SARS-CoV-2–Related Acute Respiratory Distress Syndrome Uncovers a Patient with Severe Combined Immunodeficiency Disease

COVID-19 and Inborn Errors of Immunity

“…We identified in the literature 132 patients with a diagnosis of either severe combined immunodeficiency (SCID; n = 22) or combined immunodeficiency (CID; n = 110) and COVID-19 ( 3 – 11 , 25 – 40 ). The overall mortality in this group was 12% ( n = 16) with a total of 27 patients (20%) admitted to the intensive care unit (ICU).…”

“…The overall mortality in this group was 12% ( n = 16) with a total of 27 patients (20%) admitted to the intensive care unit (ICU). In the SCID group, 14 patients had received stem cell therapy (SCT) before infection with SARS-CoV-2 ( 3 , 4 , 6 – 8 , 11 , 28 , 36 , 39 , 40 ) and their survival rate was 93% (13/14), with only one death reported in a patient that was infected on day +40 post-SCT ( 8 ). By contrast, out of eight untransplanted SCID patients who were infected with SARS-CoV-2, only four survived, with death being primarily due to respiratory failure and ARDS ( 26 ).…”

“…By contrast, out of eight untransplanted SCID patients who were infected with SARS-CoV-2, only four survived, with death being primarily due to respiratory failure and ARDS ( 26 ). Recently Al-Saud et al ( 11 ) reported of a previously healthy 5-mo-old boy with critical COVID-19 pneumonia requiring mechanical ventilation that after immune workup was diagnosed with T-B + NK SCID due to JAK3 deficiency and was successfully transplanted from a matched sibling donor. This case underlines the importance of genetic evaluation in critical cases of COVID-19 in healthy pediatric patients.…”

“…These discrepancies are mainly due to an assortment bias due to the differences in the nature of IEI included in the studies. Importantly, studies conducted on patients with IEI and life-threatening COVID-19 have revealed how impairment in selected immune genes may predispose a patient to critical SARS-CoV-2 infection and multisystem inflammatory syndrome (MIS) ( 11 – 18 ). In this context, an anti-SARS- CoV-2 vaccination campaign was implemented to protect patients with IEI.…”

Mechanisms underlying host defense and disease pathology in response to severe acute respiratory syndrome (SARS)-CoV2 infection: insights from inborn errors of immunity

“…However, the age of affected IEI patients was lower (median 24 yrs vs 48 yrs; Fig. 1A), and the proportion admitted to ICU was higher (14.0% vs 2–5%) than the general population [12,27–63,67,69–75,76 ▪▪ ]. Within the different classifications of IEIs, SARS-CoV2-infected CVID patients were significantly older (mean: 40.4 years) than SARS-CoV2 + patients with agammaglobulinemia (mean: 23.1 years), chronic granulomatous disease (CGD) (mean: 15.4 years), or APECED (mean: 24.4 years) (Fig.…”