Frequency and syndrome specificity of antibodies to aquaporin-4 in neurological patients with rheumatic disorders
Frequency and syndrome specificity of antibodies to aquaporin-4 in neurological patients with rheumatic disorders
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DOI：
10.1177/1352458511403958
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发表时间：
2011-09-01
影响因子：
5.8
通讯作者：
Wildemann, Brigitte
中科院分区：
文献类型：
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作者：
Jarius, Sven;Jacobi, Christian;Wildemann, Brigitte
背景：最近在视神经脊髓炎 (NMO) 及其形式、纵向广泛横贯性脊髓炎 (LETM) 和复发性视神经炎 (rON) 患者中发现了一种新的自身抗体（称为 NMO-IgG 或 AQP4-Ab）。然而，AQP4-Ab 也存在于同时患有风湿性疾病的患者中，例如系统性红斑狼疮 (SLE) 或干燥综合征 (SS)，这些疾病的特征是广泛、多特异性 B 细胞激活。 目的：在这项研究中，我们旨在评估患有风湿性疾病和神经系统症状的患者中 AQP4-Ab 的综合征特异性和频率。通过使用重组人 AQP4 进行基于细胞的检测，对 109 名患有结缔组织疾病 (CTD) (n = 54)、可能患有 CTD (n = 42) 或血管炎 (n = 13) 的神经系统患者进行了 AQP4-Ab 的分析。结果：在 31/40 (78%) 患有 CTD 和 NMO 谱系疾病的患者中可检测到 AQP4-Ab（中位滴度， 1:1000)，但在除 NMO、LETM 或 rON (n = 69) 之外的 CTD 或血管炎和神经系统疾病患者中均未获得样本。结论：CTD 患者视神经脊髓炎谱系疾病 (NMOSD) 抗体的高综合征特异性支持 AQP4-Ab 参与这些神经系统疾病发病机制的概念，并反对 AQP4-Ab 仅仅作为一部分多克隆 B 细胞激活通常与风湿性疾病相关。此外，AQP4-Ab 存在于 CTD 和共存 NMOSD 的患者中的发现频率与无 CTD 的患者大致相同，这一发现强化了 CTD 和 AQP4-Ab 阳性 NMOSD 代表大多数患者中两种共存但不同实体的情况。
Background: A new autoantibody (termed NMO-IgG, or AQP4-Ab) has recently been described in patients with neuromyelitis optica (NMO) and its formes frustes, longitudinally extensive transverse myelitis (LETM) and recurrent optic neuritis (rON). However, AQP4-Ab has been found also in patients with co-existing rheumatic diseases such as systemic lupus erythematosus (SLE) or Sjogren's syndrome (SS), conditions which are characterized by broad, polyspecific B cell activation.Objectives: In this study, we aimed at evaluating the syndrome specificity and frequency of AQP4-Ab in patients with rheumatic diseases and neurological symptoms.Methods: For this purpose, serum samples from 109 neurological patients with established connective tissue disorders (CTD) (n = 54), possible CTD (n = 42), or vasculitis (n = 13) were analysed for the presence of AQP4-Ab by a cell-based assay employing recombinant human AQP4.Results: AQP4-Ab was detectable in 31/40 (78%) patients with CTD and NMO spectrum disorders (median titre, 1:1000) but in none of the samples obtained from patients with CTD or vasculitis and neurological disorders other than NMO, LETM, or rON (n = 69).Conclusion: The high syndrome specificity of the antibody for neuromyelitis optica spectrum disorders (NMOSDs) in patients with CTD supports the concept of AQP4-Ab being involved in the pathogenesis of these neurological conditions, and argues against AQP4-Ab simply being part of the polyclonal B cell activation generally associated with rheumatic diseases. Moreover, the finding that AQP4-Ab is present in patients with CTD and co-existing NMOSD with approximately the same frequency as in patients without CTD strengthens the case of CTD and AQP4-Ab positive NMOSD representing two co-existing yet distinct entities in the majority of patients.