| H01467 | |
| H番号 | H01467 |
| 名称 | 原発性胆汁性胆管炎; 原発性胆汁性肝硬変 |
| 概要 | 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. |
| カテゴリ | 消化器系疾患 |
| ネットワーク | - |
| 病因遺伝子 | - |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | ウルソデオキシコール酸 [DR:D00734] |
| コメント | - |
| リンク | ICD-11: DB96.1 ICD-10: K74.3 MeSH: D008105 OMIM: 109720 |
| 文献 | PMID:28373632 著者 Tsuneyama K, Baba H, Morimoto Y, Tsunematsu T, Ogawa H タイトル Primary Biliary Cholangitis: Its Pathological Characteristics and Immunopathological Mechanisms. 雑誌 J Med Invest 64:7-13 (2017) DOI:10.2152/jmi.64.7 PMID:26250073 著者 Webb GJ, Siminovitch KA, Hirschfield GM タイトル The immunogenetics of primary biliary cirrhosis: A comprehensive review. 雑誌 J Autoimmun 64:42-52 (2015) DOI:10.1016/j.jaut.2015.07.004 PMID:19458352 著者 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 タイトル Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants. 雑誌 N Engl J Med 360:2544-55 (2009) DOI:10.1056/NEJMoa0810440 PMID:26364546 著者 Carey EJ, Ali AH, Lindor KD タイトル Primary biliary cirrhosis. 雑誌 Lancet 386:1565-75 (2015) DOI:10.1016/S0140-6736(15)00154-3 |