Table I.
Main characteristic of patients with IEI and SARS-CoV-2 infection
| IEI type (no. of patients) | Outcome | Note | Reference |
|---|---|---|---|
| CVID (5) XLA (1) ARA (1) |
20% death; mild disease course and favorable outcome in agammaglobulinemic patients in contrast to severe course and need for mechanical ventilation in patients with CVID | Authors speculate on a role of B lymphocytes in virus-induced inflammation | 93,94 |
| APECED (1) | Life-threatening COVID-19 pneumonia needing mechanical ventilation and ICU admission in a patient with anti–type I IFN autoantibodies | Beccuti et al103 speculate on an increased infection risk in patients with adrenal insufficiency, while Bastard et al104 highlighted the contribution of anti–type I IFN autoantibodies to disease outcome | 103,104 |
| PAD (53) CID (14) IDS (9) Autoinflammatory disorder (7) Phagocyte defect (6) Innate immune defect (3) Bone marrow failure (2) |
9% death; mild disease course in 37% of patients. All adult patients who died presented with preexisting comorbidities | Risk factors for severe COVID-19 in patients with IEI mirror those of the general population and IEI itself does not represent an independent risk factor. However, patients with a severe course requiring ICU admission are younger compared with the general population | 98 |
| CVID (23) PAD (12) CID (4) XLA (4) CGD (3) NF-kB deficiency (2) Others (12) |
17% death; 20% infection-fatality ratio, 31.6% case-fatality ratio, 37.5% inpatient mortality in patients with IEIs | Patients with IEIs succumb to COVID-19 at a younger age. Morbidity and mortality in patients with IEI exceed that of the general population | 99 |
| WAS (1) | Mild course in a patient who underwent gene therapy (GT) 5 mo before infection | GT-induced immune reconstitution enabled an adequate response against the infection | 98,118 |
| Good syndrome (1) | Death | Authors hypothesize the role of immune dysfunction or anatomical alterations predisposing the patient to a fatal infection outcome | 119 |
| PAD (1) | Mild disease course treated with supportive measures and no need for ICU admission | 120 | |
| CVID (4) HIGM (4) CID (5) XLA (2) CGD (2) Others (3) |
30% asymptomatic, 65% mild disease course; the whole IEI cohort was treated as outpatient | Authors point out the possible role of social distancing and precautions in determining the mild disease course observed in the cohort | 100 |
| NFKB2 LOF | Life-threatening COVID-19 pneumonia needing mechanical ventilation and ICU admission | Authors emphasize how aggressive management of the consequences of underlying immune dysregulation in the patient enabled his recovery from severe SARS-CoV-2 infection | 121 |
| CID (10) PAD (4) Phagocyte defect (2) IDS (2) Autoinflammatory disorders (1) |
42% death Almost all patients who succumbed were younger than 10 y. All subjects with IEI with SARS-CoV-2 infection were hospitalized |
Authors observed a reverse pattern of age and a 10-fold higher mortality rate in SARS-CoV-2–infected patients with IEIs compared with the population | 122 |
APECED, Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy; ARA, autosomal-recessive agammaglobulinemia; CGD, chronic granulomatous disease; CID, combined immune deficiency; CVID, common variable immune deficiency; HIGM, hyper-IgM syndrome; IDS, immunedysregulation syndrome; NFKB2 LOF, nuclear factor kappa B loss of function; PAD, primary antibody deficiency; XLA, X-linked agammaglobulinemia.