Current understanding of primary biliary cholangitis
Primary biliary cholangitis (PBC) causes chronic and persistent cholestasis in the liver, eventually resulting in cirrhosis and hepatic failure without appropriate treatment. PBC mainly develops in middle-aged women, but it is also common in young women and men. PBC is considered a model of autoimmu...
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| Format: | Article |
| Language: | English |
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Korean Association for the Study of the Liver
2021-01-01
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| Series: | Clinical and Molecular Hepatology |
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| Online Access: | http://e-cmh.org/upload/pdf/cmh-2020-0028.pdf |
| _version_ | 1818413252559765504 |
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| author | Atsushi Tanaka |
| author_facet | Atsushi Tanaka |
| author_sort | Atsushi Tanaka |
| collection | DOAJ |
| description | Primary biliary cholangitis (PBC) causes chronic and persistent cholestasis in the liver, eventually resulting in cirrhosis and hepatic failure without appropriate treatment. PBC mainly develops in middle-aged women, but it is also common in young women and men. PBC is considered a model of autoimmune disease because of the presence of disease-specific autoantibodies, that is, antimitochondrial antibodies (AMAs), intense infiltration of mononuclear cells into the bile ducts, and a high prevalence of autoimmune diseases such as comorbidities. Histologically, PBC is characterized by degeneration and necrosis of intrahepatic biliary epithelial cells surrounded by a dense infiltration of mononuclear cells, coined as chronic non-suppurative destructive cholangitis, which leads to destructive changes and the disappearance of small- or medium-sized bile ducts. Since 1990, early diagnosis with the detection of AMAs and introduction of ursodeoxycholic acid as first-line treatment has greatly altered the clinical course of PBC, and liver transplantation-free survival of patients with PBC is now comparable to that of the general population. |
| first_indexed | 2024-12-14T11:00:15Z |
| format | Article |
| id | doaj.art-0c3634d8a0364944a5332a330d57171e |
| institution | Directory Open Access Journal |
| issn | 2287-2728 2287-285X |
| language | English |
| last_indexed | 2024-12-14T11:00:15Z |
| publishDate | 2021-01-01 |
| publisher | Korean Association for the Study of the Liver |
| record_format | Article |
| series | Clinical and Molecular Hepatology |
| spelling | doaj.art-0c3634d8a0364944a5332a330d57171e2022-12-21T23:04:44ZengKorean Association for the Study of the LiverClinical and Molecular Hepatology2287-27282287-285X2021-01-0127112110.3350/cmh.2020.00281566Current understanding of primary biliary cholangitisAtsushi Tanaka0 Department of Medicine, Teikyo University School of Medicine, Tokyo, JapanPrimary biliary cholangitis (PBC) causes chronic and persistent cholestasis in the liver, eventually resulting in cirrhosis and hepatic failure without appropriate treatment. PBC mainly develops in middle-aged women, but it is also common in young women and men. PBC is considered a model of autoimmune disease because of the presence of disease-specific autoantibodies, that is, antimitochondrial antibodies (AMAs), intense infiltration of mononuclear cells into the bile ducts, and a high prevalence of autoimmune diseases such as comorbidities. Histologically, PBC is characterized by degeneration and necrosis of intrahepatic biliary epithelial cells surrounded by a dense infiltration of mononuclear cells, coined as chronic non-suppurative destructive cholangitis, which leads to destructive changes and the disappearance of small- or medium-sized bile ducts. Since 1990, early diagnosis with the detection of AMAs and introduction of ursodeoxycholic acid as first-line treatment has greatly altered the clinical course of PBC, and liver transplantation-free survival of patients with PBC is now comparable to that of the general population.http://e-cmh.org/upload/pdf/cmh-2020-0028.pdfanti-mitochondrial antibodyepidemiologybezafibrate |
| spellingShingle | Atsushi Tanaka Current understanding of primary biliary cholangitis Clinical and Molecular Hepatology anti-mitochondrial antibody epidemiology bezafibrate |
| title | Current understanding of primary biliary cholangitis |
| title_full | Current understanding of primary biliary cholangitis |
| title_fullStr | Current understanding of primary biliary cholangitis |
| title_full_unstemmed | Current understanding of primary biliary cholangitis |
| title_short | Current understanding of primary biliary cholangitis |
| title_sort | current understanding of primary biliary cholangitis |
| topic | anti-mitochondrial antibody epidemiology bezafibrate |
| url | http://e-cmh.org/upload/pdf/cmh-2020-0028.pdf |
| work_keys_str_mv | AT atsushitanaka currentunderstandingofprimarybiliarycholangitis |