The DOI matches its Crossref record; 60 of the 60 references it cites were found in a registry or an index.
Preexisting autoantibodies to type I IFNs underlie critical COVID-19 pneumonia in patients with APS-1
Open accessAbstract
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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Cites
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Metadata
- Authors
- Bastard, P., Orlova, E., Sozaeva, L., Lévy, R., James, A., Schmitt, M. M., Ochoa, S., Kareva, M., Rodina, Y., Gervais, A., Le Voyer, T., Rosain, J., Philippot, Q., Neehus, A.-L., Shaw, E., Migaud, M., Bizien, L., Ekwall, O., Berg, S., Beccuti, G., Ghizzoni, L., Thiriez, G., Pavot, A., Goujard, C., Frémond, M.-L., Carter, E., Rothenbuhler, A., Linglart, A., Mignot, B., Comte, A., Cheikh, N., Hermine, O., Breivik, L., Husebye, E. S., Humbert, S., Rohrlich, P., Coaquette, A., Vuoto, F., Faure, K., Mahlaoui, N., Kotnik, P., Battelino, T., Trebušak Podkrajšek, K., Kisand, K., Ferré, E. M. N., DiMaggio, T., Rosen, L. B., Burbelo, P. D., McIntyre, M., Kann, N. Y., Shcherbina, A., Pavlova, M., Kolodkina, A., Holland, S. M., Zhang, S.-Y., Crow, Y. J., Notarangelo, L. D., Su, H. C., Abel, L., Anderson, M. S., Jouanguy, E., Neven, B., Puel, A., Casanova, J.-L., & Lionakis, M. S.
- Venue
- Journal of Experimental Medicine
- Publisher
- Rockefeller University Press
- Published
- 2021-04-23
- Volume
- 218
- Issue
- 7
- ISSN
- 0022-1007 (ISSN-L)
- 1540-9538
- OpenAlex
- W3155911740
- Cited by
- 299
- References
- 60
- FWCI
- 18.97
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Bastard, P., Orlova, E., Sozaeva, L., Lévy, R., James, A., Schmitt, M. M., Ochoa, S., Kareva, M., Rodina, Y., Gervais, A., Le Voyer, T., Rosain, J., Philippot, Q., Neehus, A.-L., Shaw, E., Migaud, M., Bizien, L., Ekwall, O., Berg, S., . . . Lionakis, M. S. (2021). Preexisting autoantibodies to type I IFNs underlie critical COVID-19 pneumonia in patients with APS-1. Journal of Experimental Medicine. https://doi.org/10.1084/jem.20210554
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