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Patients with autoimmune polyendocrine syndrome type 1 have an increased susceptibility to severe herpesvirus infections
Clinical Immunology ( IF 8.6 ) Pub Date : 2021-09-08 , DOI: 10.1016/j.clim.2021.108851
Iivo Hetemäki 1 , Saila Laakso 2 , Hannamari Välimaa 3 , Iivari Kleino 4 , Eliisa Kekäläinen 1 , Outi Mäkitie 5 , T Petteri Arstila 1
Affiliation  

Almost all patients with autoimmune polyendocrine syndrome type 1 (APS-1) have neutralizing antibodies against type 1 interferons (IFN), important mediators of antiviral defense. Recently, neutralizing anti-IFN antibodies were shown to be a risk factor of severe COVID-19. Here we show in a cohort of 44 patients with APS-1 that higher titers of neutralizing anti-IFNα4 antibodies are associated with a higher and earlier incidence of VZV reactivation (herpes zoster). The patients also present with uncommonly severe clinical sequelae of herpetic infections. APS-1 patients had decreased humoral immune responses to varicella zoster virus, but cellular responses were comparable to healthy controls. These results suggest that blocking the type I interferon pathway in patients with APS-1 patients leads to a clinically significant immune deficiency, and susceptibility to herpesviruses should be taken into account when treating patients with APS-1.



中文翻译:

1 型自身免疫性多内分泌综合征患者对严重疱疹病毒感染的易感性增加

几乎所有 1 型自身免疫性多内分泌综合征 (APS-1) 患者都具有抗 1 型干扰素 (IFN) 的中和抗体,IFN 是抗病毒防御的重要介质。最近,中和抗 IFN 抗体被证明是严重 COVID-19 的危险因素。在这里,我们在 44 名 APS-1 患者队列中显示,更高滴度的中和抗 IFNα4 抗体与更高和更早的 VZV 再激活(带状疱疹)发生率相关。这些患者还表现出罕见的严重疱疹感染临床后遗症。APS-1 患者对水痘带状疱疹病毒的体液免疫反应降低,但细胞反应与健康对照相当。这些结果表明,阻断 APS-1 患者的 I 型干扰素通路会导致临床上显着的免疫缺陷,

更新日期:2021-09-12
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