Primary biliary cholangitis
ORPHA:186· ICD-10 K74.3
Definition
A rare autoimmune cholestatic liver disease characterized by autoimmune mediated damage of small intrahepatic bile ducts leading to cholestasis, fibrosis, and potential cirrhosis.
- Prevalence
- 1-5 / 10 000
- Inheritance
- Multigenic/multifactorial, Unknown
- Age of onset
- Adolescent, Adult, Elderly