Primary biliary cholangitis (primary biliary cirrhosis)
Primary biliary cholangitis is the most likely diagnosis in a patient with chronic cholestasis (elevated cholestatic liver enzymes) and a positive anti-mitochondrial antibody (AMA) test. [1]
Medication Selection Algorithm
Not applicable for this diagnostic question.
Key Evidence Supporting This Recommendation
PBC is considered autoimmune and has a hallmark serologic signature of AMA. [1] The AASLD 2018 PBC guidance states that the diagnosis is largely confirmed with tests for AMA. [1] The EASL PBC guideline states that diagnosis is usually based on serum liver tests indicative of a cholestatic hepatitis in association with circulating antimitochondrial antibodies. [2]
Monotherapy vs Combination Therapy
Not applicable.
Important Clarifications or Nuances
Primary biliary cholangitis and primary sclerosing cholangitis are distinct entities, but both can present with cholestatic liver enzyme abnormalities. [1]
Initiation Thresholds
Not applicable.
Common Pitfalls to Avoid
Autoimmune hepatitis typically does not require AMA positivity for diagnosis, whereas AMA positivity is a major diagnostic anchor for PBC. [1], [2]
Targets or Goals of Therapy
Not applicable.
Most likely diagnosis
Primary biliary cholangitis (primary biliary cirrhosis). [1], [2]