Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis
Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are immune-mediated chronic liver diseases. PSC is a rare disorder characterized by multi-focal bile duct strictures and progressive liver diseases that ultimately results in the need for liver transplantation in most patient...
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| Format: | Article |
| Language: | English |
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Jin Publishing & Printing Co.
2020-05-01
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| Series: | The Korean Journal of Gastroenterology |
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| Online Access: | http://www.kjg.or.kr/journal/view.html?uid=5575&vmd=Full |
| _version_ | 1819044384517128192 |
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| author | Dong-Won Ahn |
| author_facet | Dong-Won Ahn |
| author_sort | Dong-Won Ahn |
| collection | DOAJ |
| description | Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are immune-mediated chronic liver diseases. PSC is a rare disorder characterized by multi-focal bile duct strictures and progressive liver diseases that ultimately results in the need for liver transplantation in most patients. Imaging studies, such as MRCP, have an essential role in the diagnosis of most cases of PSC. PSC is usually accompanied by inflammatory bowel disease, and there is a high risk of cholangiocarcinoma and colorectal cancer in PSC. No medical therapies have been proven to delay the progression of PSC. Endoscopic intervention for tissue diagnosis or biliary drainage is frequently required in cases of PSC with a dominant stricture, acute cholangitis, or clinically suspected cholangiocarcinoma. PBC is a chronic inflammatory autoimmune cholestatic liver disease, which, when untreated, will culminate in end-stage biliary cirrhosis requiring liver transplantation. A diagnosis is usually based on the presence of serum liver tests indicative of cholestatic hepatitis in association with circulating antimitochondrial antibodies. Patient presentation and course can be diverse in PBC, and risk stratification is important for ensuring that all patients receive a personalized approach to their care. Medical therapy using ursodeoxycholic acid or obeticholic acid has an important role in reducing the progression to end-stage liver disease in PBC. |
| first_indexed | 2024-12-21T10:11:49Z |
| format | Article |
| id | doaj.art-93308a2a220647eeb1f9039af7d00937 |
| institution | Directory Open Access Journal |
| issn | 1598-9992 2233-6869 |
| language | English |
| last_indexed | 2024-12-21T10:11:49Z |
| publishDate | 2020-05-01 |
| publisher | Jin Publishing & Printing Co. |
| record_format | Article |
| series | The Korean Journal of Gastroenterology |
| spelling | doaj.art-93308a2a220647eeb1f9039af7d009372022-12-21T19:07:41ZengJin Publishing & Printing Co.The Korean Journal of Gastroenterology1598-99922233-68692020-05-0175524625610.4166/kjg.2020.75.5.246Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary CholangitisDong-Won Ahn0https://orcid.org/0000-0002-6641-2177Department of Internal Medicine, Seoul National University Boramae Medical Center, Seoul National University College of Medicine, Seoul, KoreaPrimary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are immune-mediated chronic liver diseases. PSC is a rare disorder characterized by multi-focal bile duct strictures and progressive liver diseases that ultimately results in the need for liver transplantation in most patients. Imaging studies, such as MRCP, have an essential role in the diagnosis of most cases of PSC. PSC is usually accompanied by inflammatory bowel disease, and there is a high risk of cholangiocarcinoma and colorectal cancer in PSC. No medical therapies have been proven to delay the progression of PSC. Endoscopic intervention for tissue diagnosis or biliary drainage is frequently required in cases of PSC with a dominant stricture, acute cholangitis, or clinically suspected cholangiocarcinoma. PBC is a chronic inflammatory autoimmune cholestatic liver disease, which, when untreated, will culminate in end-stage biliary cirrhosis requiring liver transplantation. A diagnosis is usually based on the presence of serum liver tests indicative of cholestatic hepatitis in association with circulating antimitochondrial antibodies. Patient presentation and course can be diverse in PBC, and risk stratification is important for ensuring that all patients receive a personalized approach to their care. Medical therapy using ursodeoxycholic acid or obeticholic acid has an important role in reducing the progression to end-stage liver disease in PBC.http://www.kjg.or.kr/journal/view.html?uid=5575&vmd=Fullprimary sclerosing cholangitisprimary biliary cholangitischolangiocarcinomaursodeoxycholic acidliver transplantation |
| spellingShingle | Dong-Won Ahn Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis The Korean Journal of Gastroenterology primary sclerosing cholangitis primary biliary cholangitis cholangiocarcinoma ursodeoxycholic acid liver transplantation |
| title | Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis |
| title_full | Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis |
| title_fullStr | Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis |
| title_full_unstemmed | Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis |
| title_short | Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis |
| title_sort | novel insights of primary sclerosing cholangitis and primary biliary cholangitis |
| topic | primary sclerosing cholangitis primary biliary cholangitis cholangiocarcinoma ursodeoxycholic acid liver transplantation |
| url | http://www.kjg.or.kr/journal/view.html?uid=5575&vmd=Full |
| work_keys_str_mv | AT dongwonahn novelinsightsofprimarysclerosingcholangitisandprimarybiliarycholangitis |