Antimitochondrial Antibody Measurements in the Diagnosis of Antimitochondrial Antibody-negative and Alkaline Phosphatase-positive Primary Biliary Cholangitis (IMAGE)
Caption
False-negative AMA-negative PBC can be due to insensitive AMA tests, delayed seroconversion, or medication-induced suppression of AMA. These should be considered and excluded, and true AMA-negative PBC should be confirmed by ANA (anti-gp210 and anti-sp100).
The presentation, progression, and response to treatment of AMA-negative PBC have been shown to be similar to those of AMA-positive PBC.
Liver biopsy is not necessary to make a diagnosis of AMA-negative PBC except in the few cases where ANA (anti-gp210 and anti-sp100) are also negative.
GLOBE and UK-PBC scores remain useful clinical tools for prognostication and can be applied to AMA-negative cases, as such cases were included in validation studies of these tools.
Although AMA-negative PBC is often discussed as an “atypical” entity separate from AMA-positive PBC, the fact that progression and outcomes are virtually identical indicates that the presence or absence of AMA appears not to significantly influence disease development or progression. Therefore, labeling AMA-negative PBC as “atypical” is somewhat misleading.
The findings indicate that future research on PBC should focus on improving un-derstanding of its pathogenesis and on the discovery of new targeted therapies, including for AMA-negative cases.
Credit
Karol Suchowiecki, George Y. Wu
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CC BY-NC