Antimitochondrial Antibody-Negative Primary Biliary Cholangitis Is It Really the Same Disease?

Antimitochondrial antibody (AMA)-negative primary biliary cholangitis (PBC) is a term reserved for the condition with clinical and histopathologic findings consistent with PBC but without positive AMA. There does not seem to be a natural progression from AMA negativity to positivity. Antinuclear and anti-smooth muscle antibodies are frequently found in the absence of histologic autoimmune hepatitis features. The disease course may be more severe than that of AMA-positive PBC. Response to standard therapy for PBC and autoimmune hepatitis varies. Nevertheless, there is insufficient evidence to suggest that AMA-negative PBC is different enough to warrant classification as a separate disease from AMA-positive PBC.