ANTIMITOCHONDRIAL ANTIBODY PROFILES IN PATIENTS WITH PRIMARY BILIARY-CIRRHOSIS BEFORE ORTHOTOPIC LIVER-TRANSPLANTATION AND TITERS OF ANTIMITOCHONDRIAL ANTIBODY-SUBTYPES AFTER TRANSPLANTATION

Four antimitochondrial antibody profiles (A-D) have been defined in pr imary biliary cirrhosis according to the presence of antibodies to M2, M4, M8, and M9 in ELISA and the complement fixation test: A: anti-M9 positive in ELISA and western blot, B: anti-M9 and/or anti-M2 positive in ELISA, C: anti-M2, -M4 and/or -M8 positive in ELISA, D: anti-M2, - M4, and/or -M8 positive in ELISA and complement fixation test. These p rofiles predict the outcome of primary biliary cirrhosis in the early stages and reflect differences in the natural course of the disease (b enign versus progressive). In this study sera from 29 patients with ad vanced primary biliary cirrhosis who had received liver transplant wer e retested before and after orthotopic liver transplantation. Twenty-e ight were antimitochondrial antibody/anti-M2 positive, and one patient had only antibodies to nuclear dots in the immunofluorescence test on cell cultures. When the antimitochondrial antibody-profiles in these 28 anti-M2 positive patients were analysed, it became evident that 26 of them belonged to subgroup C or D before orthotopic liver transplant ation. Two patients had profile B; one had high titres of antinuclear and smooth muscle antibodies indicating an overlap syndrome between pr imary biliary cirrhosis and autoimmune chronic active hepatitis. The o ther patient had antibodies to nuclear dots in association with anti-M 2. None of the patients had profile A. Antibody titres were studied af ter orthotopic liver transplantation in 23 of the 28 patients who surv ived for 1 to 13 years. Anti-M2 remained positive despite immunosuppre ssive therapy in 16 of them, although titres decreased; anti-M4, anti- M8, and anti-M9 became negative in most instances. These data confirm previous observations that antimitochondrial antibody-profiles A/B are not or only occasionally detected in patients with advanced primary b iliary cirrhosis, in contrast to profiles C/D which are reliable indic ators for a progressive course. (C) Journal of Hepatology.