SJOGRENS-SYNDROME WITH ANTIMITOCHONDRIAL ANTIBODY-NEGATIVE PRIMARY BILIARY-CIRRHOSIS - A CASE OF AUTOIMMUNE CHOLANGITIS

Michieletti et al. (I) recently described a group of 17 patients who were initially thought to have primary biliary cirrhosis (PBC); however, the immunological findings differed from those usually associated with PBC. In particular, they did not have antimitochondrial antibody (AMA) and they did have antinuclear antibodies (often at very high titers), smooth muscle antibodies (30%), and comparatively low serum immunoglobulin (Ig) M and aspartate aminotransferase (AST). The investigators believe that patients with AMA-negative PBC may have a distinct syndrome of autoimmune cholangitis. We report a case of AMA-negative PBC in a patient with Sjogren's syndrome with immunological findings compatible with the diagnosis of autoimmune cholangitis.