Preexisting autoantibodies to type I IFNs underlie critical COVID-19 pneumonia in patients with APS-1.

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Bastard, Paul (author)

Preexisting autoantibodies to type I IFNs underlie critical COVID-19 pneumonia in patients with APS-1.

Article/chapterEnglish2021

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2021

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LIBRIS-ID:oai:gup.ub.gu.se/304354
https://gup.ub.gu.se/publication/304354URI
https://doi.org/10.1084/JEM.20210554DOI

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Language:English

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Subject category:ref swepub-contenttype
Subject category:art swepub-publicationtype

Notes

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.

Subject headings and genre

MEDICIN OCH HÄLSOVETENSKAP Klinisk medicin Annan klinisk medicin hsv//swe
MEDICAL AND HEALTH SCIENCES Clinical Medicine Other Clinical Medicine hsv//eng
MEDICIN OCH HÄLSOVETENSKAP Medicinska och farmaceutiska grundvetenskaper Immunologi inom det medicinska området hsv//swe
MEDICAL AND HEALTH SCIENCES Basic Medicine Immunology in the medical area hsv//eng
Adolescent
Adult
Autoantibodies
immunology
COVID-19
immunology
Child
Female
Humans
Interferon Type I
immunology
Male
Middle Aged
Pneumonia
immunology
Polyendocrinopathies
Autoimmune
immunology
SARS-CoV-2
immunology
Young Adult

Added entries (persons, corporate bodies, meetings, titles ...)

Orlova, Elizaveta (author)
Sozaeva, Leila (author)
Lévy, Romain (author)
James, Alyssa (author)
Schmitt, Monica M (author)
Ochoa, Sebastian (author)
Kareva, Maria (author)
Rodina, Yulia (author)
Gervais, Adrian (author)
Le Voyer, Tom (author)
Rosain, Jérémie (author)
Philippot, Quentin (author)
Neehus, Anna-Lena (author)
Shaw, Elana (author)
Migaud, Mélanie (author)
Bizien, Lucy (author)
Ekwall, Olov,1968Gothenburg University,Göteborgs universitet,Institutionen för kliniska vetenskaper, Avdelningen för pediatrik,Institutionen för medicin, avdelningen för reumatologi och inflammationsforskning,Institute of Clinical Sciences, Department of Pediatrics,Institute of Medicine, Department of Rheumatology and Inflammation Research(Swepub:gu)xekwol (author)
Berg, Stefan,1959Gothenburg University,Göteborgs universitet,Institutionen för kliniska vetenskaper, Avdelningen för pediatrik,Institute of Clinical Sciences, Department of Pediatrics(Swepub:gu)xbeste (author)
Beccuti, Guglielmo (author)
Ghizzoni, Lucia (author)
Thiriez, Gérard (author)
Pavot, Arthur (author)
Goujard, Cécile (author)
Frémond, Marie-Louise (author)
Carter, Edwin (author)
Rothenbuhler, Anya (author)
Linglart, Agnès (author)
Mignot, Brigite (author)
Comte, Aurélie (author)
Cheikh, Nathalie (author)
Hermine, Olivier (author)
Breivik, Lars (author)
Husebye, Eystein S (author)
Humbert, Sébastien (author)
Rohrlich, Pierre (author)
Coaquette, Alain (author)
Vuoto, Fanny (author)
Faure, Karine (author)
Mahlaoui, Nizar (author)
Kotnik, Primož (author)
Battelino, Tadej (author)
Trebušak Podkrajšek, Katarina (author)
Kisand, Kai (author)
Ferré, Elise M N (author)
DiMaggio, Thomas (author)
Rosen, Lindsey B (author)
Burbelo, Peter D (author)
McIntyre, Martin (author)
Kann, Nelli Y (author)
Shcherbina, Anna (author)
Pavlova, Maria (author)
Kolodkina, Anna (author)
Holland, Steven M (author)
Zhang, Shen-Ying (author)
Crow, Yanick J (author)
Notarangelo, Luigi D (author)
Su, Helen C (author)
Abel, Laurent (author)
Anderson, Mark S (author)
Jouanguy, Emmanuelle (author)
Neven, Bénédicte (author)
Puel, Anne (author)
Casanova, Jean-Laurent (author)
Lionakis, Michail S (author)
Göteborgs universitetInstitutionen för kliniska vetenskaper, Avdelningen för pediatrik (creator_code:org_t)

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By the author/editor: Bastard, Paul; Orlova, Elizavet ...; Sozaeva, Leila; Lévy, Romain; James, Alyssa; Schmitt, Monica ...; show more...; Ochoa, Sebastian; Kareva, Maria; Rodina, Yulia; Gervais, Adrian; Le Voyer, Tom; Rosain, Jérémie; Philippot, Quent ...; Neehus, Anna-Len ...; Shaw, Elana; Migaud, Mélanie; Bizien, Lucy; Ekwall, Olov, 19 ...; Berg, Stefan, 19 ...; Beccuti, Gugliel ...; Ghizzoni, Lucia; Thiriez, Gérard; Pavot, Arthur; Goujard, Cécile; Frémond, Marie-L ...; Carter, Edwin; Rothenbuhler, An ...; Linglart, Agnès; Mignot, Brigite; Comte, Aurélie; Cheikh, Nathalie; Hermine, Olivier; Breivik, Lars; Husebye, Eystein ...; Humbert, Sébasti ...; Rohrlich, Pierre; Coaquette, Alain; Vuoto, Fanny; Faure, Karine; Mahlaoui, Nizar; Kotnik, Primož; Battelino, Tadej; Trebušak Podkraj ...; Kisand, Kai; Ferré, Elise M N; DiMaggio, Thomas; Rosen, Lindsey B; Burbelo, Peter D; McIntyre, Martin; Kann, Nelli Y; Shcherbina, Anna; Pavlova, Maria; Kolodkina, Anna; Holland, Steven ...; Zhang, Shen-Ying; Crow, Yanick J; Notarangelo, Lui ...; Su, Helen C; Abel, Laurent; Anderson, Mark S; Jouanguy, Emmanu ...; Neven, Bénédicte; Puel, Anne; Casanova, Jean-L ...; Lionakis, Michai ...; show less...

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