| H01467 | |
| H number | H01467 |
| Name | Primary biliary cholangitis; Primary biliary cirrhosis |
| Description | Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic, progressive cholestatic liver disease characterised by destruction of small intrahepatic bile ducts, eventually leading to fibrosis and potential cirrhosis. Patients are usually middle-aged women and presents in the fifth or sixth decade of life. The most common symptoms are pruritus. It is not clear what causes PBC. It may be relate to problems in the autoimmune mechanism. The increased risk of genetic predisposition and environmental factors (infectious or toxic agents) trigger the disease. Ursodeoxycholic acid is currently the only medicine approved for the treatment of the disease. |
| Category | Digestive system disease |
| Network | - |
| Gene | - |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | Ursodiol [DR:D00734] Obeticholic acid [DR:D09360] |
| Comment | - |
| Other DBs | ICD-11: DB96.1 ICD-10: K74.3 MeSH: D008105 OMIM: 109720 |
| Reference | PMID:28373632 AUTHORS Tsuneyama K, Baba H, Morimoto Y, Tsunematsu T, Ogawa H TITLE Primary Biliary Cholangitis: Its Pathological Characteristics and Immunopathological Mechanisms. JOURNAL J Med Invest 64:7-13 (2017) DOI:10.2152/jmi.64.7 PMID:26250073 AUTHORS Webb GJ, Siminovitch KA, Hirschfield GM TITLE The immunogenetics of primary biliary cirrhosis: A comprehensive review. JOURNAL J Autoimmun 64:42-52 (2015) DOI:10.1016/j.jaut.2015.07.004 PMID:19458352 AUTHORS Hirschfield GM, Liu X, Xu C, Lu Y, Xie G, Lu Y, Gu X, Walker EJ, Jing K, Juran BD, Mason AL, Myers RP, Peltekian KM, Ghent CN, Coltescu C, Atkinson EJ, Heathcote EJ, Lazaridis KN, Amos CI, Siminovitch KA TITLE Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants. JOURNAL N Engl J Med 360:2544-55 (2009) DOI:10.1056/NEJMoa0810440 PMID:26364546 AUTHORS Carey EJ, Ali AH, Lindor KD TITLE Primary biliary cirrhosis. JOURNAL Lancet 386:1565-75 (2015) DOI:10.1016/S0140-6736(15)00154-3 |