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. 2024 Apr 5;44(4):86.
doi: 10.1007/s10875-023-01613-5.

COVID-19 Vaccination in Patients with Inborn Errors of Immunity Reduces Hospitalization and Critical Care Needs Related to COVID-19: a USIDNET Report

John McDonnell  1 Kimberley Cousins  2 M Elizabeth M Younger  3 Adam Lane  4   5 Hassan Abolhassani  6   7 Roshini S Abraham  8   9 Salem Al-Tamemi  10 Juan Carlos Aldave-Becerra  11 Eman Hesham Al-Faris  12 Alberto Alfaro-Murillo  13 Suzan A AlKhater  14   15 Nouf Alsaati  16 Alexa Michelle Altman Doss  17 Melissa Anderson  18 Ernestina Angarola  19 Barbara Ariue  20 Danielle E Arnold  21 Amal H Assa'ad  22 Caner Aytekin  23 Meaghan Bank  24 Jenna R E Bergerson  25 Jack Bleesing  4   5 John Boesing  26 Carolina Bouso  27 Nicholas Brodszki  28 Diana Cabanillas  29 Carol Cady  30 Meghan A Callahan  31 Roberta Caorsi  32 Javier Carbone  33 Maria Carrabba  34 Riccardo Castagnoli  35   36 Jason R Catanzaro  37 Samantha Chan  38 Sharat Chandra  4   5 Hugo Chapdelaine  39 Zahra Chavoshzadeh  40 Hey Jin Chong  41 Lori Connors  42 Filippo Consonni  43 Oscar Correa-Jimenez  44 Charlotte Cunningham-Rundles  2 Katherine D'Astous-Gauthier  45 Ottavia Maria Delmonte  25 Yesim Yilmaz Demirdag  46 Deepti R Deshpande  47 Natalie M Diaz-Cabrera  48 Victoria R Dimitriades  49 Rasha El-Owaidy  50 Gehad ElGhazali  51 Suleiman Al-Hammadi  52 Giovanna Fabio  34 Astrid Schellnast Faure  53 Jin Feng  54 James M Fernandez  55 Lauren Fill  56 Guacira R Franco  57 Robert W Frenck  5   58 Ramsay L Fuleihan  59 Giuliana Giardino  60 Jessica Galant-Swafford  61 Eleonora Gambineri  43   62 Elizabeth K Garabedian  63 Ashley V Geerlinks  64 Ekaterini Goudouris  65 Octavio Grecco  57 Qiang Pan-Hammarström  6 Hedieh Haji Khodaverdi Khani  40 Lennart Hammarström  6 Nicholas L Hartog  66 Jennifer Heimall  67 Gabriela Hernandez-Molina  68 Caroline C Horner  69 Robert W Hostoffer  56 Nataliya Hristova  70 Kuang-Chih Hsiao  71   72   73 Gabriela Ivankovich-Escoto  74 Faris Jaber  54 Maaz Jalil  75 Mahnaz Jamee  76 Tiffany Jean  46 Stephanie Jeong  54 Devi Jhaveri  77 Michael B Jordan  4   5   78 Avni Y Joshi  79 Amanpreet Kalkat  56 Henry J Kanarek  80 Erinn S Kellner  81 Amer Khojah  82 Ruby Khoury  4   5 Cristina M Kokron  57 Ashish Kumar  4   5 Kelsey Lecerf  83 Heather K Lehman  84 Jennifer W Leiding  85 Harry Lesmana  86 Xin Rong Lim  87 Joao Pedro Lopes  88 Ana Laura López  89 Lucia Tarquini  90 Ingrid S Lundgren  91 Julieann Magnusson  92 Ana Karolina B B Marinho  57 Gian Luigi Marseglia  35 Giulia M Martone  84 Annamaria G Mechtler  93 Leonardo Mendonca  57   94 Joshua D Milner  95 Peter J Mustillo  96 Asal Gharib Naderi  97 Samuele Naviglio  98 Jeremy Nell  99 Hana B Niebur  100 Luigi Notarangelo  25 Matias Oleastro  27 María Claudia Ortega-López  101 Neil R Patel  102 Gordana Petrovic  103 Claudio Pignata  104 Oscar Porras  105 Benjamin T Prince  96 Jennifer M Puck  106 Nashmia Qamar  107 Marco Rabusin  98 Nikita Raje  18 Lorena Regairaz  108 Kimberly A Risma  109 Elizabeth H Ristagno  110 John Routes  111 Persio Roxo-Junior  112 Negin Salemi  113 Christopher Scalchunes  114 Susan J Schuval  115 Suranjith L Seneviratne  116 Ashwin Shankar  56 Roya Sherkat  113 Junghee Jenny Shin  117 Abeer Siddiqi  118 Sara Signa  32 Ali Sobh  119 Fabiana Mascarenhas Souza Lima  57 Kristen K Stenehjem  102 Jonathan S Tam  120 Monica Tang  121 Myrthes Toledo Barros  57 James Verbsky  111 Eleni Vergadi  122 Dayne H Voelker  123 Stefano Volpi  32   124 Luke A Wall  125 Christine Wang  126 Kelli W Williams  127 Eveline Y Wu  128 Shan Shan Wu  129   130 Jessie J Zhou  131 Alexandria Cook  111 Kathleen E Sullivan  16 Rebecca Marsh  4   5
Affiliations

COVID-19 Vaccination in Patients with Inborn Errors of Immunity Reduces Hospitalization and Critical Care Needs Related to COVID-19: a USIDNET Report

John McDonnell et al. J Clin Immunol. .

