ANCA-associated RPGN leads to renal failure through systemic vasculitis and diffuse crescentic glomerulonephritis. MPO-ANCA-RPGN patients are highly susceptible to infections. Our aim in this study was to uncover reasons why these patients were susceptible to infections. We analyzed various aspects of type I interferon system including HVJ-stimulated IFN-α producing capacity and plasmacytoid dendritic cell (pDC) number in whole blood in MPO-ANCA-RPGN patients. Compared with healthy subjects, MPO-ANCA-RPGN patients showed impaired HVJ-stimulated IFN-α producing capacity and lower pDC number with or without glucocorticoid treatment. Immuno-histological staining of MPO-ANCA-RPGN kidney samples revealed a few but apparent pDC in T cell infiltrating regions even in patients with low pDC number in their peripheral blood. Patients' low HVJ-stimulated IFN-α producing capacity and pDC numbers persisted even after patients underwent several years of treatment. Former infection was determined using patients' serum BPI, Lamp-2 and Calprotectin, since they are reflective of a history of infection. These markers were higher in MPO-ANCA-RPGN patients than in healthy subjects. These results indicate that impaired HVJ-stimulated IFN-α production as well as dysfunction of the IFN system might have resulted from a previous bout of infection and can be partially implicated in patients' long-term susceptibility and vulnerability to infection.

中文翻译：

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MPO-ANCA 相关血管炎伴快速进展性肾小球肾炎中 HVJ 刺激的干扰素产生能力受损导致易感染

ANCA 相关的 RPGN 通过全身性血管炎和弥漫性新月体肾小球肾炎导致肾功能衰竭。MPO-ANCA-RPGN 患者对感染高度敏感。我们在这项研究中的目的是揭示这些患者易受感染的原因。我们分析了 I 型干扰素系统的各个方面，包括 MPO-ANCA-RPGN 患者全血中 HVJ 刺激的 IFN-α 产生能力和浆细胞样树突细胞 (pDC) 数量。与健康受试者相比，无论是否接受糖皮质激素治疗，MPO-ANCA-RPGN 患者均表现出 HVJ 刺激的 IFN-α 产生能力受损和 pDC 数量降低。MPO-ANCA-RPGN 肾脏样本的免疫组织学染色显示，即使在外周血 pDC 数较低的患者中，T 细胞浸润区域中也有少量但明显的 pDC。耐心' 即使在患者接受几年治疗后，低 HVJ 刺激的 IFN-α 产生能力和 pDC 数量仍然存在。以前的感染是使用患者的血清 BPI、Lamp-2 和钙卫蛋白确定的，因为它们反映了感染史。MPO-ANCA-RPGN 患者的这些标志物高于健康受试者。这些结果表明 HVJ 刺激的 IFN-α 产生受损以及 IFN 系统功能障碍可能是由先前的感染引起的，并且可能与患者的长期易感性和易感染性有关。因为它们反映了感染史。MPO-ANCA-RPGN 患者的这些标志物高于健康受试者。这些结果表明 HVJ 刺激的 IFN-α 产生受损以及 IFN 系统功能障碍可能是由先前的感染引起的，并且可能与患者的长期易感性和易感染性有关。因为它们反映了感染史。MPO-ANCA-RPGN 患者的这些标志物高于健康受试者。这些结果表明 HVJ 刺激的 IFN-α 产生受损以及 IFN 系统功能障碍可能是由先前的感染引起的，并且可能与患者的长期易感性和易感染性有关。