Treatment of Primary Biliary Cholangitis

First-Line Treatment: UDCA

The European Association for the Study of the Liver recommends starting ursodeoxycholic acid (UDCA) at 13-15 mg/kg/day as first-line therapy for Primary Biliary Cholangitis (PBC), provided the patient does not have decompensated cirrhosis or evidence of portal hypertension 1, 2
UDCA at 13-15 mg/kg/day is the established treatment of choice for PBC based on multiple placebo-controlled trials and long-term case-control studies, significantly decreasing serum bilirubin, alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT), cholesterol, and immunoglobulin M levels compared to placebo 3, 2
Long-term UDCA treatment delays histological progression when started at early stages and reduces the likelihood of liver transplantation or death in patients with moderate to severe disease 3, 2
All patients with PBC should be offered UDCA therapy, with 90% of patients receiving therapy at an adequate dose or being documented as intolerant 1

After 1 year of UDCA therapy, perform biochemical response assessment using validated risk stratification tools, such as the GLOBE or UK-PBC Risk Scores, which are the most accurate 1
Monitor liver biochemistry regularly to assess treatment response, with 80% of patients having their response status documented 1

The European Association for the Study of the Liver recommends that if the patient has undergone liver transplantation for PBC, UDCA should be given lifelong at 10-15 mg/kg/day to prevent disease recurrence 4
Post-transplant UDCA reduces the risk of PBC recurrence and improves long-term graft survival and overall survival 4

UDCA does not significantly improve pruritus or fatigue, which are common symptoms in PBC 3
Evaluate all patients for the presence of fatigue and pruritus, with documentation in at least 90% of cases annually 1

Perform abdominal ultrasound to exclude alternative causes of cholestasis and assess for focal liver lesions, with 90% of patients having this at baseline 1
Assess osteoporosis risk within 5 years in 80% of patients, as PBC increases fracture risk 1
Refer to transplant hepatology if bilirubin exceeds 50 μmol/L or any evidence of decompensated liver disease develops 1

Never use high-dose UDCA (>20 mg/kg/day) as this has been associated with worse outcomes in cholestatic liver diseases 5, 2