KEGG   DISEASE: 原発性胆汁性胆管炎
エントリ  
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
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 13 消化器系の疾患
  肝疾患
   DB96  自己免疫性肝疾患
    H01467  原発性胆汁性胆管炎
指定難病 [jp08407.html]
 H01467
治療薬   
ウルソデオキシコール酸 [DR:D00734]
リンク   
ICD-11: DB96.1
ICD-10: K74.3
MeSH: D008105
OMIM: 109720
文献    
  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
文献    
  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
文献    
  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
文献    
  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
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