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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...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9337972/ https://www.ncbi.nlm.nih.gov/pubmed/35910038 http://dx.doi.org/10.1155/2022/7831165 |
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author | Mijic, Maja Saric, Ivona Delija, Bozena Lalovac, Milos Sobocan, Nikola Radetic, Eva Martincevic, Dora Filipec Kanizaj, Tajana |
author_facet | Mijic, Maja Saric, Ivona Delija, Bozena Lalovac, Milos Sobocan, Nikola Radetic, Eva Martincevic, Dora Filipec Kanizaj, Tajana |
author_sort | Mijic, Maja |
collection | PubMed |
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. |
format | Online Article Text |
id | pubmed-9337972 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Hindawi |
record_format | MEDLINE/PubMed |
spelling | pubmed-93379722022-07-30 Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients Mijic, Maja Saric, Ivona Delija, Bozena Lalovac, Milos Sobocan, Nikola Radetic, Eva Martincevic, Dora Filipec Kanizaj, Tajana Can J Gastroenterol Hepatol Review Article 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. Hindawi 2022-07-22 /pmc/articles/PMC9337972/ /pubmed/35910038 http://dx.doi.org/10.1155/2022/7831165 Text en Copyright © 2022 Maja Mijic et al. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Review Article Mijic, Maja Saric, Ivona Delija, Bozena Lalovac, Milos Sobocan, Nikola Radetic, Eva Martincevic, Dora Filipec Kanizaj, Tajana Pretransplant Evaluation and Liver Transplantation Outcome in PBC Patients |
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 |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9337972/ https://www.ncbi.nlm.nih.gov/pubmed/35910038 http://dx.doi.org/10.1155/2022/7831165 |
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