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Coronavirus disease 2019 in patients with inborn errors of immunity : an international study

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Abstract
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 (COVID19) 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.
Keywords
Immunology, Immunology and Allergy, SARS-CoV-2, COVID-19, primary immunodeficiencies, inborn errors of immunity, hypogammaglobulinemia, immune dysregulation

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MLA
Meyts, Isabelle, et al. “Coronavirus Disease 2019 in Patients with Inborn Errors of Immunity : An International Study.” JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, vol. 147, no. 5, 2021, pp. 520–31, doi:10.1016/j.jaci.2020.09.010.
APA
Meyts, I., Bucciol, G., Quinti, I., Neven, B., Fischer, A., Seoane, E., … Tangye, S. G. (2021). Coronavirus disease 2019 in patients with inborn errors of immunity : an international study. JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, 147(5), 520–531. https://doi.org/10.1016/j.jaci.2020.09.010
Chicago author-date
Meyts, Isabelle, Giorgia Bucciol, Isabella Quinti, Bénédicte Neven, Alain Fischer, Elena Seoane, Eduardo Lopez-Granados, et al. 2021. “Coronavirus Disease 2019 in Patients with Inborn Errors of Immunity : An International Study.” JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY 147 (5): 520–31. https://doi.org/10.1016/j.jaci.2020.09.010.
Chicago author-date (all authors)
Meyts, Isabelle, Giorgia Bucciol, Isabella Quinti, Bénédicte Neven, Alain Fischer, Elena Seoane, Eduardo Lopez-Granados, Carla Gianelli, Angel Robles-Marhuenda, Pierre-Yves Jeandel, Catherine Paillard, Vijay G. Sankaran, Yesim Yilmaz Demirdag, Vassilios Lougaris, Alessandro Aiuti, Alessandro Plebani, Cinzia Milito, Virgil ASH. Dalm, Kissy Guevara-Hoyer, Silvia Sánchez-Ramón, Liliana Bezrodnik, Federica Barzaghi, Luis Ignacio Gonzalez-Granado, Grant R. Hayman, Gulbu Uzel, Leonardo Oliveira Mendonça, Carlo Agostini, Giuseppe Spadaro, Raffaele Badolato, Annarosa Soresina, François Vermeulen, Cedric Bosteels, Bart Lambrecht, Michael Keller, Peter J. Mustillo, Roshini S. Abraham, Sudhir Gupta, Ahmet Ozen, Elif Karakoc-Aydiner, Safa Baris, Alexandra Freeman, Marco Yamazaki-Nakashimada, Selma Scheffler-Mendoza, Sara Espinosa-Padilla, Andrew R. Gennery, Stephen Jolles, Yasmin Espinoza, M Cecilia Poli, Claire Fieschi, Fabian Hauck, Charlotte Cunningham-Rundles, Nizar Mahlaoui, Klaus Warnatz, Kathleen E. Sullivan, and Stuart G. Tangye. 2021. “Coronavirus Disease 2019 in Patients with Inborn Errors of Immunity : An International Study.” JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY 147 (5): 520–531. doi:10.1016/j.jaci.2020.09.010.
Vancouver
1.
Meyts I, Bucciol G, Quinti I, Neven B, Fischer A, Seoane E, et al. Coronavirus disease 2019 in patients with inborn errors of immunity : an international study. JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY. 2021;147(5):520–31.
IEEE
[1]
I. Meyts et al., “Coronavirus disease 2019 in patients with inborn errors of immunity : an international study,” JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, vol. 147, no. 5, pp. 520–531, 2021.
@article{8676342,
  abstract     = {{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 (COVID19) 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.}},
  author       = {{Meyts, Isabelle and Bucciol, Giorgia and Quinti, Isabella and Neven, Bénédicte and Fischer, Alain and Seoane, Elena and Lopez-Granados, Eduardo and Gianelli, Carla and Robles-Marhuenda, Angel and Jeandel, Pierre-Yves and Paillard, Catherine and Sankaran, Vijay G. and Demirdag, Yesim Yilmaz and Lougaris, Vassilios and Aiuti, Alessandro and Plebani, Alessandro and Milito, Cinzia and Dalm, Virgil ASH. and Guevara-Hoyer, Kissy and Sánchez-Ramón, Silvia and Bezrodnik, Liliana and Barzaghi, Federica and Gonzalez-Granado, Luis Ignacio and Hayman, Grant R. and Uzel, Gulbu and Mendonça, Leonardo Oliveira and Agostini, Carlo and Spadaro, Giuseppe and Badolato, Raffaele and Soresina, Annarosa and Vermeulen, François and Bosteels, Cedric and Lambrecht, Bart and Keller, Michael and Mustillo, Peter J. and Abraham, Roshini S. and Gupta, Sudhir and Ozen, Ahmet and Karakoc-Aydiner, Elif and Baris, Safa and Freeman, Alexandra and Yamazaki-Nakashimada, Marco and Scheffler-Mendoza, Selma and Espinosa-Padilla, Sara and Gennery, Andrew R. and Jolles, Stephen and Espinoza, Yasmin and Poli, M Cecilia and Fieschi, Claire and Hauck, Fabian and Cunningham-Rundles, Charlotte and Mahlaoui, Nizar and Warnatz, Klaus and Sullivan, Kathleen E. and Tangye, Stuart G.}},
  issn         = {{0091-6749}},
  journal      = {{JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY}},
  keywords     = {{Immunology,Immunology and Allergy,SARS-CoV-2,COVID-19,primary immunodeficiencies,inborn errors of immunity,hypogammaglobulinemia,immune dysregulation}},
  language     = {{eng}},
  number       = {{5}},
  pages        = {{520--531}},
  title        = {{Coronavirus disease 2019 in patients with inborn errors of immunity : an international study}},
  url          = {{http://dx.doi.org/10.1016/j.jaci.2020.09.010}},
  volume       = {{147}},
  year         = {{2021}},
}

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