Mitochondrial antibody test—a clue to diagnosis of primary biliary cirrhosis

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PRIMARY biliary cirrhosis, described by Hanot1 in 1875 as a chronic intrahepatic cholestasis occurring mainly in women, was largely neglected as an entity until the publication of the complete clinical description by MacMahon,2 Ahrens and associates,3 Sherlock,4 and others. The studies in pathology by Albot, Nezelof, and Lunel,5 Caroli and others6 in France, and by Rubin, Schaffner, and Popper7 in this country first with the light microscope and then with the electron microscope, described the lesion to be at the level of the small intrahepatic ducts and ductules. None of the studies defined the pathogenesis of this peculiar and fatal syndrome.

Since the diagnosis of primary biliary cirrhosis requires the absence of obstruction of the extrahepatic and large intrahepatic biliary ducts, and requires histologic confirmation of intrahepatic cholestasis, in questionable cases the examining clinician is often forced into advising an exploratory laparotomy. In order to avoid surgery, new means of diagnosis have been sought. Certain clinical and pathologic features of primary biliary cirrhosis have led many investigators to focus on the immunologic aspects of this puzzling hepatic disease. Paronetto, Schaffner, and Popper8 found accumulation of IgM macroglobulins in the mesenchymal cells in the portal spaces and fibrous septa as well as antiductular antibodies in patients who had primary biliary cirrhosis. Albano and associates9 and Bevan and associates10, 11 pointed out the high concentrations of serum IgM found in primary biliary cirrhotic patients. The latex agglutination test for rheumatoid factor, the antinuclear test, the complement-fixation test with nuclei, and smooth-muscle antibody . . .



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