Institute of Epidemiology, Faculty of Medicine, University of Belgrade, 11000, Belgrade, Serbia.
Clinic of Neurology, University Clinical Center of Serbia, Belgrade, Serbia.
J Neurol. 2024 Dec;271(12):7525-7536. doi: 10.1007/s00415-024-12698-2. Epub 2024 Sep 22.
The aim of our study was to estimate the frequency of autoimmune comorbidities, in NMOSD patients from the national Serbian NMOSD Registry.
Our study comprises 136 patients with NMOSD, diagnosed according to the NMOSD criteria 2015. At the time of the study, in the Registry were collected demographic and clinical data, including those related to the coexisting comorbidities and pathogenic autoantibodies. Not all patients were tested for all autoimmune antibodies. None of the seronegative aquaporin4-IgG (AQP4-IgG) NMOSD patients, included in the Registry, were positive for the myelin oligodendrocyte glycoprotein IgG.
Among 136 NMOSD patients, 50 (36.8%) had at least one associated autoimmune disorder. AQP4-IgG was present in the sera from 106 patients (77.9%), the proportion of NMOSD patients with autoimmune comorbidities being significantly higher in the AQP4-IgG positive subgroup in comparison to the AQP4-IgG negative (p = 0.002). AQP4-IgG seropositive NMOSD patients had 5.2-fold higher risk of comorbid autoimmune diseases (OR = 5.2, 95% CI 1.4-18.5, p = 0.012). The most frequently reported diseases were autoimmune thyroid disease (15.4%), Sjogren's syndrome (11.0%), systemic lupus erythematosus (5.1%), myasthenia gravis (4.4%), and primary antiphospholipid antibody syndrome (2.9%). Antinuclear antibodies (ANAs) were frequently detected in the subgroup of NMOSD patients tested for this antibody (50/92; 54.3%). The higher frequency of ANAs and anti-extractable nuclear antigen autoantibodies, in the subgroups of AQP4-IgG-positive patients compared to the AQP4-IgG negative, tested for these antibodies, was statistically significant (p = 0.009, and p = 0.015, respectively).
In conclusion, based on our results, in a defined cohort with European ethnical background, a wide spectrum of autoimmune diseases is frequently associated with AQP4-IgG seropositive NMOSD patients.
本研究的目的是评估塞尔维亚全国 NMOSD 登记处 NMOSD 患者合并自身免疫性疾病的频率。
本研究纳入了 136 名符合 NMOSD 2015 年诊断标准的 NMOSD 患者。在研究时，登记处收集了人口统计学和临床数据，包括与并存的合并症和致病性自身抗体相关的数据。并非所有患者都接受了所有自身抗体的检测。登记处中纳入的所有血清阴性水通道蛋白 4 免疫球蛋白 G（AQP4-IgG）NMOSD 患者均为髓鞘少突胶质细胞糖蛋白 IgG 阳性。
在 136 名 NMOSD 患者中，50 名（36.8%）至少合并一种自身免疫性疾病。106 名患者的血清中存在 AQP4-IgG（77.9%），AQP4-IgG 阳性亚组 NMOSD 患者合并自身免疫性疾病的比例明显高于 AQP4-IgG 阴性亚组（p=0.002）。AQP4-IgG 阳性 NMOSD 患者合并自身免疫性疾病的风险增加 5.2 倍（OR=5.2，95%CI 1.4-18.5，p=0.012）。报告最频繁的疾病为自身免疫性甲状腺疾病（15.4%）、干燥综合征（11.0%）、系统性红斑狼疮（5.1%）、重症肌无力（4.4%）和原发性抗磷脂抗体综合征（2.9%）。在接受此项抗体检测的 NMOSD 患者亚组中，经常检测到抗核抗体（ANA）（50/92；54.3%）。与接受此项抗体检测的 AQP4-IgG 阴性患者相比，AQP4-IgG 阳性患者的 ANA 和可提取核抗原自身抗体的频率更高，差异具有统计学意义（p=0.009 和 p=0.015）。
总之，根据我们的结果，在具有欧洲人种背景的特定队列中，广泛的自身免疫性疾病经常与 AQP4-IgG 阳性 NMOSD 患者相关。
