Management of primary sclerosing cholangitis and its complications: an algorithmic approach
Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease, characterized by multiple strictures and dilatations of the intra- and extrahepatic bile ducts, leading to progressive liver fibrosis, in 10–15% cholangiocarcinoma, and ultimately end-stage liver disease. The pathogenesis is p...
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| Veröffentlicht in: | Hepatology international 2021-02, Vol.15 (1), p.6-20 |
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| description | Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease, characterized by multiple strictures and dilatations of the intra- and extrahepatic bile ducts, leading to progressive liver fibrosis, in 10–15% cholangiocarcinoma, and ultimately end-stage liver disease. The pathogenesis is poorly understood, but (epi-)genetic factors, mechanisms of innate and adaptive immunity, toxic effects of hydrophobic bile acids, and possibly intestinal dysbiosis appear to be involved. The strong link with inflammatory bowel disease (IBD) is associated with a markedly enhanced risk of colorectal cancer which next to cholangiocarcinoma represents the most serious diagnostic challenge in long-term PSC management. Despite extensive research, no medical treatment has been proven so far to prolong the time to liver transplantation (LTx), which remains the effective treatment in late-stage disease. Recurrence of PSC after LTx is observed in up to 20% of patients. Here, we briefly summarize actual views on PSC pathogenesis and provide an algorithmic approach to diagnostic procedures and recommendations for the management of PSC and its complications. We describe promising treatment options subject to current clinical trials. |
| doi_str_mv | 10.1007/s12072-020-10118-x |
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The pathogenesis is poorly understood, but (epi-)genetic factors, mechanisms of innate and adaptive immunity, toxic effects of hydrophobic bile acids, and possibly intestinal dysbiosis appear to be involved. The strong link with inflammatory bowel disease (IBD) is associated with a markedly enhanced risk of colorectal cancer which next to cholangiocarcinoma represents the most serious diagnostic challenge in long-term PSC management. Despite extensive research, no medical treatment has been proven so far to prolong the time to liver transplantation (LTx), which remains the effective treatment in late-stage disease. Recurrence of PSC after LTx is observed in up to 20% of patients. Here, we briefly summarize actual views on PSC pathogenesis and provide an algorithmic approach to diagnostic procedures and recommendations for the management of PSC and its complications. We describe promising treatment options subject to current clinical trials.</description><identifier>ISSN: 1936-0533</identifier><identifier>EISSN: 1936-0541</identifier><identifier>DOI: 10.1007/s12072-020-10118-x</identifier><identifier>PMID: 33377990</identifier><language>eng</language><publisher>New Delhi: Springer India</publisher><subject>Adaptive immunity ; Algorithms ; Bile ; Bile acids ; Bile ducts ; Cholangiocarcinoma ; Cholangitis ; Clinical trials ; Colorectal cancer ; Colorectal carcinoma ; Colorectal Surgery ; Complications ; Diagnostic systems ; Dysbacteriosis ; Fibrosis ; Genetic factors ; Health risks ; Health services ; Hepatology ; Hydrophobicity ; Inflammatory bowel diseases ; Intestine ; Liver ; Liver diseases ; Liver transplantation ; Liver transplants ; Medical research ; Medical treatment ; Medicine ; Medicine & Public Health ; Pathogenesis ; Review ; Review Article ; Stricture ; Surgery ; Toxicity ; Transplantation</subject><ispartof>Hepatology international, 2021-02, Vol.15 (1), p.6-20</ispartof><rights>The Author(s) 2020</rights><rights>The Author(s) 2020. 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The pathogenesis is poorly understood, but (epi-)genetic factors, mechanisms of innate and adaptive immunity, toxic effects of hydrophobic bile acids, and possibly intestinal dysbiosis appear to be involved. The strong link with inflammatory bowel disease (IBD) is associated with a markedly enhanced risk of colorectal cancer which next to cholangiocarcinoma represents the most serious diagnostic challenge in long-term PSC management. Despite extensive research, no medical treatment has been proven so far to prolong the time to liver transplantation (LTx), which remains the effective treatment in late-stage disease. Recurrence of PSC after LTx is observed in up to 20% of patients. Here, we briefly summarize actual views on PSC pathogenesis and provide an algorithmic approach to diagnostic procedures and recommendations for the management of PSC and its complications. We describe promising treatment options subject to current clinical trials.