Primary Biliary Cholangitis

Publication Date: August 24, 2021
Last Updated: March 14, 2022

Guidance Statements

Diagnosis

1. The diagnosis of PBC can be established when two of the following three criteria are met:
  • Biochemical evidence of cholestasis based on ALP elevation.
  • Presence of AMA, or other PBC-specific autoantibodies, including sp100 or gp210, if AMA is negative.
  • Histologic evidence of nonsuppurative destructive cholangitis and destruction of interlobular bile ducts
2. The diagnosis of AMA-negative PBC does not require a liver biopsy if other criteria are met, including cholestatic liver tests and PBC-specific autoantibodies such as sp100 or gp210.

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.

Follow-Up of PBC

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Liver tests every 3-6 months
TSH annually
Bone mineral densitometry every 2 years
Vitamins A, D, E and prothrombin time annually if bilirubin >2.0
Upper endoscopy every 1-3 years if cirrhotic, Mayo risk score >4.1, or transient elastography shows a score ≥17 kPa*
Ultrasound with or without alpha fetoprotein in patients with known or suspected cirrhosis† and men every 6 months

Overview

Title

Primary Biliary Cholangitis

Authoring Organization