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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.

ORPHA:186

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

Professionals

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.