Orphanet: Primary sclerosing cholangitis

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Primary sclerosing cholangitis

Disease definition

Primary sclerosing cholangitis (PSC) is a rare, slowly progressive liver disease characterized by inflammation and destruction of the intra- and/or extra-hepatic bile ducts that lead to cholestasis, liver fibrosis, liver cirrhosis and ultimately liver failure.


Classification level: Disorder
  • Synonym(s):
    • PSC
  • Prevalence: 1-9 / 100 000
  • Inheritance: Multigenic/multifactorial 
  • Age of onset: Adult, Adolescent, Childhood, Elderly
  • ICD-10: K83.0
  • ICD-11: DB96.2
  • OMIM: 602114  613806
  • UMLS: C0566602
  • MeSH: C536419
  • GARD: 1280
  • MedDRA: 10036732

Detailed information


Disease review articles

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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