Primary biliary cholangitis
Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an organ-specific autoimmune disease predominantly affecting middle-aged women. It does not occur in children. PBC prevalence varies depending on the geographic location of the country. Over the past 30 years, there h...
| Main Authors: | , |
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| Format: | Article |
| Language: | Russian |
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Federal Research Center of Transplantology and Artificial Organs named after V.I.Shumakov
2021-04-01
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| Series: | Vestnik Transplantologii i Iskusstvennyh Organov |
| Subjects: | |
| Online Access: | https://journal.transpl.ru/vtio/article/view/1319 |
| _version_ | 1797871930718552064 |
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| author | I. M. Iljinsky O. M. Tsirulnikova |
| author_facet | I. M. Iljinsky O. M. Tsirulnikova |
| author_sort | I. M. Iljinsky |
| collection | DOAJ |
| description | Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an organ-specific autoimmune disease predominantly affecting middle-aged women. It does not occur in children. PBC prevalence varies depending on the geographic location of the country. Over the past 30 years, there has been an increased incidence of PBC, while significant progress has been made in understanding the pathogenesis of PBC due to the development of innovative technologies in molecular biology, immunology and genetics. The presence of antimitochondrial antibodies and cholestasis on biochemical analysis is sufficient to make a diagnosis, without the need for liver biopsy. Small- and medium-sized bile ducts are the targets of PBC. In the first stage of the disease, granulomatous destruction of the bile ducts occurs; in the second stage, loss of bile ducts, their proliferation, increased size of the portal tracts with chronic inflammation; in the third stage - fibrosis with septal formation, loss of bile ducts and cholestasis; in the fourth stage - liver cirrhosis. Previously, the survival rate of PBC patients ranged from 7.5 to 16 years. However, it has improved significantly with ursodeoxycholic acid and obeticholic acid treatment. If there is no effect from treatment and end-stage liver failure sets in, liver transplantation is performed. |
| first_indexed | 2024-04-10T00:51:56Z |
| format | Article |
| id | doaj.art-653ef24db91c46efac653a351a05520a |
| institution | Directory Open Access Journal |
| issn | 1995-1191 |
| language | Russian |
| last_indexed | 2024-04-10T00:51:56Z |
| publishDate | 2021-04-01 |
| publisher | Federal Research Center of Transplantology and Artificial Organs named after V.I.Shumakov |
| record_format | Article |
| series | Vestnik Transplantologii i Iskusstvennyh Organov |
| spelling | doaj.art-653ef24db91c46efac653a351a05520a2023-03-13T10:37:27ZrusFederal Research Center of Transplantology and Artificial Organs named after V.I.ShumakovVestnik Transplantologii i Iskusstvennyh Organov1995-11912021-04-0123116217010.15825/1995-1191-2021-1-162-170960Primary biliary cholangitisI. M. Iljinsky0O. M. Tsirulnikova1Shumakov National Medical Research Center of Transplantology and Artificial OrgansShumakov National Medical Research Center of Transplantology and Artificial Organs; Sechenov UniversityPrimary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is an organ-specific autoimmune disease predominantly affecting middle-aged women. It does not occur in children. PBC prevalence varies depending on the geographic location of the country. Over the past 30 years, there has been an increased incidence of PBC, while significant progress has been made in understanding the pathogenesis of PBC due to the development of innovative technologies in molecular biology, immunology and genetics. The presence of antimitochondrial antibodies and cholestasis on biochemical analysis is sufficient to make a diagnosis, without the need for liver biopsy. Small- and medium-sized bile ducts are the targets of PBC. In the first stage of the disease, granulomatous destruction of the bile ducts occurs; in the second stage, loss of bile ducts, their proliferation, increased size of the portal tracts with chronic inflammation; in the third stage - fibrosis with septal formation, loss of bile ducts and cholestasis; in the fourth stage - liver cirrhosis. Previously, the survival rate of PBC patients ranged from 7.5 to 16 years. However, it has improved significantly with ursodeoxycholic acid and obeticholic acid treatment. If there is no effect from treatment and end-stage liver failure sets in, liver transplantation is performed.https://journal.transpl.ru/vtio/article/view/1319primary biliary cholangitispbcpathogenesisrisk factorsursodeoxycholic acidobeticholic acid |
| spellingShingle | I. M. Iljinsky O. M. Tsirulnikova Primary biliary cholangitis Vestnik Transplantologii i Iskusstvennyh Organov primary biliary cholangitis pbc pathogenesis risk factors ursodeoxycholic acid obeticholic acid |
| title | Primary biliary cholangitis |
| title_full | Primary biliary cholangitis |
| title_fullStr | Primary biliary cholangitis |
| title_full_unstemmed | Primary biliary cholangitis |
| title_short | Primary biliary cholangitis |
| title_sort | primary biliary cholangitis |
| topic | primary biliary cholangitis pbc pathogenesis risk factors ursodeoxycholic acid obeticholic acid |
| url | https://journal.transpl.ru/vtio/article/view/1319 |
| work_keys_str_mv | AT imiljinsky primarybiliarycholangitis AT omtsirulnikova primarybiliarycholangitis |