Coronavirus disease 2019 in patients with inborn errors of immunity: An international study.
COVID-19
SARS-CoV-2
hypogammaglobulinemia
immune dysregulation
inborn errors of immunity
primary immunodeficiencies
Journal
The Journal of allergy and clinical immunology
ISSN: 1097-6825
Titre abrégé: J Allergy Clin Immunol
Pays: United States
ID NLM: 1275002
Informations de publication
Date de publication:
Feb 2021
Feb 2021
Historique:
received:
27
07
2020
revised:
18
09
2020
accepted:
21
09
2020
pubmed:
28
9
2020
medline:
16
2
2021
entrez:
27
9
2020
Statut:
ppublish
Résumé
There is uncertainty about the impact of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in individuals with rare inborn errors of immunity (IEI), a population at risk of developing severe coronavirus disease 2019. This is relevant not only for these patients but also for the general population, because studies of IEIs can unveil key requirements for host defense. We sought to describe the presentation, manifestations, and outcome of SARS-CoV-2 infection in IEI to inform physicians and enhance understanding of host defense against SARS-CoV-2. An invitation to participate in a retrospective study was distributed globally to scientific, medical, and patient societies involved in the care and advocacy for patients with IEI. We gathered information on 94 patients with IEI with SARS-CoV-2 infection. Their median age was 25 to 34 years. Fifty-three patients (56%) suffered from primary antibody deficiency, 9 (9.6%) had immune dysregulation syndrome, 6 (6.4%) a phagocyte defect, 7 (7.4%) an autoinflammatory disorder, 14 (15%) a combined immunodeficiency, 3 (3%) an innate immune defect, and 2 (2%) bone marrow failure. Ten were asymptomatic, 25 were treated as outpatients, 28 required admission without intensive care or ventilation, 13 required noninvasive ventilation or oxygen administration, 18 were admitted to intensive care units, 12 required invasive ventilation, and 3 required extracorporeal membrane oxygenation. Nine patients (7 adults and 2 children) died. This study demonstrates that (1) more than 30% of patients with IEI had mild coronavirus disease 2019 (COVID-19) and (2) risk factors predisposing to severe disease/mortality in the general population also seemed to affect patients with IEI, including more younger patients. Further studies will identify pathways that are associated with increased risk of severe disease and are nonredundant or redundant for protection against SARS-CoV-2.
Sections du résumé
BACKGROUND
BACKGROUND
There is uncertainty about the impact of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in individuals with rare inborn errors of immunity (IEI), a population at risk of developing severe coronavirus disease 2019. This is relevant not only for these patients but also for the general population, because studies of IEIs can unveil key requirements for host defense.
OBJECTIVE
OBJECTIVE
We sought to describe the presentation, manifestations, and outcome of SARS-CoV-2 infection in IEI to inform physicians and enhance understanding of host defense against SARS-CoV-2.
METHODS
METHODS
An invitation to participate in a retrospective study was distributed globally to scientific, medical, and patient societies involved in the care and advocacy for patients with IEI.
RESULTS
RESULTS
We gathered information on 94 patients with IEI with SARS-CoV-2 infection. Their median age was 25 to 34 years. Fifty-three patients (56%) suffered from primary antibody deficiency, 9 (9.6%) had immune dysregulation syndrome, 6 (6.4%) a phagocyte defect, 7 (7.4%) an autoinflammatory disorder, 14 (15%) a combined immunodeficiency, 3 (3%) an innate immune defect, and 2 (2%) bone marrow failure. Ten were asymptomatic, 25 were treated as outpatients, 28 required admission without intensive care or ventilation, 13 required noninvasive ventilation or oxygen administration, 18 were admitted to intensive care units, 12 required invasive ventilation, and 3 required extracorporeal membrane oxygenation. Nine patients (7 adults and 2 children) died.
CONCLUSIONS
CONCLUSIONS
This study demonstrates that (1) more than 30% of patients with IEI had mild coronavirus disease 2019 (COVID-19) and (2) risk factors predisposing to severe disease/mortality in the general population also seemed to affect patients with IEI, including more younger patients. Further studies will identify pathways that are associated with increased risk of severe disease and are nonredundant or redundant for protection against SARS-CoV-2.
