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 w ere initially thought to have primary biliary cirrhosis (PBC); however , the immunological findings differed from those usually associated wi th PBC. In particular, they did not have antimitochondrial antibody (A MA) and they did have antinuclear antibodies (often at very high titer s), smooth muscle antibodies (30%), and comparatively low serum immuno globulin (Ig) M and aspartate aminotransferase (AST). The investigator s believe that patients with AMA-negative PBC may have a distinct synd rome of autoimmune cholangitis. We report a case of AMA-negative PBC i n a patient with Sjogren's syndrome with immunological findings compat ible with the diagnosis of autoimmune cholangitis.