Department of Clinical Immunology, Wroclaw Medical University, 50-368, Wroclaw, Poland.
Department of Clinical Immunology and Paediatrics, Provincial Hospital J.Gromkowski, 51-149, Wroclaw, Poland.
BMC Immunol. 2023 Jun 3;24(1):8. doi: 10.1186/s12865-023-00543-6.
The correlation between primary immunodeficiencies (PIDs) and autoimmunity shows ethnic and geographical diversity. The aim of our study was to accumulate more data in paediatric PID population.
58 children aged 1-17 and with PID (study group) and 14 age-matched immunocompetent individuals (control group) were included in the study. Serum levels of 17 different specific IgG antibodies against autoantigens were measured by means of a quantitative enzyme immunoassay. Immunoglobulin levels were analysed in relation to a detailed medical examination.
Autoantibodies against one or more antigens were detected in the sera of 24.14% (n = 14) subjects in the study group. The most frequent were anti-thyroid peroxidase (anti-TPO) antibodies (n = 8; 13.8%). Anti-TPO antibody levels were elevated more often in PID patients with a positive family history of autoimmune diseases (p = 0.04). The screening for anti-deamidated gliadin peptide (DGP) and anti-tissue transglutaminase (tTG) antibodies in our series allowed identifying two previously undiagnosed cases of coeliac disease in PID patients. There was no statistically significant difference between the study and the control group in terms of the autoantibodies prevalence.
This study provides data on the prevalence of autoantibodies in paediatric population diagnosed with PID. Selected autoantibodies (i.e. anti-tTG, anti-DGP) might be useful for the screening of PID to avoid the delay of diagnosis of an autoimmune disease.
原发性免疫缺陷(PID)与自身免疫之间的相关性表现出种族和地域的多样性。我们的研究旨在为儿科 PID 人群积累更多数据。
本研究纳入了 58 名 1-17 岁的 PID 儿童(研究组)和 14 名年龄匹配的免疫功能正常者(对照组)。通过定量酶免疫分析检测血清中 17 种不同自身抗原的特异性 IgG 抗体水平。对免疫球蛋白水平进行分析,并结合详细的医学检查。
在研究组中,24.14%(n=14)的患者血清中检测到一种或多种自身抗体。最常见的是抗甲状腺过氧化物酶(anti-TPO)抗体(n=8;13.8%)。抗 TPO 抗体水平在 PID 患者中有自身免疫性疾病阳性家族史的患者中升高更为常见(p=0.04)。在我们的系列中,对脱酰胺麦胶肽(DGP)和抗组织转谷氨酰胺酶(tTG)抗体的筛查,在 PID 患者中发现了两例以前未诊断的乳糜泻病例。研究组和对照组之间在自身抗体的患病率方面无统计学差异。
本研究提供了儿科 PID 患者自身抗体患病率的数据。某些自身抗体(如抗 tTG、抗 DGP)可能有助于 PID 的筛查,以避免自身免疫性疾病的诊断延迟。