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Semiquantitative measurement of aquaporin-4 antibodies as a possible surrogate marker of neuromyelitis optica spectrum disorders with systemic autoimmune diseases

机译:Aquaporin-4抗体的半定量测量可作为系统性自身免疫性疾病的视神经脊髓炎谱系障碍的替代指标

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Objectives To assess the association between serum aquaporin-4 (AQP4) autoantibodies and neuromyelitis optica spectrum disorders (NMOSDs) associated with systemic autoimmune diseases. Methods We retrospectively studied 626 hospitalized patients with systemic lupus erythematosus (SLE) or Sj?- gren's syndrome (SS). We collected serum samples from those patients with suspected NMOSDs (i.e., myelitis or optic neuritis) at the time of onset and thereafter. AQP4 antibodies were measured by a cell-based indirect immunofluorescence assay using AQP4-transfected HEK-293 cells in a semi-quantitative manner. Results Sera from 6 patients with suspected NMOSDs and SLE (n = 3) or SS (n = 3) were evaluated. Among these, 2 patients' sera samples, i.e., 1 with SLE and 1 with SS, were positive for AQP4 antibodies. There was an inverse relationship between disease amelioration and antibody titer in one NMOSD patient, whereas the antibody titer remained high in the other NMOSD patient, whose clinical manifestations of NMOSDs did not improve despite intensive immunosuppressive treatments. Conclusions These results indicate that serum AQP4 antibodies are present in some SLE/SS patients with myelitis/optic neuritis and might be associated with clinical outcomes. The semi-quantitative measurement of the AQP4 antibody might be a possible surrogate marker in patients with NMOSDs associated with systemic autoimmune diseases.
机译:目的评估血清水通道蛋白4(AQP4)自身抗体与系统性自身免疫性疾病相关的视神经脊髓炎光谱疾病(NMOSDs)之间的关系。方法我们回顾性分析了626例住院的系统性红斑狼疮(SLE)或Sj?-gren综合征(SS)患者。我们在发病时及其后从那些疑似NMOSDs(即脊髓炎或视神经炎)的患者中收集血清样本。 AQP4抗体通过基于细胞的间接免疫荧光测定法进行测定,使用AQP4转染的HEK-293细胞以半定量方式进行。结果评估了6例疑似NMOSD和SLE(n = 3)或SS(n = 3)患者的血清。在这些患者中,有2例患者的血清样本,即1例SLE和1例SS为AQP4抗体阳性。一名NMOSD患者的疾病缓解与抗体滴度之间存在反比关系,而另一名NMOSD患者的抗体滴度仍然很高,尽管进行了严格的免疫抑制治疗,但其NMOSD的临床表现并未改善。结论这些结果表明,在一些SLE / SS脊髓炎/视神经炎患者中存在血清AQP4抗体,并且可能与临床结局有关。在伴有全身性自身免疫性疾病的NMOSD患者中,AQP4抗体的半定量测量可能是一种替代指标。

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