Primary Biliary Cholangitis Management

Diagnostic Evaluation

• Abdominal ultrasound is essential in all patients with suspected Primary Biliary Cholangitis to exclude alternative causes of cholestasis, with a target of 90% of patients, as recommended by the European Association for the Study of the Liver 1

Treatment

• The American Association for the Study of Liver Diseases and the European Association for the Study of the Liver recommend Ursodeoxycholic acid (UDCA) as the first-line treatment for all patients with Primary Biliary Cholangitis, with a dose of 13-15 mg/kg/day, and strong evidence supporting its use 1
• Obeticholic acid (OCA) is recommended as a second-line therapy for patients with inadequate response to UDCA, with a starting dose of 5 mg once daily for 3 months, and titration to 10 mg once daily based on tolerability and response 1
• Patients with bilirubin >3 mg/dL should not receive OCA due to significantly increased risk of serious adverse events, and alternative therapies for PBC management should be considered, as recommended by the American Association for the Study of Liver Diseases 2
• Ocaliva is contraindicated in patients with PBC with decompensated cirrhosis (Child-Pugh Class B or C), prior decompensation events, compensated cirrhosis with evidence of portal hypertension, or complete biliary obstruction 2
• The following treatment options are available:

Monitoring and Assessment

• The American Association for the Study of Liver Diseases recommends assessing response to UDCA therapy at 12 months using biochemical response indices, including alkaline phosphatase (ALP), bilirubin, transaminases (AST/ALT), and albumin 1
• The European Association for the Study of the Liver suggests monitoring these parameters every 3 months 1
• Inadequate response criteria are defined as any of the following: ALP ≥1.67 × upper limit of normal (ULN), total bilirubin >ULN, or less than 15% reduction in ALP from baseline 1
• Patients with elevated GGT >3.2-fold ULN at 12 months or APRI score >0.54 after 12 months of UDCA therapy should be considered for earlier or more aggressive second-line therapy 3
• The American Association for the Study of Liver Diseases recommends documenting the presence or absence of pruritus in 90% of patients annually, with first-line treatment being Cholestyramine and second-line treatment being Rifampicin (with liver monitoring) 1
• The European Association for the Study of the Liver suggests evaluating fatigue in 90% of patients annually and considering psychological support for patients with profound psychological distress associated with fatigue 1

Special Considerations

• Overlap with autoimmune hepatitis is rare, but should be confirmed with liver biopsy when suspected, as recommended by the European Association for the Study of the Liver 1
• The American Association for the Study of Liver Diseases recommends risk assessment for osteoporosis in all patients with PBC, with 80% of patients having an assessment within 5 years of diagnosis, and treatment according to national osteoporosis guidelines 1
• The European Association for the Study of the Liver suggests that all patients with bilirubin >50 μmol/L or evidence of decompensated liver disease should be discussed with a transplant center 1
• UDCA should be continued during pregnancy, but OCA is not recommended due to lack of safety data, according to the European Association for the Study of the Liver 4
• The American Association for the Study of Liver Diseases recommends that UDCA should be given lifelong after liver transplantation to prevent recurrence 5
• The European Association for the Study of the Liver suggests that no specific immunosuppressive regimen is recommended over another to prevent recurrence 5

Patient Support and Education

• Offer patients the opportunity to seek support from patient support groups, and recommend resources such as the PBC Foundation, LIVErNorth, and Liver4Life, as recommended by the European Association for the Study of the Liver 1
• Consider psychological support for patients with profound psychological distress, and cognitive behavioral therapy may be beneficial, as suggested by the European Association for the Study of the Liver 1