Coronavirus disease 2019 in patients with inborn errors of immunity : an international study
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 a...
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| creator | Meyts, Isabelle Bucciol, Giorgia Quinti, Isabella Neven, Bénédicte Fischer, Alain Seoane, Elena Lopez-Granados, Eduardo Gianelli, Carla Robles-Marhuenda, Angel Jeandel, Pierre-Yves Paillard, Catherine Sankaran, Vijay G Demirdag, Yesim Yilmaz Lougaris, Vassilios Aiuti, Alessandro Plebani, Alessandro Milito, Cinzia Dalm, Virgil ASH Guevara-Hoyer, Kissy Sánchez-Ramón, Silvia Bezrodnik, Liliana Barzaghi, Federica Gonzalez-Granado, Luis Ignacio Hayman, Grant R Uzel, Gulbu Mendonça, Leonardo Oliveira Agostini, Carlo Spadaro, Giuseppe Badolato, Raffaele Soresina, Annarosa Vermeulen, François Bosteels, Cedric Lambrecht, Bart Keller, Michael Mustillo, Peter J Abraham, Roshini S Gupta, Sudhir Ozen, Ahmet Karakoc-Aydiner, Elif Baris, Safa Freeman, Alexandra Yamazaki-Nakashimada, Marco Scheffler-Mendoza, Selma Espinosa-Padilla, Sara Gennery, Andrew R Jolles, Stephen Espinoza, Yasmin Poli, M Cecilia Fieschi, Claire Hauck, Fabian Cunningham-Rundles, Charlotte Mahlaoui, Nizar Warnatz, Klaus Sullivan, Kathleen E Tangye, Stuart G |
| description | 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. |
| format | Article |
| fullrecord | <record><control><sourceid>ghent</sourceid><recordid>TN_cdi_ghent_librecat_oai_archive_ugent_be_8676342</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>oai_archive_ugent_be_8676342</sourcerecordid><originalsourceid>FETCH-ghent_librecat_oai_archive_ugent_be_86763423</originalsourceid><addsrcrecordid>eNqdjE0KwjAQRrNQsP7cYS5QiK3W1m1RPIBLIaQ62pGayExa8fZG8ASuPvge741UstTVJi3KbD1RU5G71rrKyypRp9qzd3Yg7gUuJGgFIdPLCsjB0wZCFwReFNp4NJ4dILNnAX8Fejx6R-ENW7Au4oDsohFzHUjoL--5Gl9tJ7j47Uxl-92xPqS3NmZNRw3j2QbjLRnL55YGNP3tixo0ZbEp8lWW_yV9AD31T4o</addsrcrecordid><sourcetype>Institutional Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype></control><display><type>article</type><title>Coronavirus disease 2019 in patients with inborn errors of immunity : an international study</title><source>Ghent University Academic Bibliography</source><source>Elsevier ScienceDirect Journals</source><creator>Meyts, Isabelle ; Bucciol, Giorgia ; Quinti, Isabella ; Neven, Bénédicte ; Fischer, Alain ; Seoane, Elena ; Lopez-Granados, Eduardo ; Gianelli, Carla ; Robles-Marhuenda, Angel ; Jeandel, Pierre-Yves ; Paillard, Catherine ; Sankaran, Vijay G ; Demirdag, Yesim Yilmaz ; Lougaris, Vassilios ; Aiuti, Alessandro ; Plebani, Alessandro ; Milito, Cinzia ; Dalm, Virgil ASH ; Guevara-Hoyer, Kissy ; Sánchez-Ramón, Silvia ; Bezrodnik, Liliana ; Barzaghi, Federica ; Gonzalez-Granado, Luis Ignacio ; Hayman, Grant R ; Uzel, Gulbu ; Mendonça, Leonardo Oliveira ; Agostini, Carlo ; Spadaro, Giuseppe ; Badolato, Raffaele ; Soresina, Annarosa ; Vermeulen, François ; Bosteels, Cedric ; Lambrecht, Bart ; Keller, Michael ; Mustillo, Peter J ; Abraham, Roshini S ; Gupta, Sudhir ; Ozen, Ahmet ; Karakoc-Aydiner, Elif ; Baris, Safa ; Freeman, Alexandra ; Yamazaki-Nakashimada, Marco ; Scheffler-Mendoza, Selma ; Espinosa-Padilla, Sara ; Gennery, Andrew R ; Jolles, Stephen ; Espinoza, Yasmin ; Poli, M Cecilia ; Fieschi, Claire ; Hauck, Fabian ; Cunningham-Rundles, Charlotte ; Mahlaoui, Nizar ; Warnatz, Klaus ; Sullivan, Kathleen E ; Tangye, Stuart G</creator><creatorcontrib>Meyts, Isabelle ; Bucciol, Giorgia ; Quinti, Isabella ; Neven, Bénédicte ; Fischer, Alain ; Seoane, Elena ; Lopez-Granados, Eduardo ; Gianelli, Carla ; Robles-Marhuenda, Angel ; Jeandel, Pierre-Yves ; Paillard, Catherine ; Sankaran, Vijay G ; Demirdag, Yesim Yilmaz ; Lougaris, Vassilios ; Aiuti, Alessandro ; Plebani, Alessandro ; Milito, Cinzia ; Dalm, Virgil ASH ; Guevara-Hoyer, Kissy ; Sánchez-Ramón, Silvia ; Bezrodnik, Liliana ; Barzaghi, Federica ; Gonzalez-Granado, Luis Ignacio ; Hayman, Grant R ; Uzel, Gulbu ; Mendonça, Leonardo Oliveira ; Agostini, Carlo ; Spadaro, Giuseppe ; Badolato, Raffaele ; Soresina, Annarosa ; Vermeulen, François ; Bosteels, Cedric ; Lambrecht, Bart ; Keller, Michael ; Mustillo, Peter J ; Abraham, Roshini S ; Gupta, Sudhir ; Ozen, Ahmet ; Karakoc-Aydiner, Elif ; Baris, Safa ; Freeman, Alexandra ; Yamazaki-Nakashimada, Marco ; Scheffler-Mendoza, Selma ; Espinosa-Padilla, Sara ; Gennery, Andrew R ; Jolles, Stephen ; Espinoza, Yasmin ; Poli, M Cecilia ; Fieschi, Claire ; Hauck, Fabian ; Cunningham-Rundles, Charlotte ; Mahlaoui, Nizar ; Warnatz, Klaus ; Sullivan, Kathleen E ; Tangye, Stuart G</creatorcontrib><description>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.</description><identifier>ISSN: 1097-6825</identifier><identifier>ISSN: 0091-6749</identifier><language>eng</language><subject>COVID-19 ; hypogammaglobulinemia ; immune dysregulation ; Immunology ; Immunology and Allergy ; inborn errors of immunity ; Medicine and Health Sciences ; primary immunodeficiencies ; SARS-CoV-2</subject><creationdate>2021</creationdate><rights>No license (in copyright) info:eu-repo/semantics/openAccess</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,315,776,780,4010,27837</link.rule.ids></links><search><creatorcontrib>Meyts, Isabelle</creatorcontrib><creatorcontrib>Bucciol, Giorgia</creatorcontrib><creatorcontrib>Quinti, Isabella</creatorcontrib><creatorcontrib>Neven, Bénédicte</creatorcontrib><creatorcontrib>Fischer, Alain</creatorcontrib><creatorcontrib>Seoane, Elena</creatorcontrib><creatorcontrib>Lopez-Granados, Eduardo</creatorcontrib><creatorcontrib>Gianelli, Carla</creatorcontrib><creatorcontrib>Robles-Marhuenda, Angel</creatorcontrib><creatorcontrib>Jeandel, Pierre-Yves</creatorcontrib><creatorcontrib>Paillard, Catherine</creatorcontrib><creatorcontrib>Sankaran, Vijay G</creatorcontrib><creatorcontrib>Demirdag, Yesim Yilmaz</creatorcontrib><creatorcontrib>Lougaris, Vassilios</creatorcontrib><creatorcontrib>Aiuti, Alessandro</creatorcontrib><creatorcontrib>Plebani, Alessandro</creatorcontrib><creatorcontrib>Milito, Cinzia</creatorcontrib><creatorcontrib>Dalm, Virgil