Abstract

Background: The CDC and ACIP recommend COVID-19 vaccination for patients with inborn errors of immunity (IEI). Not much is known about vaccine safety in IEI, and whether vaccination attenuates infection severity in IEI.

Objective: To estimate COVID-19 vaccination safety and examine effect on outcomes in patients with IEI.

Methods: We built a secure registry database in conjunction with the US Immunodeficiency Network to examine vaccination frequency and indicators of safety and effectiveness in IEI patients. The registry opened on January 1, 2022, and closed on August 19, 2022.

Results: Physicians entered data on 1245 patients from 24 countries. The most common diagnoses were antibody deficiencies (63.7%). At least one COVID-19 vaccine was administered to 806 patients (64.7%), and 216 patients received vaccination prior to the development of COVID-19. The most common vaccines administered were mRNA-based (84.0%). Seventeen patients were reported to seek outpatient clinic or emergency room care for a vaccine-related complication, and one patient was hospitalized for symptomatic anemia. Eight hundred twenty-three patients (66.1%) experienced COVID-19 infection. Of these, 156 patients required hospitalization (19.0%), 47 required ICU care (5.7%), and 28 died (3.4%). Rates of hospitalization (9.3% versus 24.4%, p < 0.001), ICU admission (2.8% versus 7.6%, p = 0.013), and death (2.3% versus 4.3%, p = 0.202) in patients who had COVID-19 were lower in patients who received vaccination prior to infection. In adjusted logistic regression analysis, not having at least one COVID-19 vaccine significantly increased the odds of hospitalization and ICU admission.

Conclusion: Vaccination for COVID-19 in the IEI population appears safe and attenuates COVID-19 severity.

Keywords: Immunization; Immunodeficiency; Outcomes; Viruses: respiratory diseases.

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Conflict of interest statement

Deepti Deshpande is employed by Regeneron Pharmaceuticals, spouse employed by Arch Oncology. Elizabeth Ristagno owns stock in Moderna and Pfizer. Kathleen Sullivan is a consultant for the Immune Deficiency Foundation. Rebecca Marsh is an employee of Pharming Healthcare, Inc. Nikita Raje is a speaker and on the medical advisory board for Enzyvant, a consultant for Pfizer, and a speaker for Pharming.

Figures

Fig. 1
Fig. 1
Patient diagnoses in USIDNET registry, categorized by International Union of Immunologic Societies (IUIS) schema. General IUIS categories further subclassified based on phenotype or genetic defect. Abbreviations: SCID, severe combined immune deficiency; CID, combined immune deficiency; A-T, ataxia-telangiectasia; WAS, Wiskott-Aldrich syndrome; CHARGE, coloboma/heart defects/atresia choanae/growth retardation/genital abnormalities/ear abnormalities; NEMO, nuclear factor-kappa B essential modulator deficiency; CVID, common variable immune deficiency; hypogamma, hypogammaglobulinemia; agamma, agammaglobulinemia; Comp. Def., complement deficiency; SAD, specific antibody deficiency; Subclass Def., IgG subclass deficiency; IgA Def., IgA deficiency; HLH/EBV Susc., hemophagocytic lymphohistiocytosis and EBV susceptibility; ALPS, autoimmune lymphoproliferative syndrome; IPEX, immune dysregulation/polyendocrinopathy/enteropathy/X-linked syndrome; VEO-IBD, very early onset inflammatory bowel disease; CGD, chronic granulomatous disease; MSMD, Mendelian susceptibility to mycobacterial disease; Cong. Neut., congenital neutropenia; Marrow Fail., bone marrow failure; Viral Predisp., predisposition to severe viral infection
Fig. 2
Fig. 2
Hospitalization, ICU admission, and death among the USIDNET registry cohort. Categorization was adapted from International Union of Immunological Societies (IUIS) phenotypic classification. Age quartile (years) is based on patient age at time of COVID-19 infection. Three infected patients lacked data on age. COVID-19 risk factors included history of lung disease, immunosuppressive medication use in the 3 months preceding infection, obesity, and renal disease. Additionally, a measure of “other risk factors” was determined, representing a composite of uncommonly observed risk factors in the cohort—neuromuscular disease, tracheostomy, heart disease, sickle cell disease, and diabetes. Any patient with at least one of these uncommonly observed risk factors was counted for this measure. Vaccination was determined as receipt of at least one COVID-19 vaccine prior to SARS-CoV-2 infection. Sixty-six patients lacked adequate information on timing of vaccination relative to infection and were not included

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