Lalovac, Nikola Sobocan, Eva Radetic, Dora Martincevic, Tajana Filipec Kanizaj
Format: Article
Language:English
Published: Wiley 2022-01-01
Series:Canadian Journal of Gastroenterology and Hepatology
Online Access:http://dx.doi.org/10.1155/2022/7831165
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author Maja Mijic
Ivona Saric
Bozena Delija
Milos Lalovac
Nikola Sobocan
Eva Radetic
Dora Martincevic
Tajana Filipec Kanizaj
author_facet Maja Mijic
Ivona Saric
Bozena Delija
Milos Lalovac
Nikola Sobocan
Eva Radetic
Dora Martincevic
Tajana Filipec Kanizaj
author_sort Maja Mijic
collection DOAJ
description Primary biliary cholangitis (PBC) is an autoimmune chronic cholestatic liver disease characterized by progressive cholangiocyte and bile duct destruction leading to fibrosis and finally to liver cirrhosis. The presence of disease-specific serological antimitochondrial antibody (AMA) together with elevated alkaline phosphatase (ALP) as a biomarker of cholestasis is sufficient for diagnosis. Ursodeoxycholic acid (UDCA) is the first treatment option for PBC. Up to 40% of patients have an incomplete response to therapy, and over time disease progresses to liver cirrhosis. Several risk scores are proposed for better evaluation of patients before and during treatment to stratify patients at increased risk of disease progression. GLOBE score and UK PBC risk score are used for the evaluation of UDCA treatment and Mayo risk score for transplant-free survival. Liver transplantation (LT) is the only treatment option for end-stage liver disease. More than 10 years after LT, 40% of patients experience recurrence of the disease. A liver biopsy is required to establish rPBC (recurrent primary biliary cholangitis). The only treatment option for rPBC is UDCA, and data show biochemical and clinical improvement, plus potential beneficial effects for use after transplantation for the prevention of rPBC development. Additional studies are required to assess the full impact of rPBC on graft and recipient survival and for treatment options for rPBC.
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spelling doaj-art-2fc13a340a1a41aaab2c606a73a1c2622025-02-03T05:49:23ZengWileyCanadian Journal of Gastroenterology and Hepatology2291-27972022-01-01202210.1155/2022/7831165Pretransplant Evaluation and Liver Transplantation Outcome in PBC PatientsMaja Mijic0Ivona Saric1Bozena Delija2Milos Lalovac3Nikola Sobocan4Eva Radetic5Dora Martincevic6Tajana Filipec Kanizaj7Department of GastroenterologyDepartment of GastroenterologyDepartment of GastroenterologyDepartment of GastroenterologyDepartment of GastroenterologyUniversity of ZagrebUniversity of ZagrebDepartment of GastroenterologyPrimary biliary cholangitis (PBC) is an autoimmune chronic cholestatic liver disease characterized by progressive cholangiocyte and bile duct destruction leading to fibrosis and finally to liver cirrhosis. The presence of disease-specific serological antimitochondrial antibody (AMA) together with elevated alkaline phosphatase (ALP) as a biomarker of cholestasis is sufficient for diagnosis. Ursodeoxycholic acid (UDCA) is the first treatment option for PBC. Up to 40% of patients have an incomplete response to therapy, and over time disease progresses to liver cirrhosis. Several risk scores are proposed for better evaluation of patients before and during treatment to stratify patients at increased risk of disease progression. GLOBE score and UK PBC risk score are used for the evaluation of UDCA treatment and Mayo risk score for transplant-free survival. Liver transplantation (LT) is the only treatment option for end-stage liver disease. More than 10 years after LT, 40% of patients experience recurrence of the disease. A liver biopsy is required to establish rPBC (recurrent primary biliary cholangitis). The only treatment option for rPBC is UDCA, and data show biochemical and clinical improvement, plus potential beneficial effects for use after transplantation for the prevention of rPBC development. Additional studies are required to assess the full impact of rPBC on graft and recipient survival and for treatment options for rPBC.http://dx.doi.org/10.1155/2022/7831165
spellingShingle Maja Mijic
Ivona Saric
Bozena Delija
Milos Lalovac
Nikola Sobocan
Eva Radetic
Dora Martincevic
Tajana Filipec Kanizaj
Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients
Canadian Journal of Gastroenterology and Hepatology
title Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients
title_full Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients
title_fullStr Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients
title_full_unstemmed Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients
title_short Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients
title_sort pretransplant evaluation and liver transplantation outcome in pbc patients
url http://dx.doi.org/10.1155/2022/7831165
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