Identifiants
pubmed: 32980424
pii: S0091-6749(20)31320-8
doi: 10.1016/j.jaci.2020.09.010
pmc: PMC7832563
pii:
doi:
Types de publication
Journal Article
Multicenter Study
Langues
eng
Sous-ensembles de citation
IM
Pagination
520-531Subventions
Organisme : NIAID NIH HHS
ID : P01 AI061093
Pays : United States
Informations de copyright
Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.
Références
N Engl J Med. 2020 Mar 26;382(13):1199-1207
pubmed: 31995857
Cell. 2020 Jun 11;181(6):1194-1199
pubmed: 32405102
Pediatr Allergy Immunol. 2020 Jul;31(5):565-569
pubmed: 32319118
J Allergy Clin Immunol. 2020 Jul;146(1):211-213.e4
pubmed: 32333914
Intensive Care Med. 2003 Apr;29(4):530-8
pubmed: 12664219
Euro Surveill. 2020 Mar;25(12):
pubmed: 32234121
Science. 2020 Oct 23;370(6515):
pubmed: 32972996
J Allergy Clin Immunol. 2020 Jul;146(1):128-136.e4
pubmed: 32425269
J Clin Immunol. 2020 Jan;40(1):66-81
pubmed: 32048120
EMBO Mol Med. 2020 Jul 7;12(7):e12421
pubmed: 32428990
JAMA Intern Med. 2020 Jul 1;180(7):934-943
pubmed: 32167524
Immunity. 2020 Sep 15;53(3):487-495
pubmed: 32853545
Pediatr Blood Cancer. 2020 Sep;67(9):e28382
pubmed: 32495391
J Clin Invest. 2020 May 1;130(5):2620-2629
pubmed: 32217835
J Clin Immunol. 2020 Aug;40(6):807-819
pubmed: 32572726
N Engl J Med. 2020 Jul 23;383(4):334-346
pubmed: 32598831
J Clin Immunol. 2020 Jan;40(1):24-64
pubmed: 31953710
JAMA. 2020 Apr 28;323(16):1574-1581
pubmed: 32250385
N Engl J Med. 2020 Feb 20;382(8):727-733
pubmed: 31978945
Sci Immunol. 2020 Jul 10;5(49):
pubmed: 32651211
Curr Opin Immunol. 2020 Feb;62:79-90
pubmed: 31935567
JAMA. 2020 Mar 17;323(11):1061-1069
pubmed: 32031570
Pediatr Allergy Immunol. 2021 Jan;32(1):199-201
pubmed: 32683750
Lancet. 2020 Mar 28;395(10229):1054-1062
pubmed: 32171076
J Allergy Clin Immunol. 2020 Jul;146(1):110-118
pubmed: 32294485
BMJ. 2020 Jun 3;369:m2094
pubmed: 32493739
Science. 2020 Oct 23;370(6515):
pubmed: 32972995
J Clin Immunol. 2020 Aug;40(6):791-806
pubmed: 32638194
Autoimmun Rev. 2020 Jul;19(7):102567
pubmed: 32376392
J Med Virol. 2020 Nov;92(11):2852-2856
pubmed: 32515499
Pediatr Blood Cancer. 2007 Feb;48(2):124-31
pubmed: 16937360
Lancet Infect Dis. 2020 Jun;20(6):669-677
pubmed: 32240634
Intensive Care Med. 2020 May;46(5):846-848
pubmed: 32125452
Blood. 2006 Mar 15;107(6):2423-31
pubmed: 16293610
Hum Genet. 2020 Jun;139(6-7):941-948
pubmed: 32025908
Lancet. 2020 Feb 15;395(10223):497-506
pubmed: 31986264
J Clin Immunol. 2018 May;38(4):513-526
pubmed: 29882021
J Allergy Clin Immunol Pract. 2020 Sep;8(8):2793-2795
pubmed: 32652231
J Allergy Clin Immunol. 2014 Jun;133(6):1644-50.e4
pubmed: 24612681
MMWR Morb Mortal Wkly Rep. 2020 Jun 19;69(24):759-765
pubmed: 32555134
PLoS Med. 2006 Jul;3(7):e289
pubmed: 16784312