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

I Meyts; G Bucciol; I Quinti; B Neven; A Fischer; E Seoane; E Lopez-Grandos; C Gianelli; A Robles-Marhuenda; PY Jeandel; C Paillard; V G Sankaran; YY Demirdag; V Lougaris; A Aiuti; A Plebani; C Milito; V.A.S.H. Dalm; K Guevara-Hoyer; S Sanchez-Ramon; L Bezrodnik; F Barzaghi; LI Gonzalez-Granado; GR Hayman; G Uzel; LO Mendonca; C Agostini; G Spadaro; R Badolato; A Soresina; F Vermeulen; C Bosteels; BNM Lambrecht; M Keller; PJ Mustillo; RS Abraham; S Gupta; A Ozen; E Karakoc-Aydiner; S Baris; AF Freeman; M Yamazaki-Nakashimada; S Scheffler-Mendoza; S Espinosa-Padilla; AR Gennery; S Jolles; Y Espinosa; MC Poli; C Fieschi; F Hauck; C Cunningham-Rundles; N Mahlaoui; K Warnatz; KE Sullivan; SG Tangye

doi:10.1016/j.jaci.2020.09.010

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

I Meyts, G Bucciol, I Quinti, B Neven, A Fischer, E Seoane, E Lopez-Grandos, C Gianelli, A Robles-Marhuenda, PY Jeandel, C Paillard, V G Sankaran, YY Demirdag, V Lougaris, A Aiuti, A Plebani, C Milito, V.A.S.H. Dalm, K Guevara-Hoyer, S Sanchez-RamonL Bezrodnik, F Barzaghi, LI Gonzalez-Granado, GR Hayman, G Uzel, LO Mendonca, C Agostini, G Spadaro, R Badolato, A Soresina, F Vermeulen, C Bosteels, BNM Lambrecht, M Keller, PJ Mustillo, RS Abraham, S Gupta, A Ozen, E Karakoc-Aydiner, S Baris, AF Freeman, M Yamazaki-Nakashimada, S Scheffler-Mendoza, S Espinosa-Padilla, AR Gennery, S Jolles, Y Espinosa, MC Poli, C Fieschi, F Hauck, C Cunningham-Rundles, N Mahlaoui, K Warnatz, KE Sullivan, SG Tangye

Internal Medicine

Research output: Contribution to journal › Article › Academic › peer-review

290 Citations (Scopus)

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 (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.

Original language	English
Pages (from-to)	520-531
Number of pages	12
Journal	Journal of Allergy and Clinical Immunology
Volume	147
Issue number	2
DOIs	https://doi.org/10.1016/j.jaci.2020.09.010
Publication status	Published - 1 Feb 2021

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Meyts, I., Bucciol, G., Quinti, I., Neven, B., Fischer, A., Seoane, E., Lopez-Grandos, E., Gianelli, C., Robles-Marhuenda, A., Jeandel, PY., Paillard, C., Sankaran, V. G., Demirdag, YY., Lougaris, V., Aiuti, A., Plebani, A., Milito, C., Dalm, V. A. S. H., Guevara-Hoyer, K., ... Tangye, SG. (2021). Coronavirus disease 2019 in patients with inborn errors of immunity: An international study. Journal of Allergy and Clinical Immunology, 147(2), 520-531. https://doi.org/10.1016/j.jaci.2020.09.010

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title = "Coronavirus disease 2019 in patients with inborn errors of immunity: An international study",

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 (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.",

author = "I Meyts and G Bucciol and I Quinti and B Neven and A Fischer and E Seoane and E Lopez-Grandos and C Gianelli and A Robles-Marhuenda and PY Jeandel and C Paillard and Sankaran, {V G} and YY Demirdag and V Lougaris and A Aiuti and A Plebani and C Milito and V.A.S.H. Dalm and K Guevara-Hoyer and S Sanchez-Ramon and L Bezrodnik and F Barzaghi and LI Gonzalez-Granado and GR Hayman and G Uzel and LO Mendonca and C Agostini and G Spadaro and R Badolato and A Soresina and F Vermeulen and C Bosteels and BNM Lambrecht and M Keller and PJ Mustillo and RS Abraham and S Gupta and A Ozen and E Karakoc-Aydiner and S Baris and AF Freeman and M Yamazaki-Nakashimada and S Scheffler-Mendoza and S Espinosa-Padilla and AR Gennery and S Jolles and Y Espinosa and MC Poli and C Fieschi and F Hauck and C Cunningham-Rundles and N Mahlaoui and K Warnatz and KE Sullivan and SG Tangye",

