Autoimmune cholangitis.

Autoimmune cholangitis is the term that has been used to describe patients who have the clinical, biochemical, and histologic characteristics of primary biliary cirrhosis (PBC), but who are antinuclear antibody positive rather than anti-mitochondrial antibody (AMA) positive in their sera. The course of their disease is similar to AMA positive cases, and the associated nonhepatic autoimmune diseases are the same in both AMA-positive and AMA-negative PBC. Serial testing for AMA using highly sensitive and specific techniques over time suggests that in subjects with autoimmune cholangitis, their AMA negative status remains negative. The beneficial response to treatment with ursodeoxycholic acid is the same as for AMA-positive PBC. It may be preferable to use the term autoimmune cholangitis, further stratified by AMA status, instead of the somewhat innapropriate term primary biliary cirrhosis.