TY - JOUR
T1 - Coronavirus disease 2019 in patients with inborn errors of immunity
T2 - An international study
AU - IUIS Committee of Inborn Errors of Immunity
AU - Meyts, Isabelle
AU - Bucciol, Giorgia
AU - Quinti, Isabella
AU - Neven, Bénédicte
AU - Fischer, Alain
AU - Seoane, Elena
AU - Lopez-Granados, Eduardo
AU - Gianelli, Carla
AU - Robles-Marhuenda, Angel
AU - Jeandel, Pierre Yves
AU - Paillard, Catherine
AU - Sankaran, Vijay G.
AU - Demirdag, Yesim Yilmaz
AU - Lougaris, Vassilios
AU - Aiuti, Alessandro
AU - Plebani, Alessandro
AU - Milito, Cinzia
AU - Dalm, Virgil ASH
AU - Guevara-Hoyer, Kissy
AU - Sánchez-Ramón, Silvia
AU - Bezrodnik, Liliana
AU - Barzaghi, Federica
AU - Gonzalez-Granado, Luis Ignacio
AU - Hayman, Grant R.
AU - Uzel, Gulbu
AU - Mendonça, Leonardo Oliveira
AU - Agostini, Carlo
AU - Spadaro, Giuseppe
AU - Badolato, Raffaele
AU - Soresina, Annarosa
AU - Vermeulen, François
AU - Bosteels, Cedric
AU - Lambrecht, Bart N.
AU - Keller, Michael
AU - Mustillo, Peter J.
AU - Abraham, Roshini S.
AU - Gupta, Sudhir
AU - Ozen, Ahmet
AU - Karakoc-Aydiner, Elif
AU - Baris, Safa
AU - Freeman, Alexandra F.
AU - Yamazaki-Nakashimada, Marco
AU - Scheffler-Mendoza, Selma
AU - Espinosa-Padilla, Sara
AU - Gennery, Andrew R.
AU - Jolles, Stephen
AU - Espinosa, Yazmin
AU - Poli, M. Cecilia
AU - Fieschi, Claire
AU - Cunningham-Rundles, Charlotte
N1 - Publisher Copyright:
© 2020 The Authors
PY - 2021/2
Y1 - 2021/2
N2 - 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: 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: 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: 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: 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.
AB - 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: 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: 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: 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: 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.
KW - COVID-19
KW - SARS-CoV-2
KW - hypogammaglobulinemia
KW - immune dysregulation
KW - inborn errors of immunity
KW - primary immunodeficiencies
UR - https://www.scopus.com/pages/publications/85092905201
U2 - 10.1016/j.jaci.2020.09.010
DO - 10.1016/j.jaci.2020.09.010
M3 - Article
C2 - 32980424
AN - SCOPUS:85092905201
SN - 0091-6749
VL - 147
SP - 520
EP - 531
JO - Journal of Allergy and Clinical Immunology
JF - Journal of Allergy and Clinical Immunology
IS - 2
ER -