year = "2021",

month = feb,

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doi = "10.1016/j.jaci.2020.09.010",

language = "English",

volume = "147",

pages = "520--531",

journal = "Journal of Allergy and Clinical Immunology",

issn = "0091-6749",

publisher = "Elsevier Inc.",

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Meyts, I, Bucciol, G, Quinti, I, Neven, B, Fischer, A, Seoane, E, Lopez-Grandos, E, Gianelli, C, Robles-Marhuenda, A, Jeandel, PY, Paillard, C, Sankaran, VG, Demirdag, YY, Lougaris, V, Aiuti, A, Plebani, A, Milito, C, Dalm, VASH, Guevara-Hoyer, K, Sanchez-Ramon, S, Bezrodnik, L, Barzaghi, F, Gonzalez-Granado, LI, Hayman, GR, Uzel, G, Mendonca, LO, Agostini, C, Spadaro, G, Badolato, R, Soresina, A, Vermeulen, F, Bosteels, C, Lambrecht, BNM, Keller, M, Mustillo, PJ, Abraham, RS, Gupta, S, Ozen, A, Karakoc-Aydiner, E, Baris, S, Freeman, AF, Yamazaki-Nakashimada, M, Scheffler-Mendoza, S, Espinosa-Padilla, S, Gennery, AR, Jolles, S, Espinosa, Y, Poli, MC, Fieschi, C, Hauck, F, Cunningham-Rundles, C, Mahlaoui, N, Warnatz, K, Sullivan, KE & Tangye, SG 2021, 'Coronavirus disease 2019 in patients with inborn errors of immunity: An international study', Journal of Allergy and Clinical Immunology, vol. 147, no. 2, pp. 520-531. https://doi.org/10.1016/j.jaci.2020.09.010

TY - JOUR

T1 - Coronavirus disease 2019 in patients with inborn errors of immunity: An international study

AU - Meyts, I

AU - Bucciol, G

AU - Quinti, I

AU - Neven, B

AU - Fischer, A

AU - Seoane, E

AU - Lopez-Grandos, E

AU - Gianelli, C

AU - Robles-Marhuenda, A

AU - Jeandel, PY

AU - Paillard, C

AU - Sankaran, V G

AU - Demirdag, YY

AU - Lougaris, V

AU - Aiuti, A

AU - Plebani, A

AU - Milito, C

AU - Dalm, V.A.S.H.

AU - Guevara-Hoyer, K

AU - Sanchez-Ramon, S

AU - Bezrodnik, L

AU - Barzaghi, F

AU - Gonzalez-Granado, LI

AU - Hayman, GR

AU - Uzel, G

AU - Mendonca, LO

AU - Agostini, C

AU - Spadaro, G

AU - Badolato, R

AU - Soresina, A

AU - Vermeulen, F

AU - Bosteels, C

AU - Lambrecht, BNM

AU - Keller, M

AU - Mustillo, PJ

AU - Abraham, RS

AU - Gupta, S

AU - Ozen, A

AU - Karakoc-Aydiner, E

AU - Baris, S

AU - Freeman, AF

AU - Yamazaki-Nakashimada, M

AU - Scheffler-Mendoza, S

AU - Espinosa-Padilla, S

AU - Gennery, AR

AU - Jolles, S

AU - Espinosa, Y

AU - Poli, MC

AU - Fieschi, C

AU - Hauck, F

AU - Cunningham-Rundles, C

AU - Mahlaoui, N

AU - Warnatz, K

AU - Sullivan, KE

AU - Tangye, SG

PY - 2021/2/1

Y1 - 2021/2/1

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.

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U2 - 10.1016/j.jaci.2020.09.010

DO - 10.1016/j.jaci.2020.09.010

M3 - Article

C2 - 32980424

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 -

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

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