KEGG DISEASE: Primary biliary cholangitis

KEGG

DISEASE: Primary biliary cholangitis

Entry

H01467                      Disease

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

Brite

Human diseases in ICD-11 classification [BR:br08403]
13 Diseases of the digestive system
  Diseases of liver
   DB96  Autoimmune liver disease
    H01467  Primary biliary cholangitis

Drug

Ursodiol [DR:D00734]
Obeticholic acid [DR:D09360]
Elafibranor [DR:D11208]
Seladelpar lysine [DR:D11257]

Other DBs

ICD-11:

MeSH:

OMIM:

Reference

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

Reference

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

Reference

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

Reference

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

LinkDB

» Japanese version

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