ASH</creatorcontrib><creatorcontrib>Guevara-Hoyer, Kissy</creatorcontrib><creatorcontrib>Sánchez-Ramón, Silvia</creatorcontrib><creatorcontrib>Bezrodnik, Liliana</creatorcontrib><creatorcontrib>Barzaghi, Federica</creatorcontrib><creatorcontrib>Gonzalez-Granado, Luis Ignacio</creatorcontrib><creatorcontrib>Hayman, Grant R</creatorcontrib><creatorcontrib>Uzel, Gulbu</creatorcontrib><creatorcontrib>Mendonça, Leonardo Oliveira</creatorcontrib><creatorcontrib>Agostini, Carlo</creatorcontrib><creatorcontrib>Spadaro, Giuseppe</creatorcontrib><creatorcontrib>Badolato, Raffaele</creatorcontrib><creatorcontrib>Soresina, Annarosa</creatorcontrib><creatorcontrib>Vermeulen, François</creatorcontrib><creatorcontrib>Bosteels, Cedric</creatorcontrib><creatorcontrib>Lambrecht, Bart</creatorcontrib><creatorcontrib>Keller, Michael</creatorcontrib><creatorcontrib>Mustillo, Peter J</creatorcontrib><creatorcontrib>Abraham, Roshini S</creatorcontrib><creatorcontrib>Gupta, Sudhir</creatorcontrib><creatorcontrib>Ozen, Ahmet</creatorcontrib><creatorcontrib>Karakoc-Aydiner, Elif</creatorcontrib><creatorcontrib>Baris, Safa</creatorcontrib><creatorcontrib>Freeman, Alexandra</creatorcontrib><creatorcontrib>Yamazaki-Nakashimada, Marco</creatorcontrib><creatorcontrib>Scheffler-Mendoza, Selma</creatorcontrib><creatorcontrib>Espinosa-Padilla, Sara</creatorcontrib><creatorcontrib>Gennery, Andrew R</creatorcontrib><creatorcontrib>Jolles, Stephen</creatorcontrib><creatorcontrib>Espinoza, Yasmin</creatorcontrib><creatorcontrib>Poli, M Cecilia</creatorcontrib><creatorcontrib>Fieschi, Claire</creatorcontrib><creatorcontrib>Hauck, Fabian</creatorcontrib><creatorcontrib>Cunningham-Rundles, Charlotte</creatorcontrib><creatorcontrib>Mahlaoui, Nizar</creatorcontrib><creatorcontrib>Warnatz, Klaus</creatorcontrib><creatorcontrib>Sullivan, Kathleen E</creatorcontrib><creatorcontrib>Tangye, Stuart G</creatorcontrib><title>Coronavirus disease 2019 in patients with inborn errors of immunity : an international study</title><description>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.</description><subject>COVID-19</subject><subject>hypogammaglobulinemia</subject><subject>immune dysregulation</subject><subject>Immunology</subject><subject>Immunology and Allergy</subject><subject>inborn errors of immunity</subject><subject>Medicine and Health Sciences</subject><subject>primary immunodeficiencies</subject><subject>SARS-CoV-2</subject><issn>1097-6825</issn><issn>0091-6749</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>ADGLB</sourceid><recordid>eNqdjE0KwjAQRrNQsP7cYS5QiK3W1m1RPIBLIaQ62pGayExa8fZG8ASuPvge741UstTVJi3KbD1RU5G71rrKyypRp9qzd3Yg7gUuJGgFIdPLCsjB0wZCFwReFNp4NJ4dILNnAX8Fejx6R-ENW7Au4oDsohFzHUjoL--5Gl9tJ7j47Uxl-92xPqS3NmZNRw3j2QbjLRnL55YGNP3tixo0ZbEp8lWW_yV9AD31T4o</recordid><startdate>2021</startdate><enddate>2021</enddate><creator>Meyts, Isabelle</creator><creator>Bucciol, Giorgia</creator><creator>Quinti, Isabella</creator><creator>Neven, Bénédicte</creator><creator>Fischer, Alain</creator><creator>Seoane, Elena</creator><creator>Lopez-Granados, Eduardo</creator><creator>Gianelli, Carla</creator><creator>Robles-Marhuenda, Angel</creator><creator>Jeandel, Pierre-Yves</creator><creator>Paillard, Catherine</creator><creator>Sankaran, Vijay