[Atypical primary biliary cirrhosis or autoimmune cholangitis?]

The term autoimmune cholangitis is used for a disease with clinical and pathological features of primary biliary cirrhosis (PBC), lack of anti-mitochondrial antibodies (AMA), presence of anti-nuclear antibodies (ANA), and good response to immunosuppresive therapy. The current knowledge of this condition is limited and it is not established whether or not it is a different syndrome from the CBP. We report a 51-year-old woman with chronic cholestasis, histological features of PBC, negative AMA and positive peripheral fluorescent ANA pattern, without response to prednisone.