tetano
Editor, Senior Moderator
J Allergy Clin Immunol
. 2020 Sep 24;S0091-6749(20)31320-8.
doi: 10.1016/j.jaci.2020.09.010. Online ahead of print.
Coronavirus Disease 2019 in patients with inborn errors of immunity: an international study
Isabelle Meyts[SUP] 1 [/SUP], Giorgia Bucciol[SUP] 2 [/SUP], Isabella Quinti[SUP] 3 [/SUP], B?n?dicte Neven[SUP] 4 [/SUP], Alain Fischer[SUP] 5 [/SUP], Elena Seoane[SUP] 6 [/SUP], Eduardo Lopez-Granados[SUP] 7 [/SUP], Carla Gianelli[SUP] 7 [/SUP], Angel Robles-Marhuenda[SUP] 7 [/SUP], Pierre-Yves Jeandel[SUP] 8 [/SUP], Catherine Paillard[SUP] 9 [/SUP], Vijay G Sankaran[SUP] 10 [/SUP], Yesim Yilmaz Demirdag[SUP] 11 [/SUP], Vassilios Lougaris[SUP] 12 [/SUP], Alessandro Aiuti[SUP] 13 [/SUP], Alessandro Plebani[SUP] 12 [/SUP], Cinzia Milito[SUP] 3 [/SUP], Virgil Ash Dalm[SUP] 14 [/SUP], Kissy Guevara-Hoyer[SUP] 15 [/SUP], Silvia S?nchez-Ram?n[SUP] 15 [/SUP], Liliana Bezrodnik[SUP] 16 [/SUP], Federica Barzaghi[SUP] 17 [/SUP], Luis Ignacio Gonzalez-Granado[SUP] 18 [/SUP], Grant R Hayman[SUP] 19 [/SUP], Gulbu Uzel[SUP] 20 [/SUP], Leonardo Oliveira Mendon?a[SUP] 21 [/SUP], Carlo Agostini[SUP] 22 [/SUP], Giuseppe Spadaro[SUP] 23 [/SUP], Raffaele Badolato[SUP] 24 [/SUP], Annarosa Soresina[SUP] 24 [/SUP], Fran?ois Vermeulen[SUP] 25 [/SUP], Cedric Bosteels[SUP] 26 [/SUP], Bart N Lambrecht[SUP] 26 [/SUP], Michael Keller[SUP] 27 [/SUP], Peter J Mustillo[SUP] 28 [/SUP], Roshini S Abraham[SUP] 29 [/SUP], Sudhir Gupta[SUP] 11 [/SUP], Ahmet Ozen[SUP] 30 [/SUP], Elif Karakoc-Aydiner[SUP] 30 [/SUP], Safa Baris[SUP] 30 [/SUP], Alexandra Freeman[SUP] 20 [/SUP], Marco Yamazaki-Nakashimada[SUP] 31 [/SUP], Selma Scheffler-Mendoza[SUP] 31 [/SUP], Sara Espinosa-Padilla[SUP] 31 [/SUP], Andrew R Gennery[SUP] 32 [/SUP], Stephen Jolles[SUP] 33 [/SUP], Yasmin Espinoza[SUP] 34 [/SUP], M Cecilia Poli[SUP] 34 [/SUP], Claire Fieschi[SUP] 35 [/SUP], Fabian Hauck[SUP] 36 [/SUP], Charlotte Cunningham-Rundles[SUP] 37 [/SUP], Nizar Mahlaoui[SUP] 38 [/SUP], IUIS Committee of Inborn Errors of Immunity; Klaus Warnatz[SUP] 39 [/SUP], Kathleen E Sullivan[SUP] 40 [/SUP], Stuart G Tangye[SUP] 41 [/SUP]
Affiliations
Abstract
Background: There is uncertainty about the impact of SARS-CoV-2 infection in individuals with rare inborn errors of immunity (IEI), a population at risk of developing severe COVID-19. This is relevant not only for these patients but also the general population, as studies of IEIs can unveil key requirements for host defense.
Objective: 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 IEI patients with SARS-CoV-2 infection. Median age was 25-34 years. 53 patients (56%) suffered from primary antibody deficiency, 9 (9.6%) had immune dysregulation syndrome, 6 (6.4%) a phagocyte defect, 7 (7.4%) auto-inflammatory 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 non-invasive ventilation or oxygen administration, 18 were admitted to intensive care units, 12 requiring invasive ventilation, and 3 extra corporeal membrane oxygenation. Nine patients (seven adults, two children) died.
Conclusions: This study demonstrates that (1) >30% of IEI patients had mild COVID19, and (2) risk factors predisposing to severe disease/mortality in the general population also seemed to affect IEI patients, including more younger patients. Further studies will identify pathways that are associated with increased risk of severe disease and are non-redundant or redundant for protection against SARS-CoV-2.
Keywords: COVID19; SARS CoV2; hypogammaglobulinemia; immune dysregulation; inborn errors of immunity; primary immunodeficiencies.
