Primary biliary cholangitis

Autoimmune disease of small intrahepatic bile ducts with progressive cholestasis and fibrosis. Ursodeoxycholic acid is the cornerstone of disease-modifying therapy.

ICD-10 K74.31 drug

Also known as: PBC, Primary biliary cirrhosis

Drugs used for this condition

AUrsodeoxycholic acidFirst line · Bile acid preparationsFirst-line disease-modifying therapy for primary biliary cholangitis per EASL 2017 and AASLD 2018. Dose 13–15 mg/kg/day in 1–4 divided doses. Lowers alkaline phosphatase and bilirubin, slows fibrosis progression, and improves transplant-free survival. Biochemical response is assessed at 12 months. Non-responders may receive obeticholic acid or fibrates per EASL.

How to read tiers A–F

Evidence tiers belong to the drug-indication pair, not to the drug as a whole: the same substance can be A for one condition and F for another.

AStrong evidence – first or second line in international guidelines
BRecommended in selected cases
CStudy data contradict each other
DBenefit not proven, no documented harm
FDocumented harm, or risk without benefit