PBC Algorithm

View PDF


The diagnosis of PBC can be established when two of the following three criteria are met:

  1. Biochemical evidence of cholestasis based on ALP elevation.
  2. Presence of AMA
  3. Histologic evidence of nonsuppurative destructive cholangitis and destruction of interlobular bile ducts.

Treatment:

  1. 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.
  2. Biochemical response to UDCA should be evaluated at 6 to 12 months after treatment initiation to determine whether patients should be considered for second-line therapy.
  3. If intolerance to UCDA then start Ocaliva monotherapy. If incomplete biochemical response to UCDA, then add Ocaliva
    1. Dosing – 5mg daily x 3 months. If incomplete response, then increase to 10mg daily
    2. Ocaliva contraindicated with decompensated cirrhosis or compensated cirrhosis with portal hypertension
  4. If concern for advanced fibrosis or cirrhosis by imaging, or lab markers (FIB 4 or APRI), then consideration of elastography to determine fibrosis.
  5. EGD for variceal screening and HCC screening if cirrhosis

References

Lindor KD et al. Primary Biliary Cholangitis: 2018 Practice Guidance From the American Association for the Study of Liver Diseases 2018.

Louie JS et al. Primary Biliary Cholangitis: A Brief Overview. Clinical Liver Disease, VOL 15, NO 3, MARCH 2020.