Jańczyk Wojciech, Bierła Joanna B, Trojanowska Ilona, Wierzbicka-Rucińska Aldona, Cukrowska Bożena, Socha Piotr
Department of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730 Warsaw, Poland.
Department of Pathomorphology, Children's Memorial Health Institute, Al. Dzieci Polskich 20, 04-730 Warsaw, Poland.
Diagnostics (Basel). 2023 Feb 17;13(4):768. doi: 10.3390/diagnostics13040768.
Autoantibodies occur in healthy subjects as well as in children with Wilson's disease (WD), but their prevalence and significance are unknown. Thus, we aimed to assess the prevalence of autoantibodies and autoimmune markers, and their relationship to liver injury in WD children. The study included 74 WD and 75 healthy children as a control group. Patients with WD underwent transient elastography (TE) examinations, as well as determination of liver function tests, copper metabolism markers, and serum immunoglobulins (Ig). In the sera of the WD patients and controls, anti-nuclear (ANA), anti-smooth muscle, anti-mitochondrial, anti-parietal cell, anti-liver/kidney microsomal, anti-neutrophil cytoplasmic autoantibodies, and specific celiac antibodies were determined. Among the autoantibodies, only the prevalence of ANA in children with WD was higher than in the controls. There was no significant relationship between the presence of autoantibodies and liver steatosis or stiffness after TE. However, advanced liver stiffness (E > 8.2 kPa) was related to IgA, IgG, and gamma globulin production. The type of treatment did not influence the prevalence of autoantibodies. Our results suggest that autoimmune disturbances in WD might not be directly related to liver damage as expressed by steatosis and/or liver stiffness after TE.
自身抗体在健康受试者以及患有威尔逊病（WD）的儿童中均有出现，但其患病率及意义尚不清楚。因此，我们旨在评估WD患儿自身抗体和自身免疫标志物的患病率，以及它们与肝损伤的关系。该研究纳入了74例WD患儿和75例健康儿童作为对照组。WD患者接受了瞬时弹性成像（TE）检查，以及肝功能测试、铜代谢标志物和血清免疫球蛋白（Ig）的测定。在WD患者和对照组的血清中，测定了抗核抗体（ANA）、抗平滑肌抗体、抗线粒体抗体、抗壁细胞抗体、抗肝肾微粒体抗体、抗中性粒细胞胞浆自身抗体和特异性乳糜泻抗体。在这些自身抗体中，只有WD患儿的ANA患病率高于对照组。TE检查后，自身抗体的存在与肝脏脂肪变性或硬度之间没有显著关系。然而，严重的肝脏硬度（E>8.2 kPa）与IgA、IgG和γ球蛋白的产生有关。治疗方式并未影响自身抗体的患病率。我们的结果表明，WD中的自身免疫紊乱可能与TE检查后肝脏脂肪变性和/或肝脏硬度所表现出的肝损伤没有直接关系。