G</creator><creator>Demirdag, Yesim Yilmaz</creator><creator>Lougaris, Vassilios</creator><creator>Aiuti, Alessandro</creator><creator>Plebani, Alessandro</creator><creator>Milito, Cinzia</creator><creator>Dalm, Virgil ASH</creator><creator>Guevara-Hoyer, Kissy</creator><creator>Sánchez-Ramón, Silvia</creator><creator>Bezrodnik, Liliana</creator><creator>Barzaghi, Federica</creator><creator>Gonzalez-Granado, Luis Ignacio</creator><creator>Hayman, Grant R</creator><creator>Uzel, Gulbu</creator><creator>Mendonça, Leonardo Oliveira</creator><creator>Agostini, Carlo</creator><creator>Spadaro, Giuseppe</creator><creator>Badolato, Raffaele</creator><creator>Soresina, Annarosa</creator><creator>Vermeulen, François</creator><creator>Bosteels, Cedric</creator><creator>Lambrecht, Bart</creator><creator>Keller, Michael</creator><creator>Mustillo, Peter J</creator><creator>Abraham, Roshini S</creator><creator>Gupta, Sudhir</creator><creator>Ozen, Ahmet</creator><creator>Karakoc-Aydiner, Elif</creator><creator>Baris, Safa</creator><creator>Freeman, Alexandra</creator><creator>Yamazaki-Nakashimada, Marco</creator><creator>Scheffler-Mendoza, Selma</creator><creator>Espinosa-Padilla, Sara</creator><creator>Gennery, Andrew R</creator><creator>Jolles, Stephen</creator><creator>Espinoza, Yasmin</creator><creator>Poli, M Cecilia</creator><creator>Fieschi, Claire</creator><creator>Hauck, Fabian</creator><creator>Cunningham-Rundles, Charlotte</creator><creator>Mahlaoui, Nizar</creator><creator>Warnatz, Klaus</creator><creator>Sullivan, Kathleen E</creator><creator>Tangye, Stuart G</creator><scope>ADGLB</scope></search><sort><creationdate>2021</creationdate><title>Coronavirus disease 2019 in patients with inborn errors of immunity : an international study</title><author>Meyts, Isabelle ; Bucciol, Giorgia ; Quinti, Isabella ; Neven, Bénédicte ; Fischer, Alain ; Seoane, Elena ; Lopez-Granados, Eduardo ; Gianelli, Carla ; Robles-Marhuenda, Angel ; Jeandel, Pierre-Yves ; Paillard, Catherine ; Sankaran, Vijay G ; Demirdag, Yesim Yilmaz ; Lougaris, Vassilios ; Aiuti, Alessandro ; Plebani, Alessandro ; Milito, Cinzia ; Dalm, Virgil ASH ; Guevara-Hoyer, Kissy ; Sánchez-Ramón, Silvia ; Bezrodnik, Liliana ; Barzaghi, Federica ; Gonzalez-Granado, Luis Ignacio ; Hayman, Grant R ; Uzel, Gulbu ; Mendonça, Leonardo Oliveira ; Agostini, Carlo ; Spadaro, Giuseppe ; Badolato, Raffaele ; Soresina, Annarosa ; Vermeulen, François ; Bosteels, Cedric ; Lambrecht, Bart ; Keller, Michael ; Mustillo, Peter J ; Abraham, Roshini S ; Gupta, Sudhir ; Ozen, Ahmet ; Karakoc-Aydiner, Elif ; Baris, Safa ; Freeman, Alexandra ; Yamazaki-Nakashimada, Marco ; Scheffler-Mendoza, Selma ; Espinosa-Padilla, Sara ; Gennery, Andrew R ; Jolles, Stephen ; Espinoza, Yasmin ; Poli, M Cecilia ; Fieschi, Claire ; Hauck, Fabian ; Cunningham-Rundles, Charlotte ; Mahlaoui, Nizar ; Warnatz, Klaus ; Sullivan, Kathleen E ; Tangye, Stuart