Fujikura S, Davis P A, Prindiville T, Leung P, Fox R I, Gershwin M E
Division of Rheumatology, Allergy and Clinical Immunology, University of California 95616.
J Rheumatol. 1990 Nov;17(11):1453-7.
Sjögren's syndrome is well known for the presence of antibodies directed at specific nuclear antigens. However, the presence of antibodies reacting with a variety of other self antigens, including antimitochondrial antibodies, has often been reported although their significance is unknown. Moreover, patients with Sjögren's syndrome have been occasionally reported to be concordant with primary biliary cirrhosis. To address this issue we studied in a group of 96 patients with Sjögren's syndrome the presence of autoantibodies to the dihydrolipoamide acetyltransferases of both pyruvate dehydrogenase and branch chain ketoacid dehydrogenase and to alpha-ketoglutarate dehydrogenase; these latter enzymes are the mitochondrial target antigens of primary biliary cirrhosis. We report that 7 of the 96 patients reacted with the mitochondrial antigens that are prominent in primary biliary cirrhosis. Moreover, in those patients showing reactivity with mitochondrial antigens, the autoantibodies were directed at the same immunodominant epitopes that have been previously characterized in primary biliary cirrhosis. One of the 7 positive patients was known to have primary biliary cirrhosis. We hypothesize that the remaining 6 patients are at clinical risk for the development of primary biliary cirrhosis and/or that abnormalities would be found on liver biopsy.
干燥综合征以存在针对特定核抗原的抗体而闻名。然而，尽管其意义尚不清楚，但经常有报道称存在与多种其他自身抗原反应的抗体，包括抗线粒体抗体。此外，偶尔有报道称干燥综合征患者与原发性胆汁性肝硬化相符。为了解决这个问题，我们在一组96例干燥综合征患者中研究了针对丙酮酸脱氢酶和支链酮酸脱氢酶的二氢硫辛酰胺乙酰转移酶以及α-酮戊二酸脱氢酶的自身抗体的存在情况；后一种酶是原发性胆汁性肝硬化的线粒体靶抗原。我们报告说，96例患者中有7例与原发性胆汁性肝硬化中突出的线粒体抗原发生反应。此外，在那些与线粒体抗原呈反应性的患者中，自身抗体针对的是先前在原发性胆汁性肝硬化中已鉴定出的相同免疫显性表位。7例阳性患者中有1例已知患有原发性胆汁性肝硬化。我们推测，其余6例患者有发生原发性胆汁性肝硬化的临床风险，和/或肝活检会发现异常。
