Purpose: We examined the relationship between autoantibodies to erythropoietin receptor (EPOR) and renal outcome in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).Materials and methods: Sixty-three Japanese patients with AAV were enrolled and followed for a median of 31.4 months. Patients were screened for serum anti-EPOR antibodies using an enzyme-linked immunosorbent assay. Associations of anti-EPOR antibodies with clinical parameters were analyzed using logistic-regression models.Results: Anti-EPOR antibodies were detected in 7 (11%) of the 63 patients, and levels of the antibodies decreased with immunosuppressive therapy. The presence of anti-EPOR antibodies was associated with a higher Birmingham vasculitis activity score. In addition, anti-EPOR antibodies were more frequently observed in patients with renal outcomes, which was defined as a sustained 50% reduction in the estimated glomerular filtration rate from baseline, than in those without. Multiple logistic regression analysis revealed that presence of anti-EPOR antibodies, as well as age at disease onset, were as risk factors for the renal outcome.Conclusion: Anti-EPOR antibodies were associated with the progression of renal dysfunction in patients with AAV.

中文翻译：

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抗中性粒细胞胞浆抗体相关性血管炎患者促红细胞生成素受体自身抗体与肾预后的关系。

目的：我们研究了抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）患者的促红细胞生成素受体（EPOR）自身抗体与肾预后之间的关系。材料与方法：纳入并追踪了63例日本AAV患者中位数为31.4个月。使用酶联免疫吸附测定法筛选患者的血清抗EPOR抗体。结果：在63例患者中有7例（11％）检出了抗EPOR抗体，并通过免疫抑制治疗降低了抗EPOR抗体的水平。抗EPOR抗体的存在与更高的伯明翰血管炎活性评分相关。此外，在有肾结局的患者中观察到抗EPOR抗体的频率更高，这被定义为与没有肾结石的患者相比，估计的肾小球滤过率较基线水平持续降低了50％。多元logistic回归分析显示，抗EPOR抗体的存在以及疾病发作的年龄是肾结局的危险因素。结论：抗EPOR抗体与AAV患者肾功能障碍的进展有关。