Objective: In the background of an emerging role for immune dysregulation in neurodegenerative dementias, this study aimed to investigate the relationship between systemic autoimmunity and dementia. The objective was to study the frequency and profile of disease-specific autoantibodies in Alzheimer’s dementia (AD), frontotemporal dementia (FTD), and dementia with Lewy bodies (DLB). Methods: Immunological testing was performed in a large cohort of neurodegenerative dementia diagnosed based on standard clinical and imaging criteria. Patients were evaluated for the presence of autoantibodies specific for systemic autoimmune diseases that included anti-extractable nuclear antibody profile, rheumatoid factor antibody (RA), perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA), and cytoplasmic anti-neutrophil cytoplasmic antibody (c-ANCA) in serum. Results: Of 174 patients with degenerative dementia (FTD = 114, AD = 53, and DLB = 7) evaluated with immunological testing, 18.9% (n = 33) were seropositive for autoantibodies. The common antibodies detected were anti-Scl-70 (25%), anti-Ro-52 (18.7%), anti-nRNP-Sm (12.5%), and anti-CENP-B (9.3%). There were no significant systemic complaints in the majority of patients. A wider range of antibodies were positive in FTD compared to AD and DLB. While no difference was observed in the mean age, sex, or duration of illness between seropositive and negative patients, family history of dementia was more frequent among seronegative patients. Conclusion: Our findings indicate an emerging role for immune dysregulation in patients with classical neurodegenerative dementias, especially those with FTD. These autoantibodies could play a role in immune degradation of protein aggregates that characterize neurodegeneration. Study findings emphasize the need to explore the complex relationship between systemic autoimmunity and neurodegenerative dementia.
Dement Geriatr Cogn Disord

中文翻译：

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自身抗体在神经退行性痴呆中的作用：一个新兴的协会

目的：在免疫失调在神经退行性痴呆中发挥新作用的背景下，本研究旨在调查全身性自身免疫与痴呆之间的关系。目的是研究阿尔茨海默病 (AD)、额颞叶痴呆 (FTD) 和路易体痴呆 (DLB) 中疾病特异性自身抗体的频率和特征。方法：在根据标准临床和影像学标准诊断出的一大群神经退行性痴呆患者中进行了免疫学测试。评估患者是否存在全身性自身免疫性疾病特异性自身抗体，包括抗可提取核抗体谱、类风湿因子抗体 (RA)、核周抗中性粒细胞胞质抗体 (p-ANCA) 和细胞质抗中性粒细胞胞质抗体 (c -ANCA) 在血清中。结果：在接受免疫学检测评估的 174 名退行性痴呆患者（FTD = 114，AD = 53，DLB = 7）中，18.9%（n= 33) 自身抗体呈血清阳性。检测到的常见抗体是抗 Scl-70 (25%)、抗 Ro-52 (18.7%)、抗 nRNP-Sm (12.5%) 和抗 CENP-B (9.3%)。大多数患者没有明显的全身不适。与 AD 和 DLB 相比，FTD 中更广泛的抗体呈阳性。虽然血清阳性和阴性患者的平均年龄、性别或病程没有差异，但血清阴性患者的痴呆家族史更为常见。结论：我们的研究结果表明免疫失调在经典神经退行性痴呆患者中的新作用，尤其是那些 FTD。这些自身抗体可以在表征神经变性的蛋白质聚集体的免疫降解中发挥作用。研究结果强调需要探索全身性自身免疫与神经退行性痴呆之间的复杂关系。
老年痴呆症认知障碍