Adverse COVID-19 outcomes in immune deficiencies: Inequality exists between subclasses

Elif Karakoc Aydiner, Sevgi Bilgic Eltan, Royale Babayeva, Omer Aydiner, Eda Kepenekli, Burcu Kolukisa, Asena Pinar Sefer, Ezgi Yalcin Gungoren, Esra Karabiber, Esra Ozek Yucel, Oner Ozdemir, Ayca Kiykim, Hasibe Artac, Nalan Yakici, Koray Yalcin, Haluk Cokugras, Tulin Tiraje Celkan, Fazil Orhan, Mehmet Akif Yesilipek, Safa BarisAhmet Ozen

Research output: Contribution to journalArticlepeer-review

36 Citations (Scopus)

Abstract

Background: Genetic deficiencies of immune system, referred to as inborn errors of immunity (IEI), serve as a valuable model to study human immune responses. In a multicenter prospective cohort, we evaluated the outcome of SARS-CoV-2 infection among IEI subjects and analyzed genetic and immune characteristics that determine adverse COVID-19 outcomes. Methods: We studied 34 IEI patients (19M/15F, 12 [min: 0.6-max: 43] years) from six centers. We diagnosed COVID-19 infection by finding a positive SARS-CoV-2 PCR test (n = 25) and/or a lung tomography scoring (CORADS) ≥4 (n = 9). We recorded clinical and laboratory findings prospectively, fitted survival curves, and calculated fatality rates for the entire group and each IEI subclass. Results: Nineteen patients had combined immune deficiency (CID), six with predominantly antibody deficiency (PAD), six immune dysregulation (ID), two innate immune defects, and one in the autoinflammatory class. Overall, 23.5% of cases died, with disproportionate fatality rates among different IEI categories. PAD group had a relatively favorable outcome at any age, but CIDs and IDs were particularly vulnerable. At admission, presence of dyspnea was an independent risk for COVID-related death (OR: 2.630, 95% CI; 1.198–5.776, p <.001). Concerning predictive roles of laboratory markers at admission, deceased subjects compared to survived had significantly higher CRP, procalcitonin, Troponin-T, ferritin, and total-lung-score (p =.020, p =.003, p =.014, p =.013, p =.020; respectively), and lower absolute lymphocyte count, albumin, and trough IgG (p =.012, p =.022, p =.011; respectively). Conclusion: Our data disclose a highly vulnerable IEI subgroup particularly disadvantaged for COVID-19 despite their youth. Future studies should address this vulnerability and consider giving priority to these subjects in SARS-Cov-2 therapy trials.

Original languageEnglish
Pages (from-to)282-295
Number of pages14
JournalAllergy: European Journal of Allergy and Clinical Immunology
Volume77
Issue number1
DOIs
Publication statusPublished - Jan 2022
Externally publishedYes

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