Chevailler A, Saint-André J P, Préel J L, Boyer J, Joubaud F, Hurez D
Laboratoire d'Immunologie, CHU, Angers.
Ann Med Interne (Paris). 1988;139(1):19-23.
In a 13 year retrospective study mitochondrial antibodies were found in 1.71 p. 100 of patients about 60 years old with a strong female predominance. The presence of these antibodies was associated with a significantly increased level of the three classes of immunoglobulin and a marked cholestatic syndrome. In 76.9 p. 100 of cases the antibodies were associated with hepatic disease, mainly of immunological origin (67.7 p. 100). Primary biliary cirrhosis was the most frequent with significantly increased levels of mitochondrial antibodies, immunoglobin M, alkaline phosphatase and cholesterol. However, there was no correlation between the antibody levels and the clinical, biological and histological stages, thus ruling out any prognostic significance. The use of human cultured cells in the antibody detection assay increased the positivity of antinuclear antibodies compared with assays using classical rat liver substrates. Six sera were positive for anticentromere antibodies: 5/6 showed signs of the CRST syndrome with a primary biliary cirrhosis in 3/5 cases. The frequency of the association of primary biliary cirrhosis and other autoimmune diseases supports the results of previous reports as well as the finding of an association between mitochondrial antibodies and other auto-antibodies. In 3 cases primary biliary cirrhosis was associated with a chronic pancreatitis, suggesting a pluriglandular sicca syndrome, and in 3 other cases with a monoclonal IgA gammopathy. Mitochondrial antibodies are associated with other auto-immune non-hepatic diseases in 15.4 p. 100 of cases. The presence of increased levels of mitochondrial antibodies without any other auto-antibody associated with a chronic non-surgical cholestasis and an increased level of immunoglobulin M is still strongly suggestive of primary biliary cirrhosis.
在一项为期13年的回顾性研究中，在约60岁的患者中，每100人中有1.71人发现线粒体抗体，女性占比显著更高。这些抗体的存在与三类免疫球蛋白水平显著升高以及明显的胆汁淤积综合征相关。在76.9%的病例中，抗体与肝脏疾病相关，主要是免疫源性的（67.7%）。原发性胆汁性肝硬化最为常见，线粒体抗体、免疫球蛋白M、碱性磷酸酶和胆固醇水平显著升高。然而，抗体水平与临床、生物学和组织学阶段之间没有相关性，因此排除了任何预后意义。与使用经典大鼠肝脏底物的检测方法相比，在抗体检测试验中使用人培养细胞增加了抗核抗体的阳性率。6份血清抗着丝点抗体呈阳性：6例中有5例表现出CRST综合征的体征，其中3例伴有原发性胆汁性肝硬化。原发性胆汁性肝硬化与其他自身免疫性疾病的关联频率支持了先前报告的结果，以及线粒体抗体与其他自身抗体之间存在关联的发现。在3例中，原发性胆汁性肝硬化与慢性胰腺炎相关，提示存在多腺体干燥综合征，在另外3例中与单克隆IgA丙种球蛋白病相关。在15.4%的病例中，线粒体抗体与其他非肝脏自身免疫性疾病相关。线粒体抗体水平升高，且不存在任何与慢性非手术性胆汁淤积和免疫球蛋白M水平升高相关的其他自身抗体，这仍然强烈提示原发性胆汁性肝硬化。