G</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-ghent_librecat_oai_archive_ugent_be_86763423</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2021</creationdate><topic>COVID-19</topic><topic>hypogammaglobulinemia</topic><topic>immune dysregulation</topic><topic>Immunology</topic><topic>Immunology and Allergy</topic><topic>inborn errors of immunity</topic><topic>Medicine and Health Sciences</topic><topic>primary immunodeficiencies</topic><topic>SARS-CoV-2</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Meyts, Isabelle</creatorcontrib><creatorcontrib>Bucciol, Giorgia</creatorcontrib><creatorcontrib>Quinti, Isabella</creatorcontrib><creatorcontrib>Neven, Bénédicte</creatorcontrib><creatorcontrib>Fischer, Alain</creatorcontrib><creatorcontrib>Seoane, Elena</creatorcontrib><creatorcontrib>Lopez-Granados, Eduardo</creatorcontrib><creatorcontrib>Gianelli, Carla</creatorcontrib><creatorcontrib>Robles-Marhuenda, Angel</creatorcontrib><creatorcontrib>Jeandel, Pierre-Yves</creatorcontrib><creatorcontrib>Paillard, Catherine</creatorcontrib><creatorcontrib>Sankaran, Vijay G</creatorcontrib><creatorcontrib>Demirdag, Yesim Yilmaz</creatorcontrib><creatorcontrib>Lougaris, Vassilios</creatorcontrib><creatorcontrib>Aiuti, Alessandro</creatorcontrib><creatorcontrib>Plebani, Alessandro</creatorcontrib><creatorcontrib>Milito, Cinzia</creatorcontrib><creatorcontrib>Dalm, Virgil ASH</creatorcontrib><creatorcontrib>Guevara-Hoyer, Kissy</creatorcontrib><creatorcontrib>Sánchez-Ramón, Silvia</creatorcontrib><creatorcontrib>Bezrodnik, Liliana</creatorcontrib><creatorcontrib>Barzaghi, Federica</creatorcontrib><creatorcontrib>Gonzalez-Granado, Luis Ignacio</creatorcontrib><creatorcontrib>Hayman, Grant R</creatorcontrib><creatorcontrib>Uzel, Gulbu</creatorcontrib><creatorcontrib>Mendonça, Leonardo Oliveira</creatorcontrib><creatorcontrib>Agostini, Carlo</creatorcontrib><creatorcontrib>Spadaro, Giuseppe</creatorcontrib><creatorcontrib>Badolato, Raffaele</creatorcontrib><creatorcontrib>Soresina, Annarosa</creatorcontrib><creatorcontrib>Vermeulen, François</creatorcontrib><creatorcontrib>Bosteels, Cedric</creatorcontrib><creatorcontrib>Lambrecht, Bart</creatorcontrib><creatorcontrib>Keller, Michael</creatorcontrib><creatorcontrib>Mustillo, Peter J</creatorcontrib><creatorcontrib>Abraham, Roshini S</creatorcontrib><creatorcontrib>Gupta, Sudhir</creatorcontrib><creatorcontrib>Ozen, Ahmet</creatorcontrib><creatorcontrib>Karakoc-Aydiner, Elif</creatorcontrib><creatorcontrib>Baris, Safa</creatorcontrib><creatorcontrib>Freeman, Alexandra</creatorcontrib><creatorcontrib>Yamazaki-Nakashimada, Marco</creatorcontrib><creatorcontrib>Scheffler-Mendoza, Selma</creatorcontrib><creatorcontrib>Espinosa-Padilla, Sara</creatorcontrib><creatorcontrib>Gennery, Andrew R</creatorcontrib><creatorcontrib>Jolles, Stephen</creatorcontrib><creatorcontrib>Espinoza, Yasmin</creatorcontrib><creatorcontrib>Poli, M Cecilia</creatorcontrib><creatorcontrib>Fieschi, Claire</creatorcontrib><creatorcontrib>Hauck, Fabian</creatorcontrib><creatorcontrib>Cunningham-Rundles, Charlotte</creatorcontrib><creatorcontrib>Mahlaoui, Nizar</creatorcontrib><creatorcontrib>Warnatz, Klaus</creatorcontrib><creatorcontrib>Sullivan, Kathleen