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

• DOI: 10.1111/all.15025
• Publication Date: 2021-07-27T17:06:00Z
• Authors: 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 Yesil...; Safa Baris; Ahmet Ozen
• Keywords: 0301 basic medicine; 0303 health sciences; inborn errors of immunity; Adolescent; SARS-CoV-2; SARS-Cov-2; Primary Immunodeficiency Diseases; Immunologic Deficiency Syndromes; COVID-19; 3. Good health; 03 medical and health sciences; outcome; Humans; Prospective Studies

AbstractBackgroundGenetic 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.MethodsWe 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.ResultsNineteen 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).ConclusionOur 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.