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Primary biliary cholangitis

Disease definition

Primary biliary cholangitis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure.

ORPHA:186

  • Synonym(s):
    • Hanot syndrome
    • PBC
    • Primary biliary cirrhosis
  • Prevalence: 1-5 / 10 000
  • Inheritance: Unknown 
  • Age of onset: Adolescent, Adult, Elderly
  • ICD-10: K74.3
  • OMIM: 109720  613007  613008  614220  614221
  • UMLS: C0008312  C0859942
  • MeSH: -
  • GARD: 7459
  • MedDRA: 10004661  10019137

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