Overview
Primary biliary cholangitis (formerly primary biliary cirrhosis) is a chronic, progressive, autoimmune, cholestatic liver disease more common in middle-aged women. It is characterized by destruction of small to medium bile ducts leading to cholestasis and frequently, end-stage liver disease.
Diagnostic features are chronic biochemical cholestasis, presence of antimitochondrial antibodies and the characteristic liver biopsy findings.
Patients with abnormal liver biochemistry (regardless of histologic stage) and confirmed primary biliary cholangitis should be given ursodeoxycholic acid (UDCA). It is the chief medication used to deter disease progression. While obeticholic acid (OCA) may be used as monotherapy in patients intolerant to UDCA or in
combination with UDCA in patients with inadequate response to a year of
UDCA therapy.
For further information regarding the management of Primary Biliary Cholangitis, please refer to Disease Algorithm for the Treatment Guideline.
