- Review Article- Autoimmune Cholangitis: Is It an Antimitochondrial Antibody Negative Primary Biliary Cirrhosis?

Autoimmune cholangitis/cholangiopathy (AIC) is a re-cently described disease characterized by chronic cholangitis resembling primary biliary cirrhosis (PBC) with a high frequency of antinuclear antibodies (ANA) and with sero-negativity for antimitochondrial antibodies (AMA). Whether AIC is a disease entity distinct from PBC and autoimmune hepatitis (AIH) or whether it is an AMA-negative variant of PBC or a cholangiopathic variant of AIH have so far been controversial. We recently examined the specificities of AMA and ANA in Japanese patients with AIC, PBC and AIH by immunofluorescence, immunoblotting and enzyme inhibition assays using various mitochondrial and nuclear autoantigens including 2-oxo-acid dehydrogenase complex, Sp100, gp210, and p62, and found that AIC and PBC had similar patterns of immunoreactivity. However, this duo is of interest because, usually, among sets of autoimmune syndromes, differences in serological targetting are matched by differences in clinical presentation: AIC and PBC seem to be an exception to this rule. While it is true that a single etiological agent can produce a wide range of disease expression, it is possi-ble that seemingly similar clinicopathological features can be induced by pathogenetic mechanisms caused by a diver-sity of etiological agents. Key Words: autoimmune cholangitis, primary biliary cirrho-sis, autoimmune hepatitis, antimitochondrial anti