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Selected Aspects of the Intricate Background of Immune-Related Cholangiopathies—A Critical Overview
Primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are rare immune-related cholangiopathies with still poorly explained pathogenesis. Although triggers of chronic inflammation with subsequent fibrosis that affect cholangiocytes leading to obliteration of bile ducts and conve...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9921714/ https://www.ncbi.nlm.nih.gov/pubmed/36771465 http://dx.doi.org/10.3390/nu15030760 |
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author | Kasztelan-Szczerbinska, Beata Rycyk-Bojarzynska, Anna Szczerbinska, Agnieszka Cichoz-Lach, Halina |
author_facet | Kasztelan-Szczerbinska, Beata Rycyk-Bojarzynska, Anna Szczerbinska, Agnieszka Cichoz-Lach, Halina |
author_sort | Kasztelan-Szczerbinska, Beata |
collection | PubMed |
description | Primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are rare immune-related cholangiopathies with still poorly explained pathogenesis. Although triggers of chronic inflammation with subsequent fibrosis that affect cholangiocytes leading to obliteration of bile ducts and conversion to liver cirrhosis are unclear, both disorders are regarded to be multifactorial. Different factors can contribute to the development of hepatocellular injury in the course of progressive cholestasis, including (1) body accumulation of bile acids and their toxicity, (2) decreased food intake and nutrient absorption, (3) gut microbiota transformation, and (4) reorganized host metabolism. Growing evidence suggests that intestinal microbiome composition not only can be altered by liver dysfunction, but in turn, it actively impacts hepatic conditions. In this review, we highlight the role of key factors such as the gut–liver axis, intestinal barrier integrity, bile acid synthesis and circulation, and microbiome composition, which seem to be strongly related to PBC and PSC outcome. Emerging treatments and future therapeutic strategies are also presented. |
format | Online Article Text |
id | pubmed-9921714 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-99217142023-02-12 Selected Aspects of the Intricate Background of Immune-Related Cholangiopathies—A Critical Overview Kasztelan-Szczerbinska, Beata Rycyk-Bojarzynska, Anna Szczerbinska, Agnieszka Cichoz-Lach, Halina Nutrients Review Primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are rare immune-related cholangiopathies with still poorly explained pathogenesis. Although triggers of chronic inflammation with subsequent fibrosis that affect cholangiocytes leading to obliteration of bile ducts and conversion to liver cirrhosis are unclear, both disorders are regarded to be multifactorial. Different factors can contribute to the development of hepatocellular injury in the course of progressive cholestasis, including (1) body accumulation of bile acids and their toxicity, (2) decreased food intake and nutrient absorption, (3) gut microbiota transformation, and (4) reorganized host metabolism. Growing evidence suggests that intestinal microbiome composition not only can be altered by liver dysfunction, but in turn, it actively impacts hepatic conditions. In this review, we highlight the role of key factors such as the gut–liver axis, intestinal barrier integrity, bile acid synthesis and circulation, and microbiome composition, which seem to be strongly related to PBC and PSC outcome. Emerging treatments and future therapeutic strategies are also presented. MDPI 2023-02-02 /pmc/articles/PMC9921714/ /pubmed/36771465 http://dx.doi.org/10.3390/nu15030760 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Kasztelan-Szczerbinska, Beata Rycyk-Bojarzynska, Anna Szczerbinska, Agnieszka Cichoz-Lach, Halina Selected Aspects of the Intricate Background of Immune-Related Cholangiopathies—A Critical Overview |
title | Selected Aspects of the Intricate Background of Immune-Related Cholangiopathies—A Critical Overview |
title_full | Selected Aspects of the Intricate Background of Immune-Related Cholangiopathies—A Critical Overview |
title_fullStr | Selected Aspects of the Intricate Background of Immune-Related Cholangiopathies—A Critical Overview |
title_full_unstemmed | Selected Aspects of the Intricate Background of Immune-Related Cholangiopathies—A Critical Overview |
title_short | Selected Aspects of the Intricate Background of Immune-Related Cholangiopathies—A Critical Overview |
title_sort | selected aspects of the intricate background of immune-related cholangiopathies—a critical overview |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9921714/ https://www.ncbi.nlm.nih.gov/pubmed/36771465 http://dx.doi.org/10.3390/nu15030760 |
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