Gregorio G V, Davies E T, Mieli-Vergani G, Vergani D
Department of Immunology, King's College School of Medicine & Dentistry, London, UK.
Clin Exp Immunol. 1995 Nov;102(2):308-13. doi: 10.1111/j.1365-2249.1995.tb03782.x.
Antinuclear antibody (ANA) is found in connective tissue disorders and in autoimmune liver disease. While ANA-positive connective tissue disorders are subdivided according to possession of specific antibodies to extractable nuclear antigens (ENA) (anti-ribonucleoprotein (anti-RNP), anti-Smith (anti-Sm), anti-Ro, anti-La), little is known about the presence and significance of ENA in autoimmune liver disease. To investigate this, we have tested 35 children with autoimmune hepatitis (AIH) (19 ANA and/or smooth muscle antibody-positive (ANA/SMA+ve); 16 liver kidney microsomal 1-positive (LKM-1 + ve)) and 14 with ANA/SMA+ve autoimmune sclerosing cholangitis (ASC), using both double dimension immunodiffusion and ELISAs. Eighty children with non-autoimmune liver disease (20 alpha 1-antitrypsin deficiency, 20 Wilson's disease, 20 Alagille's syndrome and 20 chronic hepatitis B virus infection) and 20 healthy controls were also tested. ENA were detected in seven (20%) patients with AIH: two ANA-positive, one SMA-positive and four LKM-1-positive. Three were positive for anti-Sm, two for anti-La, one for anti-Sm/anti-La and one for anti-Sm/anti-La/anti-Ro. ENA-positive had more severe liver disease than ENA-negative patients (P < 0.03). ENA were not detected in ASC, non-autoimmune liver diseases and controls. Our results indicate that ENA reactivity, including anti-Sm and anti-La, characteristic of systemic lupus erythematosus and Sjögren's syndrome, respectively, are present in some patients with AIH even in the absence of ANA, and may characterize a particularly severe form of the disease.
抗核抗体（ANA）见于结缔组织病和自身免疫性肝病。虽然ANA阳性的结缔组织病可根据是否存在针对可提取核抗原（ENA）的特异性抗体（抗核糖核蛋白（抗RNP）、抗史密斯（抗Sm）、抗Ro、抗La）进行细分，但关于ENA在自身免疫性肝病中的存在情况及意义却知之甚少。为对此进行研究，我们对35例自身免疫性肝炎（AIH）患儿（19例ANA和/或平滑肌抗体阳性（ANA/SMA+ve）；16例肝肾微粒体1阳性（LKM-1 + ve））和14例ANA/SMA+ve自身免疫性硬化性胆管炎（ASC）患儿进行了检测，采用了双向免疫扩散法和酶联免疫吸附测定法（ELISA）。还对80例非自身免疫性肝病患儿（20例α1-抗胰蛋白酶缺乏症、20例威尔逊病、20例阿拉吉尔综合征和20例慢性乙型肝炎病毒感染）以及20例健康对照进行了检测。在7例（20%）AIH患儿中检测到了ENA：2例ANA阳性，1例SMA阳性，4例LKM-1阳性。3例抗Sm阳性，2例抗La阳性，1例抗Sm/抗La阳性，1例抗Sm/抗La/抗Ro阳性。ENA阳性的患儿比ENA阴性的患儿肝病更严重（P < 0.03）。在ASC、非自身免疫性肝病患儿和对照中未检测到ENA。我们的结果表明，分别为系统性红斑狼疮和干燥综合征特征性的ENA反应性，包括抗Sm和抗La，在一些AIH患儿中即使不存在ANA也存在，并且可能是该疾病一种特别严重形式的特征。
