Synopsis
Confirm primary biliary cholangitis, start weight-based ursodeoxycholic acid, identify inadequate response and manage cholestatic symptoms and cirrhosis systematically.
- PBC is a chronic autoimmune destruction of small intrahepatic bile ducts that causes cholestasis, progressive fibrosis and eventually portal hypertension in some patients.
- A persistent alkaline-phosphatase-predominant pattern plus antimitochondrial antibodies usually establishes the diagnosis after extrahepatic obstruction has been excluded.
- PBC-specific antinuclear antibodies, particularly anti-gp210 or anti-sp100 where available, support diagnosis when antimitochondrial antibodies are absent.
Key red flags
Splenomegaly, falling platelets, varices, ascites, jaundice or muscle loss indicates advanced fibrosis or portal hypertension and changes surveillance and transplant planning.
Investigation priorities
Confirm persistent cholestasis and establish severity or treatment response.
Management branches
Alkaline phosphatase remains elevated without an obvious transient explanation.
- Repeat and fractionate the clinical problem through medicine, alcohol, pregnancy and symptom review, and assess bilirubin, albumin, INR and platelets.
- Perform ultrasound to exclude dilated ducts, stones or mass and request AMA with immunoglobulins and PBC-specific ANA when appropriate.
PBC is confirmed or strongly established by a specialist.
Key medicines
Ursodeoxycholic acidUse a licensed PBC formulation at approximately 13–15 mg/kg daily, within the product range of 12–16 mg/kg, divided initially if required and continued indefinitely; recalculate when weight changes.
SeladelparNICE TA1171 supports use within its marketing authorisation; the current Livdelzi SmPC dose is 10 mg by mouth once daily with or without food, added to UDCA after inadequate response or used alone when UDCA is not tolerated.