Preexisting autoantibodies to type I IFNs underlie critical COVID-19 pneumonia in patients with APS-1.
Preexisting autoantibodies to type I IFNs underlie critical COVID-19 pneumonia in patients with APS-1.
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DOI：
10.1084/jem.20210554
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发表时间：
2021-07-05
期刊：
影响因子：
--
通讯作者：
Lionakis MS
中科院分区：
文献类型：
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作者：
Bastard P;Orlova E;Sozaeva L;Lévy R;James A;Schmitt MM;Ochoa S;Kareva M;Rodina Y;Gervais A;Le Voyer T;Rosain J;Philippot Q;Neehus AL;Shaw E;Migaud M;Bizien L;Ekwall O;Berg S;Beccuti G;Ghizzoni L;Thiriez G;Pavot A;Goujard C;Frémond ML;Carter E;Rothenbuhler A;Linglart A;Mignot B;Comte A;Cheikh N;Hermine O;Breivik L;Husebye ES;Humbert S;Rohrlich P;Coaquette A;Vuoto F;Faure K;Mahlaoui N;Kotnik P;Battelino T;Trebušak Podkrajšek K;Kisand K;Ferré EMN;DiMaggio T;Rosen LB;Burbelo PD;McIntyre M;Kann NY;Shcherbina A;Pavlova M;Kolodkina A;Holland SM;Zhang SY;Crow YJ;Notarangelo LD;Su HC;Abel L;Anderson MS;Jouanguy E;Neven B;Puel A;Casanova JL;Lionakis MS
自身免疫性多内分泌综合征-1(APS-1)患者有循环中和大多数I型干扰素的自身抗体。这些自身抗体可能是普通人群中危及生命的新冠肺炎肺炎的基础。作者报告了22名感染SARS-CoV-2的APS-1患者，其中15人(68%)发展为危及生命的疾病。AIRE双等位功能丧失变种的患者患有1型自身免疫性多内分泌综合征(APS-1)，并产生广泛的自身抗体(Auto-Abs)，包括循环中和大多数I型干扰素(IFN)的Auto-Abbs。最近有报道称，在普通人群中，这些自身抗体至少占危及生命的新冠肺炎肺炎病例的10%。我们报告了来自7个国家21个家庭的22例APS-1患者，年龄在8岁到48岁之间，自2020年2月以来感染了SARS-CoV-2。21例患者中和干扰素-α亚型和/或干扰素-ω，1例抗-干扰素-β和1例抗-干扰素-ε，但无一例抗-干扰素-κ。引人注目的是，19名患者(86%)因新冠肺炎肺炎入院，其中15人(68%)住进重症监护病房，11人(50%)需要机械通气，4人(18%)死亡。3名患者(14%)的非卧床疾病可能是由先前或早期的特定干预引起的。AP-1患者中预先存在的自身抗体中和I型IFN在任何年龄都有非常高的患危及生命的新冠肺炎肺炎的风险。
Patients with autoimmune polyendocrine syndrome type-1 (APS-1) have circulating auto-Abs neutralizing most type I interferons. These auto-Abs can underlie life-threatening COVID-19 pneumonia in the general population. The authors report 22 APS-1 patients infected with SARS-CoV-2, including 15 (68%) who developed life-threatening disease. Patients with biallelic loss-of-function variants of AIRE suffer from autoimmune polyendocrine syndrome type-1 (APS-1) and produce a broad range of autoantibodies (auto-Abs), including circulating auto-Abs neutralizing most type I interferons (IFNs). These auto-Abs were recently reported to account for at least 10% of cases of life-threatening COVID-19 pneumonia in the general population. We report 22 APS-1 patients from 21 kindreds in seven countries, aged between 8 and 48 yr and infected with SARS-CoV-2 since February 2020. The 21 patients tested had auto-Abs neutralizing IFN-α subtypes and/or IFN-ω; one had anti–IFN-β and another anti–IFN-ε, but none had anti–IFN-κ. Strikingly, 19 patients (86%) were hospitalized for COVID-19 pneumonia, including 15 (68%) admitted to an intensive care unit, 11 (50%) who required mechanical ventilation, and four (18%) who died. Ambulatory disease in three patients (14%) was possibly accounted for by prior or early specific interventions. Preexisting auto-Abs neutralizing type I IFNs in APS-1 patients confer a very high risk of life-threatening COVID-19 pneumonia at any age.
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DOI：
10.1056/nejmoa2029849
发表时间：
2021-01-21
期刊：
The New England journal of medicine
影响因子：
--
作者：
Chen P;Nirula A;Heller B;Gottlieb RL;Boscia J;Morris J;Huhn G;Cardona J;Mocherla B;Stosor V;Shawa I;Adams AC;Van Naarden J;Custer KL;Shen L;Durante M;Oakley G;Schade AE;Sabo J;Patel DR;Klekotka P;Skovronsky DM;BLAZE-1 Investigators
通讯作者：
BLAZE-1 Investigators
影响因子：
16.8
作者：
Hoffmann HH;Schneider WM;Rice CM
通讯作者：
Rice CM
DOI：
10.1111/nyas.12960
发表时间：
2015-01-01
期刊：
YEAR IN IMMUNOLOGY
影响因子：
--
作者：
Chan, Alice Y.;Anderson, Mark S.
通讯作者：
Anderson, Mark S.
影响因子：
8
作者：
Ferre, Elise M. N.;Rose, Stacey R.;Lionakis, Michail S.
通讯作者：
Lionakis, Michail S.
1型自身免疫性多腺体综合征（APS-1）的免疫生物学和临床特征
影响因子：
13.6
作者：
Guo, Can-Jie;Leung, Patrick S. C.;Gershwin, M. Eric
通讯作者：
Gershwin, M. Eric