Peakman M, Bevis L, Mieli-Vergani G, Mowat A P, Vergani D
Department of Immunology, King's College School of Medicine and Dentistry, Denmark Hill, London.
Autoimmunity. 1989;3(4):271-80. doi: 10.3109/08916938908997097.
Autoimmune chronic active hepatitis (aCAH) and primary sclerosing cholangitis (PSC) are liver disorders occurring in childhood in which non-organ specific autoantibodies, such as anti-nuclear antibody (ANA) are frequently found. Antibodies to double stranded DNA (dsDNA), which are typically present in systemic lupus erythematosus (SLE), have been detected in both acute and chronic liver diseases in adults. In this study, using a radioimmunoassay technique widely employed to measure antibodies to dsDNA, we have demonstrated significantly increased levels (median and range; 11.9, 1.0-36.5 U/ml) in 21 children with aCAH compared with normal children (1.0, 0.7-2.1 U/ml; p less than 0.01). Five children with aCAH had levels in the range considered diagnostic for SLE (greater than 25 U/ml) and of these, three had ANA and two had anti-liver kidney microsomal antibody. In addition, one child had antibodies to dsDNA as detected by the Crithidia luciliae test. DNA binding in aCAH was correlated with serum aspartate-amino transferase levels (r = 0.68; p less than 0.001), suggesting a direct relationship with disease activity. In PSC, levels of antibodies to dsDNA were significantly increased compared to normal controls (median and range; 7.0, 5.6-10.2 U/ml; p less than 0.01) but were not as high as those found in aCAH.
自身免疫性慢性活动性肝炎（aCAH）和原发性硬化性胆管炎（PSC）是儿童期出现的肝脏疾病，在这些疾病中经常发现非器官特异性自身抗体，如抗核抗体（ANA）。在成人的急性和慢性肝病中均检测到通常存在于系统性红斑狼疮（SLE）中的双链DNA（dsDNA）抗体。在本研究中，我们使用广泛用于测量dsDNA抗体的放射免疫测定技术，发现21例aCAH儿童的dsDNA抗体水平显著升高（中位数及范围；11.9，1.0 - 36.5 U/ml），而正常儿童的水平为（1.0，0.7 - 2.1 U/ml；p小于0.01）。5例aCAH儿童的dsDNA抗体水平处于被认为可诊断SLE的范围内（大于25 U/ml），其中3例有ANA，2例有抗肝肾微粒体抗体。此外，1例儿童通过利什曼原虫试验检测到dsDNA抗体。aCAH中的DNA结合与血清天冬氨酸氨基转移酶水平相关（r = 0.68；p小于0.001），提示与疾病活动存在直接关系。在PSC中，dsDNA抗体水平与正常对照组相比显著升高（中位数及范围；7.0，5.6 - 10.2 U/ml；p小于0.01），但不如aCAH中发现的水平高。