. 2020 Sep 24;S0091-6749(20)31320-8.
doi: 10.1016/j.jaci.2020.09.010. Online ahead of print.
Coronavirus Disease 2019 in patients with inborn errors of immunity: an international study
Isabelle Meyts[SUP] 1 [/SUP], Giorgia Bucciol[SUP] 2 [/SUP], Isabella Quinti[SUP] 3 [/SUP], B?n?dicte Neven[SUP] 4 [/SUP], Alain Fischer[SUP] 5 [/SUP], Elena Seoane[SUP] 6 [/SUP], Eduardo Lopez-Granados[SUP] 7 [/SUP], Carla Gianelli[SUP] 7 [/SUP], Angel Robles-Marhuenda[SUP] 7 [/SUP], Pierre-Yves Jeandel[SUP] 8 [/SUP], Catherine Paillard[SUP] 9 [/SUP], Vijay G Sankaran[SUP] 10 [/SUP], Yesim Yilmaz Demirdag[SUP] 11 [/SUP], Vassilios Lougaris[SUP] 12 [/SUP], Alessandro Aiuti[SUP] 13 [/SUP], Alessandro Plebani[SUP] 12 [/SUP], Cinzia Milito[SUP] 3 [/SUP], Virgil Ash Dalm[SUP] 14 [/SUP], Kissy Guevara-Hoyer[SUP] 15 [/SUP], Silvia S?nchez-Ram?n[SUP] 15 [/SUP], Liliana Bezrodnik[SUP] 16 [/SUP], Federica Barzaghi[SUP] 17 [/SUP], Luis Ignacio Gonzalez-Granado[SUP] 18 [/SUP], Grant R Hayman[SUP] 19 [/SUP], Gulbu Uzel[SUP] 20 [/SUP], Leonardo Oliveira Mendon?a[SUP] 21 [/SUP], Carlo Agostini[SUP] 22 [/SUP], Giuseppe Spadaro[SUP] 23 [/SUP], Raffaele Badolato[SUP] 24 [/SUP], Annarosa Soresina[SUP] 24 [/SUP], Fran?ois Vermeulen[SUP] 25 [/SUP], Cedric Bosteels[SUP] 26 [/SUP], Bart N Lambrecht[SUP] 26 [/SUP], Michael Keller[SUP] 27 [/SUP], Peter J Mustillo[SUP] 28 [/SUP], Roshini S Abraham[SUP] 29 [/SUP], Sudhir Gupta[SUP] 11 [/SUP], Ahmet Ozen[SUP] 30 [/SUP], Elif Karakoc-Aydiner[SUP] 30 [/SUP], Safa Baris[SUP] 30 [/SUP], Alexandra Freeman[SUP] 20 [/SUP], Marco Yamazaki-Nakashimada[SUP] 31 [/SUP], Selma Scheffler-Mendoza[SUP] 31 [/SUP], Sara Espinosa-Padilla[SUP] 31 [/SUP], Andrew R Gennery[SUP] 32 [/SUP], Stephen Jolles[SUP] 33 [/SUP], Yasmin Espinoza[SUP] 34 [/SUP], M Cecilia Poli[SUP] 34 [/SUP], Claire Fieschi[SUP] 35 [/SUP], Fabian Hauck[SUP] 36 [/SUP], Charlotte Cunningham-Rundles[SUP] 37 [/SUP], Nizar Mahlaoui[SUP] 38 [/SUP], IUIS Committee of Inborn Errors of Immunity; Klaus Warnatz[SUP] 39 [/SUP], Kathleen E Sullivan[SUP] 40 [/SUP], Stuart G Tangye[SUP] 41 [/SUP]
Affiliations
- PMID: 32980424
- DOI: 10.1016/j.jaci.2020.09.010
Abstract
Background: There is uncertainty about the impact of SARS-CoV-2 infection in individuals with rare inborn errors of immunity (IEI), a population at risk of developing severe COVID-19. This is relevant not only for these patients but also the general population, as studies of IEIs can unveil key requirements for host defense.
Objective: 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 IEI patients with SARS-CoV-2 infection. Median age was 25-34 years. 53 patients (56%) suffered from primary antibody deficiency, 9 (9.6%) had immune dysregulation syndrome, 6 (6.4%) a phagocyte defect, 7 (7.4%) auto-inflammatory 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 non-invasive ventilation or oxygen administration, 18 were admitted to intensive care units, 12 requiring invasive ventilation, and 3 extra corporeal membrane oxygenation. Nine patients (seven adults, two children) died.
Conclusions: This study demonstrates that (1) >30% of IEI patients had mild COVID19, and (2) risk factors predisposing to severe disease/mortality in the general population also seemed to affect IEI patients, including more younger patients. Further studies will identify pathways that are associated with increased risk of severe disease and are non-redundant or redundant for protection against SARS-CoV-2.
Keywords: COVID19; SARS CoV2; hypogammaglobulinemia; immune dysregulation; inborn errors of immunity; primary immunodeficiencies.