Coronavirus disease 2019 in patients with inborn errors of immunity: An international study.
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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 N, Keller Michael, Mustillo Peter J, Abraham Roshini S, Gupta Sudhir, Ozen Ahmet, Karakoc-Aydiner Elif, Baris Safa, Freeman Alexandra F, Yamazaki-Nakashimada Marco, Scheffler-Mendoza Selma, Espinosa-Padilla Sara, Gennery Andrew R, Jolles Stephen, Espinosa Yazmin, Poli M Cecilia, Fieschi Claire, Hauck Fabian, Cunningham-Rundles Charlotte, Mahlaoui Nizar, IUIS Committee of Inborn Errors of Immunity, Warnatz Klaus, Sullivan Kathleen E, Tangye Stuart G
Аннотация
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.