Leonardi Lucia, Lorenzetti Giulia, Carsetti Rita, Piano Mortari Eva, Guido Cristiana Alessia, Zicari Anna Maria, Förster-Waldl Elisabeth, Loffredo Lorenzo, Duse Marzia, Spalice Alberto
Department of Maternal, Infantile and Urological Sciences, Sapienza University of Rome, Rome, Italy.
Department of Pediatrics, University of Rome Tor Vergata, Rome, Italy.
Front Pediatr. 2024 Jan 4;11:1216282. doi: 10.3389/fped.2023.1216282. eCollection 2023.
This cross-sectional study aimed to contribute to the definition of Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS) pathophysiology. An extensive immunological assessment has been conducted to investigate both immune defects, potentially leading to recurrent Group A β-hemolytic Streptococcus (GABHS) infections, and immune dysregulation responsible for a systemic inflammatory state. Twenty-six PANDAS patients with relapsing-remitting course of disease and 11 controls with recurrent pharyngotonsillitis were enrolled. Each subject underwent a detailed phenotypic and immunological assessment including cytokine profile. A possible correlation of immunological parameters with clinical-anamnestic data was analyzed. No inborn errors of immunity were detected in either group, using first level immunological assessments. However, a trend toward higher TNF-alpha and IL-17 levels, and lower C3 levels, was detected in the PANDAS patients compared to the control group. Maternal autoimmune diseases were described in 53.3% of PANDAS patients and neuropsychiatric symptoms other than OCD and tics were detected in 76.9% patients. ASO titer did not differ significantly between the two groups. A possible correlation between enduring inflammation (elevated serum TNF-α and IL-17) and the persistence of neuropsychiatric symptoms in PANDAS patients beyond infectious episodes needs to be addressed. Further studies with larger cohorts would be pivotal to better define the role of TNF-α and IL-17 in PANDAS pathophysiology.
这项横断面研究旨在为与链球菌感染相关的儿童自身免疫性神经精神障碍(PANDAS)的病理生理学定义提供帮助。已进行了广泛的免疫学评估,以调查可能导致A组β溶血性链球菌(GABHS)反复感染的免疫缺陷,以及导致全身炎症状态的免疫失调。招募了26例疾病呈复发-缓解病程的PANDAS患者和11例复发性咽扁桃体炎患者作为对照。每位受试者都接受了详细的表型和免疫学评估,包括细胞因子谱分析。分析了免疫参数与临床病史数据之间可能存在的相关性。使用一级免疫学评估,两组均未检测到先天性免疫缺陷。然而,与对照组相比,PANDAS患者中检测到TNF-α和IL-17水平升高以及C3水平降低的趋势。53.3%的PANDAS患者有母体自身免疫性疾病,76.9%的患者检测到除强迫症和抽动外的神经精神症状。两组之间抗链球菌溶血素O滴度无显著差异。需要探讨持续性炎症(血清TNF-α和IL-17升高)与PANDAS患者感染发作后神经精神症状持续存在之间可能存在的相关性。更大样本量的进一步研究对于更好地定义TNF-α和IL-17在PANDAS病理生理学中的作用至关重要。