Primary biliary cholangitis

Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by lymphocytic destruction of the small, intrahepatic bile ducts, causing chronic cholestasis and progressive fibrosis that eventually leads to biliary cirrhosis. It is a complex disorder resulting from the interaction of genetic and environmental factors and is strongly predominant in female patients (female:male 9:1). Typical symptoms include cholestatic pruritus and the PBC fatigue symptom complex. Features of end-stage liver disease (ESLD) from PBC are the same as those of ESLD from other causes, i.e.