</description><subject>Adaptive immunity</subject><subject>Algorithms</subject><subject>Bile</subject><subject>Bile acids</subject><subject>Bile ducts</subject><subject>Cholangiocarcinoma</subject><subject>Cholangitis</subject><subject>Clinical trials</subject><subject>Colorectal cancer</subject><subject>Colorectal carcinoma</subject><subject>Colorectal Surgery</subject><subject>Complications</subject><subject>Diagnostic systems</subject><subject>Dysbacteriosis</subject><subject>Fibrosis</subject><subject>Genetic factors</subject><subject>Health risks</subject><subject>Health services</subject><subject>Hepatology</subject><subject>Hydrophobicity</subject><subject>Inflammatory bowel diseases</subject><subject>Intestine</subject><subject>Liver</subject><subject>Liver diseases</subject><subject>Liver transplantation</subject><subject>Liver transplants</subject><subject>Medical research</subject><subject>Medical treatment</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Pathogenesis</subject><subject>Review</subject><subject>Review Article</subject><subject>Stricture</subject><subject>Surgery</subject><subject>Toxicity</subject><subject>Transplantation</subject><issn>1936-0533</issn><issn>1936-0541</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>C6C</sourceid><recordid>eNp9kT9v2zAQxYkiQey4_QIdCgKZ1fKPRIkdAgRG0hRwkKWZMhAnipJpSKRKyoHz7cPUjtsumUjwfvfuHh9Cnyn5Sgkpv0XKSMkywkhGCaVVtvuA5lRykZEipyfHO-czdB7jhpCiEFScoRnnvCylJHP0eAcOOjMYN2Hf4jHYAcIzjro3wUfrOqzXvgfX2clGDK7BdopY-2HsrYbJehe_p2cMfeeDndaD1RjGMXjQ64_otIU-mk-Hc4Eebq5_LW-z1f2Pn8urVabzMp8y2tayaRtuuC6JrExFgQHTwjSsqQVvoTENryFPNKuZLmvZ1kVVQCUkJNuSL9DlXnfc1oNpdPISoFcHL8qDVf9XnF2rzj-psqpExWkSuDgIBP97a-KkNn4bXNpZsVwSRiUTIlFsT-n0MzGY9jiBEvUaiNoHolIg6k8gapeavvy727HlLYEE8D0QU8l1Jvyd_Y7sCxrpmmc</recordid><startdate>20210201</startdate><enddate>20210201</enddate><creator>Prokopič, Michal</creator><creator>Beuers, Ulrich</creator><general>Springer India</general><general>Springer Nature B.V</general><scope>C6C</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7U7</scope><scope>C1K</scope><scope>K9.</scope><scope>NAPCQ</scope><scope>5PM</scope><orcidid>https://orcid.org/0000-0001-5114-7799</orcidid></search><sort><creationdate>20210201</creationdate><title>Management of primary sclerosing cholangitis and its complications: an algorithmic approach</title><author>Prokopič, Michal ; Beuers, Ulrich</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c474t-1fb9dfd3e3c7098e81a2a2c6ed2db63faded3ba4c472b2c7b9fb585a869a01193</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2021</creationdate><topic>Adaptive immunity</topic><topic>Algorithms</topic><topic>Bile</topic><topic>Bile acids</topic><topic>Bile ducts</topic><topic>Cholangiocarcinoma</topic><topic>Cholangitis</topic><topic>Clinical trials</topic><topic>Colorectal cancer</topic><topic>Colorectal carcinoma</topic><topic>Colorectal Surgery</topic><topic>Complications</topic><topic>Diagnostic systems</topic><topic>Dysbacteriosis</topic><topic>Fibrosis</topic><topic>Genetic factors</topic><topic>Health risks</topic><topic>Health services</topic><topic>Hepatology</topic><topic>Hydrophobicity</topic><topic>Inflammatory bowel diseases</topic><topic>Intestine</topic><topic>Liver</topic><topic>Liver diseases</topic><topic>Liver transplantation</topic><topic>Liver transplants</topic><topic>Medical research</topic><topic>Medical treatment</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Pathogenesis</topic><topic>Review</topic><topic>Review Article</topic><topic>Stricture</topic><topic>Surgery</topic><topic>Toxicity</topic><topic>Transplantation</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Prokopič, Michal</creatorcontrib><creatorcontrib>Beuers, Ulrich</creatorcontrib><collection>Springer Nature OA Free Journals</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Toxicology Abstracts</collection><collection>Environmental Sciences and Pollution Management</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Premium</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Hepatology international</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Prokopič, Michal</au><au>Beuers, Ulrich</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Management of primary sclerosing cholangitis and its complications: an algorithmic approach</atitle><jtitle>Hepatology international</jtitle><stitle>Hepatol Int</stitle><addtitle>Hepatol Int</addtitle><date>2021-02-01</date><risdate>2021</risdate><volume>15</volume><issue>1</issue><spage>6</spage><epage>20</epage><pages>6-20</pages><issn>1936-0533</issn><eissn>1936-0541</eissn><abstract>Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease, characterized by multiple strictures and dilatations of the intra- and extrahepatic bile ducts, leading to progressive liver fibrosis, in 10–15% cholangiocarcinoma, and ultimately end-stage liver disease. The pathogenesis is poorly understood, but (epi-)genetic factors, mechanisms of innate and adaptive immunity, toxic effects of hydrophobic bile acids, and possibly intestinal dysbiosis appear to be involved. The strong link with inflammatory bowel disease (IBD) is associated with a markedly enhanced risk of colorectal cancer which next to cholangiocarcinoma represents the most serious diagnostic challenge in long-term PSC management. Despite extensive research, no medical treatment has been proven so far to prolong the time to liver transplantation (LTx), which remains the effective treatment in late-stage disease. Recurrence of PSC after LTx is observed in up to 20% of patients. Here, we briefly summarize actual views on PSC pathogenesis and provide an algorithmic approach to diagnostic procedures and recommendations for the management of PSC and its complications. 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| subjects | Adaptive immunity Algorithms Bile Bile acids Bile ducts Cholangiocarcinoma Cholangitis Clinical trials Colorectal cancer Colorectal carcinoma Colorectal Surgery Complications Diagnostic systems Dysbacteriosis Fibrosis Genetic factors Health risks Health services Hepatology Hydrophobicity Inflammatory bowel diseases Intestine Liver Liver diseases Liver transplantation Liver transplants Medical research Medical treatment Medicine Medicine & Public Health Pathogenesis Review Review Article Stricture Surgery Toxicity Transplantation |
| title | Management of primary sclerosing cholangitis and its complications: an algorithmic approach |
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