Primary biliary cholangitis
What is Primary biliary cholangitis?
A rare autoimmune cholestatic liver disease characterized by autoimmune mediated damage of small intrahepatic bile ducts leading to cholestasis, fibrosis, and potential cirrhosis.
Other condition names
- Hanot syndrome
- PBC
- Primary biliary cirrhosis
Inheritance type
Multigenic/multifactorial, Unknown
Prevalence
- Worldwide: 1-5 in 10,000
- Europe: 1-5 in 10,000
- United States: 1-5 in 10,000
Age of Onset
- Adolescent
- Adult
- Elderly
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.