Mach, Marc-Alexander von2008-06-172008-06-172002-12-181611-2156http://hdl.handle.net/2003/2562310.17877/DE290R-15144Two former classmates aged 58 with a history of more than 20 years of elevated liver function tests presented to our clinic. As they were both tested positive for antimitochondrial antibodies and liver biopsy showed distinctive bile duct lesions, they were given the diagnosis of primary biliary cirrhosis. To confirm presence of AMAs and subtype of AMAs western blotting was performed showing two distinct bands at 62 kD (PDH-E2) and 48 kD (2ODH-E2) in patient 1 and only one band at 62 kD in patient 2. Searching for potential explanations for this unusual occurence of this rare disease in two close friends a comparison of restriction fragment lengths polymorphism was performed and excluded any familial relationship. Interestingly, histocompatibility leukocyte antigen typing revealed strong similarities as they both shared A2, B51, DR4, DR8 and DR53. These results may point to an unknown environmental factor what caused primary biliary cirrhosis in immunologically susceptible individuals.enEXCLI Journal ; Vol. 1, 2002antibodyautoimmunitetiologyfrequencyprimary biliary cirrhosis610article (journal)