Table I.
SARS-CoV-2 infection in defined IEI
| Type of IEI | Gene defect/IEI | Approximate no. of patients | Study or studies |
|---|---|---|---|
| Severe combined immunodeficiency (n = 25) | JAK3 | 1 | 70 |
| RAG | 3 | 92, 97, 115 | |
| IL7RA | 1 | 91, 94 | |
| DCLRE1C | 1 | 49 | |
| IL2RG | 4 | 77, 95, 115 | |
| CD3D | 1 | 105 | |
| Not specified | 15 | 95, 99, 108 | |
| Combined immunodeficiency (n = 91) | STAT3 DN | 7 | 103, 109, 115, 176 |
| PGM3 | 1 | 102, 115 | |
| ARPC1B | 1 | 47, 105, 115 | |
| WAS | 8 | 47, 48, 95, 99, 100, 103, 105, 108, 109, 115 | |
| ZAP70 | 1 | 115 | |
| CD40L | 9 | 94, 95, 97, 103, 109, 111, 116, 143 | |
| RASGRP1 | 1 | 92 | |
| CARD11 | 1 | 92, 103 | |
| RELB | 3 | 97, 116 | |
| STK4 | 1 | 89 | |
| DNMT3B/NBS1 | 4 | 89, 91, 94 | |
| ICOS | 1 | 15, 103 | |
| IKBKG (NEMO) | 3 | 72, 78, 94 | |
| ATM | 11 | 91, 92, 94, 99, 100, 102, 103, 108 | |
| Di George syndrome | 16 | 99, 100, 108 | |
| Not specified | 23 | 89, 92, 94, 95, 99, 103, 108 | |
| Predominantly antibody deficient (n = 714) | CVID∗ | 589 | 51, 52, 58, 71, 75, 83, 92, 94, 95, 97, 98, 99, 100, 102, 103, 104, 105, 106, 107, 108, 109, 111, 112, 113, 114, 115, 143 |
| BTK | 98 | 15, 46, 51, 53, 55, 60, 61, 66, 73, 85, 86, 91, 92, 94, 95, 97, 98, 99, 100, 102, 103, 104, 105, 108, 109, 111, 115, 116, 139, 140, 143 | |
| AR agammaglobulinemia | 9 | 99, 100, 115 | |
| PIK3R1/PIK3CD GOF | 7 | 64, 82, 91, 95, 99, 100, 115 | |
| NFKB1 | 4 | 15, 91, 103, 111, 115 | |
| NFKB2 | 3 | 43, 103, 115, 143 | |
| IKZF1 | 1 | 91 | |
| Immune dysregulation (n = 64) | AIRE (APS1/APECED) | 29 | 57, 84, 94, 118, 122, 149 |
| CTLA4 | 7 | 15, 97, 103, 115, 177 | |
| LRBA | 3 | 92, 97, 115 | |
| SOCS1 | 1 | 76 | |
| STAT3 GOF | 1 | 111 | |
| RAB27A | 1 | 89 | |
| CD70 | 1 | 89 | |
| ALPS | 5 | 95, 99, 102, 108 | |
| XLP (XIAP, SH2D1A) | 4 | 63, 95, 108, 109, 115 | |
| PRKCD | 1 | 115 | |
| RLTPR/CARMIL2 | 2 | 94 | |
| CD137 | 1 | 94 | |
| STXBP2 | 2 | 88, 94 | |
| Not specified/other | 6 | 92, 99, 105, 108 | |
| Phagocytic defects, bone marrow failure (n = 36) | Chronic granulomatous disease (CYBB; NCF2) | 28 | 15, 59, 89, 95, 97, 102, 103, 105, 108, 115 |
| GATA2 | 2 | 15, 103, 115 | |
| DNAJC21 | 1 | 115 | |
| Not specified/other | 5 | 92, 99 | |
| Innate immune defects (n = 75) | TLR3/UNC93B/TRIF/IRF3/IRF7/IRF9/TBK1 | 23 | 65, 68, 69, 120, 123 |
| IFNAR1/2 | 7 | 42, 56, 87, 126 | |
| STAT1/TYK2 | 2 | 126 | |
| TLR7 | 22 | 90, 124, 125, 126 | |
| MYD88/IRAK4 | 8 | 45, 81, 95, 99, 102 | |
| IFNGR1/IFNGR2/IL12RB1 | 5 | 54, 79, 95, 111, 115 | |
| STAT1 GOF | 6 | 50, 92, 95, 102, 109, 115 | |
| CXCR4 GOF | 2 | 94, 95 | |
| Autoinflammatory disorders (n = 96) | MEFV | 68 | 93, 95, 110, 115 |
| IL1RN | 1 | 89 | |
| Aicardi-Goutières syndrome (RNASEH2B, SAMHD1) | 5 | 15, 99, 100, 115 | |
| TNFAIP3 | 1 | 15 | |
| NLRP1, NLRP3, NLRP12 | 3 | 91, 95 | |
| IL36RN | 1 | 74 | |
| ADA2 | 1 | 94 | |
| Not specified/other | 16 | 95, 108 | |
| Complement deficiencies (n = 55) | Hereditary angioedema (pathogenic SERPING variants), C3 deficiency, other | 55 | 15, 91, 95, 96, 109 |
| Phenocopies of IEI | Good syndrome | 13 | 83, 100, 103, 105, 109 |
| Autoantibodies to type I IFNs | Many! | 128, 129, 130, 131, 132, 133, 134, 135, 136 |
∗
Including hypogamma, immunoglobulin subclass deficiency, and specific antibody deficiency.