Summary
A histopathological study was carried out on 27 patients with chronic inflammatory liver disease and clinical and/or biochemical evidence of cholestasis who had either mitochondrial antibodies against mitochondrial antigen fractions of 1.19 density (“PBC antigen”; 14 cases) or of 1.13 density (“CAH-PBC mixed-type antigen”; 13 cases). For comparison, the liver biopsies of 17 patients with chronic-aggressive hepatitis (CAH) and antinuclear and/or anti-smooth muscle antibodies but without cholestasis and mitochondrial antibodies, were evaluated. The 14 patients with mitochondrial antibodies against the PBC antigen showed the typical histological features of primary biliary cirrhosis (PBC). The 13 patients with mitochondrial antibodies against the CAH-PBC mixed-type antigen had heterogenous liver alterations. In 11 cases highly active CAH and/or active postnecrotic cirrhosis (AC) were found both with augmented ductular proliferation. Some of these cases showed distinct criteria of PBC as early bile duct lesions or absence of regular bile ducts. The liver histology of one case corresponded to classical PBC; another case to chronic persistent hepatitis. The CAH-patients without cholestasis and mitochondrial antibodies only occasionally showed bile duct proliferation. In conclusion, a high correlation was found between mitochondrial antibodies against the CAH-PBC mixed-type antigen and highly active CAH or early AC with augmented ductular proliferation. This represents an overlapping of CAH and PBC. In contrast, the cases with antibodies reacting to the PBC antigen showed the slowly progressive liver changes of typical PBC.
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References
Ahrens, E.H., Jr., Payne, M.A., Kunkel, H.G., Eisenmenger, W.J., Bondheim, S.H.: Primary biliary cirrhosis. Medicine (Baltimore) 29, 299 (1950)
Berg, P.A., Binder, T.: Demonstration of mitochondrial antibodies in sera from patients with chronic liver diseases reacting with two different types of complement-fixing antigens. In: Chronic hepatitis. Int. Symp. Montecatini 1975, p. 79. Basel: Karger 1976
Berg, P.A., Binder, T., Lindner, H., Bannaski, H., Maas, D., Henning, H., Brügel, H.: HeterogenitÄt mitochondrialer Autoantikörper. Zur serologischen Diagnose der primÄren-biliÄren Zirrhose und des Pseudo-Lupus erythematodes mit Hilfe von zwei verschiedenen Antigenfraktionen. Dtsch. med. Wschr. 100, 1123 (1975)
Ber, P.A., Doniach, D.: Autoimmune Hepatitiden. Klinische und immunpathologische Aspekte. Dtsch. med. Wschr. 97, 1468 (1972)
Berg, P.A., Lindner, H., Breitsohl-Mörk, C., Merk, K.H., Schlote, W.: Different specificities of antimitochondrial antibodies in sera from patients with PBC and chronic active hepatitis with cholestasis. EASL Meeting Kopenhagen 1976, abstract 2
Berg, P.A., MÄrker, A., Traunecker, U.: Antikörpermuster bei autoimmunen Hepatiden: Klinische Signifikanz und differentialdiagnostische Bedeutung. Verh. dtsch. Ges. inn. Med. 79, 650 (1973)
Berg, P.A., Muscatello, U., Horne, R.W., Roitt, I.M., Doniach, D.: Mitochondrial antibodies in primary biliary cirrhosis. II. The complement fixing antigen as a component of inner mitochondrial membranes. Brit. J. exp. Path. 50, 200 (1969)
Bianchi, F.B., Penfold, P.L., Roitt, I.M.: Mitochondrial antibodies in primary biliary cirrhosis. V. Ultrastructural localization of the antigen to the inner mitochondrial membrane using direct peroxidase conjugate. Brit. J. exp. Path. 54, 652 (1973)
Boyer, J.L.: Chronic hepatitis — a perspective on classification and determinations of prognosis. Gastroenterology 70, 1161 (1976)
Brunner, G., Vido, I., Schmidt, G.: Die Diagnose der primÄr-biliÄren Zirrhose. Klinische, biochemische, immunologische und morphologische Befunde. Dtsch. med. Wschr. 97, 1448 (1972)
Christoffersen, P., Poulsen, H., Scheuer, P.J.: Abnormal bile duct epithelium in chronic aggressive hepatitis and primary biliary cirrhosis. Hum. Path. 3, 227 (1972)
Datta, D.V., Sherlock, S., Scheuer, P.J.: Postnecrotic cirrhosis with chronic cholestasis. Gut 4, 223 (1963)
De Groote, J., Desmet, V., Gedigk, P., Korb, G., Popper, H., Poulsen, H., Scheuer, P.J., Schmid, M., Thaler, H., Uehlinger, E., Wepler, W.: Systematik der chronischen Hepatitis. Dtsch. med. Wschr. 93, 2101 (1968)
Doniach, D.: The concept of an “autoallergic” hepatitis. Proc. roy. Soc. Med. 63, 527 (1970)
Doniach, D., Walker, J.G.: A unified concept of autoimmune hepatitis. Lancet 1969 I, 813
Foulk, W.T., Baggenstoss, A.H., Butt, H.R.: Primary biliary cirrhosis: reevaluation by clinical and histologic study of 49 cases. Gastroenterology 47, 354 (1964)
Grob, P.J., Müller-Schoop, J.W., HÄcki, M.A., Joller-Jemelka, H.: Drug induced pseudolupus. Lancet 1975 II, 144
Hadziyannis, S., Scheuer, P.J., Feizi, T., Naccarato, R., Doniach, D., Sherlock, S.: Immunological and histological studies in primary biliary cirrhosis. J. clin. Path. 23, 95 (1970)
Hanot, V.: Etude sur une forme de cirrhose hypertrophique du foie (Cirrhose hypertrophique avec ictère chronique). Paris: J.B. Baillière 1876
Hoffbauer, F.W.: Primary biliary cirrhosis. Observation on the natural course of the disease in 25 women. Amer. J. dig. Dis. 5, 348 (1960)
Jones, W.A., Tisdale, W.A.: Posthepatitic cirrhosis clinically simulating extrahepatic biliary obstruction (socalled “primary biliary cirrhosis”). New Engl. J. Med. 268, 629 (1963)
Klatskin, G.: Subacute hepatic necrosis and postnecrotic cirrhosis due to anicteric infections with the hepatitis virus. Amer. J. Med. 25, 333 (1958)
Klatskin, G., Kantor, F.S.: Mitochondrial antibody in primary biliary cirrhosis and other diseases. Ann. intern. Med. 77, 533 (1972)
Maas, D., Schubothe, H.: Ein Lupus-erythematodes-Ähnliches Syndrom mit antimitochondrialen Antikörpern. Dtsch. med. Wschr. 98, 131 (1973)
MacKay, I.R.: Lupoid hepatitis and primary biliary cirrhosis: autoimmune diseases of the liver. Bull. rheum. Dis. 18, 487 (1968)
MacKay, I.R., Wood, I.J.: Lupoid hepatitis: a comparison of 22 cases with other types of chronic liver diseases. Quart. J. Med. 31, 485 (1962)
MacLachlan, M.J., Rodnan, G.P., Cooper, W.M., Fennel, R.M., Jr.: Chronic active (“lupoid”) hepatitis. A clinical, serological and pathological study of 20 patients. Ann. intern. Med. 62, 425 (1965)
Lindner, H., Dammermann, R., Klöppel, G.: Zur laparoskopischen Stadieneinstellung der primÄrbiliÄren Leberzirrhose. In: Fortschritte der gastroenterologischen Endoskopie, p. 79. Ed. H. Lindner. Baden-Baden: Witzstrock 1975
Lüders, C.J., Kruck, P., Henning, H., Look, D.: Histologische und klinische Untersuchungen zur Differentialdiagnose der aggressiven chronischen Hepatitis und der primÄren biliÄren Lebercirrhose. Z. Gastroent. 12, 467 (1974)
Pisi, E., Bianchi, F.B., Marchesini, G., Zauli, D.: Morphologic and immunological aspects of primary biliary cirrhosis. In: Intrahepatic cholestasis, pp. 105. Ed. P. Gentilini, U. Teodori, S. Gorini and H. Popper. New York: Raven Press 1975
Rubin, E., Schaffner, F., Popper, H.: Primary biliary cirrhosis. Chronic non-suppurative destructive cholangitis. Amer. J. Path. 46, 387 (1965)
Ruckstuhl, P., Cueni, B., Schmid, M.: Chronisch-aggressive Hepatitis in Kombination mit chronisch destruierender, nicht-eitriger Cholangitis (sogenannte primÄr biliÄre Zirrhose). Schweiz. med. Wschr. 101, 741 (1971)
Schaffner, F.: Primary biliary cirrhosis. Clin. Gastroent. 4, 351 (1975)
Scheuer, P.J.: Primary biliary cirrhosis. Proc. roy. Soc. Med. 60, 1257 (1967)
Schmid, M.: Der chronische Verschlu\ikterus und seine Beziehung zur biliÄren Leberzirrhose. In: Akute und chronische Lebererkrankungen, p. 63. Ed. H. Begemann, A. Kühn, R. Mancke und H. Kalk. Stuttgart: Thieme 1966
Schmidt, G., Kienle, J., Schmidt, D.S., Brunner, G.: HÄufigkeit der chronischen nicht-eitrigen destruierenden Cholangitis und primÄren biliÄren Leberzirrhose im Vergleich mit anderen chronischen entzündlichen Lebererkrankungen. Dtsch. med. Wschr. 101, 693 (1976)
Selmair, H., Vido, I., Wildhirt, E., Ortmanns, H.: Die chronisch-nekrotisierende Hepatitis. Dtsch. med. Wschr. 95, 1397 (1970)
Sherlock, S.: Primary biliary cirrhosis (chronic intrahepatic obstructive jaundice). Gastroenterology 31, 574 (1959)
Sherlock, S.: The immunology of liver diseases. Amer. J. Med. 49, 693 (1970)
Sherlock, S., Scheuer, P.J.: The presentation and diagnosis of 100 patients with primary biliary cirrhosis. New Engl. J. Med. 289, 674 (1973)
Walker, J.G., Doniach, D., Roitt, J.M., Sherlock, S.: Serological tests in diagnosis of primary biliary cirrhosis. Lancet 1965 I, 827
Wildhirt, E.: Die chronische-nekrotisierende Hepatitis. In: Chronische Hepatitis-Zirrhosen, p. 70. Stuttgart: Thieme 1974
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Klöppel, G., Seifert, G., Lindner, H. et al. Histopathological features in mixed types of chronic aggressive hepatitis and primary biliary cirrhosis. Virchows Arch. A Path. Anat. and Histol. 373, 143–160 (1977). https://doi.org/10.1007/BF00432159
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DOI: https://doi.org/10.1007/BF00432159