[Autoimmune cholangitis successfully treated with corticotherapy. One case].

Autoimmune cholangitis is a rare cause of chronic liver disease which has recently been described and associates the clinical, biological, and histological patterns of primary biliary cirrhosis without serum anti-mitochondrial antibodies. We report a case of this disease in a 67-year-old female. The patient presented with jaundice and marked biological cholestasis associated with pulmonary fibrosis and salivary and lacrymal sicca syndrome. Serum anti-smooth muscle antibodies were found without anti-mitochondrial antibodies. Corticotherapy resulted in rapid improvement of clinical and hepatic abnormalities, as well as of pulmonary lesions. The patient was still healthy 18 months later, with low dose corticotherapy. This report emphasizes the possible effectiveness of corticotherapy in autoimmune cholangitis.