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10.1084/jem.20210554

http://scihub22266oqcxt.onion/10.1084/jem.20210554
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33890986!8077172!33890986
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suck abstract from ncbi


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pmid33890986      J+Exp+Med 2021 ; 218 (7): ä
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  • Preexisting autoantibodies to type I IFNs underlie critical COVID-19 pneumonia in patients with APS-1 #MMPMID33890986
  • Bastard P; Orlova E; Sozaeva L; Levy 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; Fremond 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; Trebusak Podkrajsek K; Kisand K; Ferre 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
  • J Exp Med 2021[Jul]; 218 (7): ä PMID33890986show ga
  • 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-alpha subtypes and/or IFN-omega; one had anti-IFN-beta and another anti-IFN-epsilon, but none had anti-IFN-kappa. 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.
  • |Adolescent[MESH]
  • |Adult[MESH]
  • |Autoantibodies/*immunology[MESH]
  • |COVID-19/*immunology[MESH]
  • |Child[MESH]
  • |Female[MESH]
  • |Humans[MESH]
  • |Interferon Type I/*immunology[MESH]
  • |Male[MESH]
  • |Middle Aged[MESH]
  • |Pneumonia/*immunology[MESH]
  • |Polyendocrinopathies, Autoimmune/*immunology[MESH]
  • |SARS-CoV-2/immunology[MESH]


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