<prompt> Anti mitochondrial antibody is typically seen in: March 2005Hepatic cirrhosisCardiac cirrhosisPrimary sclerosing cholangitisPrimary biliary cirrhosis  <response>: Ans. D: Primary biliary cirrhosis 90 of patients with primary biliary cirrhosis PBC have autoantibodies in their blood that react with the inner lining of mitochondria. These autoantibodies are called antimitochondrial antibodies AMA. AMA specifically react against a component of this multienzyme complex called E2. In PBC, AMA preferentially react with the E2 component of one of the multienzymes that is called the pyruvate dehydrogenase complex PDC. Accordingly, the antigen is designated as PDC-E2. The practical impoance of all of this is that the PDC-E2 antigen is now used,in a diagnostic test for PBC. The PDC-E2 antigen is also referred to as M2, a term introduced to designate it as the second mitochondrial antigen. P-ANCA Perinuclear antineutrophil cytoplasmic antibody. It is positive in about 65 of patients with Primary sclerosing cholangitis PSC. 