Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Kasztelan-Szczerbinska, Beata, Rycyk-Bojarzynska, Anna, Szczerbinska, Agnieszka, Cichoz-Lach, Halina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
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
_version_ 1784887377955651584
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
work_keys_str_mv AT kasztelanszczerbinskabeata selectedaspectsoftheintricatebackgroundofimmunerelatedcholangiopathiesacriticaloverview
AT rycykbojarzynskaanna selectedaspectsoftheintricatebackgroundofimmunerelatedcholangiopathiesacriticaloverview
AT szczerbinskaagnieszka selectedaspectsoftheintricatebackgroundofimmunerelatedcholangiopathiesacriticaloverview
AT cichozlachhalina selectedaspectsoftheintricatebackgroundofimmunerelatedcholangiopathiesacriticaloverview