3. Liver biopsy to rule out concomitant AIH or other liver disease should be considered in PBC patients when the alanine aminotransferase activity is more than 5 times the upper limit of normal.
4. In cases of suspected PBC/AIH overlap, treatment should be targeted at the predominant histological pattern of injury.
5. UDCA in a dose of 13 to 15 mg/kg/day orally is recommended for patients with PBC who have abnormal liver enzyme values regardless of histologic stage.
6. For patients requiring bile acid sequestrants, UDCA should be given at least 1 hour before or 4 hours after the bile acid sequestrant.
7. Biochemical response to UDCA should be evaluated at 12 months after treatment initiation to determine whether patients should be considered for second-line therapy.
8. Patients who are inadequate responders to UDCA (Table 1) should be considered for treatment with OCA, starting at 5 mg/day.
9. Fibrates can be considered as off-label alternatives for patients with PBC and inadequate response to ursodeoxycholic acid, although fibrates are discouraged in patients with decompensated liver disease.
10. OCA is contraindicated in patients with advanced cirrhosis. This is defined as cirrhosis with current or prior evidence of liver decompensation (e.g., encephalopathy, coagulopathy) or portal hypertension (e.g., ascites, gastroesophageal varices, or persistent thrombocytopenia). Furthermore, we would recommend careful monitoring of any patient with cirrhosis, even if not advanced, receiving OCA.