Orphanet: Primary biliary cholangitis

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Primary biliary cholangitis

Disease definition

A rare autoimmune cholestatic liver disease characterized by autoimmune mediated damage of small intrahepatic bile ducts leading to cholestasis, fibrosis, and potential cirrhosis.


Classification level: Disorder
  • Synonym(s):
    • Hanot syndrome
    • PBC
    • Primary biliary cirrhosis
  • Prevalence: 1-5 / 10 000
  • Inheritance: Multigenic/multifactorial or 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

Detailed information


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