E</creatorcontrib><creatorcontrib>Tangye, Stuart G</creatorcontrib><collection>Ghent University Academic Bibliography</collection></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Meyts, Isabelle</au><au>Bucciol, Giorgia</au><au>Quinti, Isabella</au><au>Neven, Bénédicte</au><au>Fischer, Alain</au><au>Seoane, Elena</au><au>Lopez-Granados, Eduardo</au><au>Gianelli, Carla</au><au>Robles-Marhuenda, Angel</au><au>Jeandel, Pierre-Yves</au><au>Paillard, Catherine</au><au>Sankaran, Vijay G</au><au>Demirdag, Yesim Yilmaz</au><au>Lougaris, Vassilios</au><au>Aiuti, Alessandro</au><au>Plebani, Alessandro</au><au>Milito, Cinzia</au><au>Dalm, Virgil ASH</au><au>Guevara-Hoyer, Kissy</au><au>Sánchez-Ramón, Silvia</au><au>Bezrodnik, Liliana</au><au>Barzaghi, Federica</au><au>Gonzalez-Granado, Luis Ignacio</au><au>Hayman, Grant R</au><au>Uzel, Gulbu</au><au>Mendonça, Leonardo Oliveira</au><au>Agostini, Carlo</au><au>Spadaro, Giuseppe</au><au>Badolato, Raffaele</au><au>Soresina, Annarosa</au><au>Vermeulen, François</au><au>Bosteels, Cedric</au><au>Lambrecht, Bart</au><au>Keller, Michael</au><au>Mustillo, Peter J</au><au>Abraham, Roshini S</au><au>Gupta, Sudhir</au><au>Ozen, Ahmet</au><au>Karakoc-Aydiner, Elif</au><au>Baris, Safa</au><au>Freeman, Alexandra</au><au>Yamazaki-Nakashimada, Marco</au><au>Scheffler-Mendoza, Selma</au><au>Espinosa-Padilla, Sara</au><au>Gennery, Andrew R</au><au>Jolles, Stephen</au><au>Espinoza, Yasmin</au><au>Poli, M Cecilia</au><au>Fieschi, Claire</au><au>Hauck, Fabian</au><au>Cunningham-Rundles, Charlotte</au><au>Mahlaoui, Nizar</au><au>Warnatz, Klaus</au><au>Sullivan, Kathleen E</au><au>Tangye, Stuart G</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Coronavirus disease 2019 in patients with inborn errors of immunity : an international study</atitle><date>2021</date><risdate>2021</risdate><issn>1097-6825</issn><issn>0091-6749</issn><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.</abstract><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 1097-6825 |
| ispartof | |
| issn | 1097-6825 0091-6749 |
| language | eng |
| recordid | cdi_ghent_librecat_oai_archive_ugent_be_8676342 |
| source | Ghent University Academic Bibliography; Elsevier ScienceDirect Journals |
| subjects | COVID-19 hypogammaglobulinemia immune dysregulation Immunology Immunology and Allergy inborn errors of immunity Medicine and Health Sciences primary immunodeficiencies SARS-CoV-2 |
| title | Coronavirus disease 2019 in patients with inborn errors of immunity : an international study |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-01T21%3A17%3A36IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-ghent&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Coronavirus%20disease%202019%20in%20patients%20with%20inborn%20errors%20of%20immunity%20:%20an%20international%20study&rft.au=Meyts,%20Isabelle&rft.date=2021&rft.issn=1097-6825&rft_id=info:doi/&rft_dat=%3Cghent%3Eoai_archive_ugent_be_8676342%3C/ghent%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_id=info:pmid/&rfr_iscdi=true |