Clinical outcome, incidence, and SARS-CoV-2 infection-fatality rates in Italian patients with inborn errors of immunity

According to World Health Organization criteria 2020,4 patients might be stratified in asymptomatic, mild, moderate, and severe COVID-19. Because patients with IEI might struggle with clearing the infection, we calculated the time from the first SARS-CoV-2–positive PCR test result to the first SARS-CoV-2–negative PCR test result. The purely descriptive data set on patients with IEI might be the basis for a comparison over time of the trend of SARS-CoV-2 infection in this population as is for data on the trend of SARS-CoV-2 infection in the general population.Acknowledgments We thank our patients and their families.Online Repository SARS-CoV-2 positive % Mean age ≤18 y 25.1 9.6 ± 5.7 Male 60.6 Asymptomatic 36.3 6.2 ± 2.9 Mild/moderate 60.6 5.6 ± 4.2 Severe 3.03 1 Death 0 >18 y 74.8 43.9 ± 15.8 Male 58.2 Asymptomatic 24.5 38.0 ± 17.0∗ Mild/moderate 55.1 41.6 ± 16.8 Severe 15.8 50.9 ± 14.8 Death 5.1 48.5 ± 13.0 Table I Demographic data, and disease severity of SARS-CoV-2–positive patients with IEI IEI entity No. of SARS-CoV-2–positive patients No. of patients with IEI enrolled Cumulative incidence (per 100,000) Infection-fatality rate (%) CVID 74 1161∗ 6.4 4.05 XLA 13 148 8.8 0 ARA 3 17 17.6 0 SIgAD 7 961 0.7 0 Good’s syndrome 3 24 12.5 33.3 Del 22q11 12 527 2.3 8.3 WAS 0 5 0 0 CGD 0 66 0 0 AT 2 54 3.7 0 HIE syndrome 0 50 0 0 ALPS 1 12 8.3 0 CD4 lymphopenia 2 26 7.7 0 APDS 2 2 † 0 Aicardi-Goutiers 1 1 † 0 Prolidase deficiency 1 1 † 0 MyD88 deficiency 1 1 † 0 NBAS deficiency 1 1 † 0 XIAP 0 1 † 0 Neutropenia 2 39 5.1 0 Post-HSCT, post–gene therapy, and postthymic transplant 6 162 3.70 0 IEI (total number) 131 3,263 4.01 3.81 18 y 98 1,867 5.25 5.10 Italian population (total number) 3,123,368 59,816,655 5.22 3.28 18 y 2,705,616 49,656,655 5.45 3.68 Table II Cumulative incidence per 100,000, and infection-fatality percent for IEI by diagnosis: