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Primary sclerosing cholangitis

ORPHA171
Synonym(s) PSC
Prevalence 1-9 / 100 000
Inheritance Multigenic/multifactorial
Age of onset Adult
ICD-10
  • K83.0
OMIM
UMLS
  • C0566602
MeSH
  • C536419
MedDRA
  • 10036732

Summary

Disease definition

Primary sclerosing cholangitis (PSC) is a rare, slowly progressive, genetic liver disease characterized by inflammation and destruction of the intra-hepatic and/or extra-hepatic bile ducts, leading to cholestasis, fibrosis, liver failure, and cirrhosis. The disorder is also associated with an increased risk of malignancy.

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