Table III.
Subcategorization of PAD cases
| Primary (n = 52) |
Secondary (n = 10) | P | ||||
|---|---|---|---|---|---|---|
| Subtype | Mild (n = 12) | Moderate (n = 21) | Severe (n = 19) | P | ||
| PAD diagnosis | ||||||
| Clinical entities | Immunoglobulin subclass deficiency (3) Specific antibody deficiency (5) PHG (4) |
Common variable immunodeficiency (21) | Complicated PAD (19): Activated PI3K-δ syndrome (4) TACI deficiency (3) Nuclear factor-κB1 deficiency (1) Complicated common variable immunodeficiency/specific antibody deficiency (gene not known) (11) |
Diagnosis confounded by: Clonal suppression (3) Immunosuppression (7) |
||
| PID genetic testing | ||||||
| Yes (% [n]) | 33.3 (4) | 42.8 (9) | 84.2 (16) | .005 | 10.0 (1) | .0074 |
| Pathogenic variant (% [n]) | 0 | 0 | 42.1 (8): PIK3CD (3) TNFRSF13B (3) PIK3AP1 (1) NFκB1 (1) |
<.0001 | 0 | .19 |
| IgR | ||||||
| Yes (% [n]) | 33.3 (4) | 85.7 (18) | 84.2 (16) | .0012 | 40.0 (4) | .041 |
| Immunosuppression (ever) | ||||||
| Yes (% [n]) | 75.0 (9) | 90.5 (19) | 78.9 (15) | .47 | 90.0 (9) | .57 |
| Intermittent prednisone or hydroxychloroquine only (% [n]) | 66.7 (8) | 52.4 (11) | 15.8 (3) | .0082 | 0 | .01 |
| B-cell depletion before diagnosis (% [n]) | 8.7 (1, >5 y) | 0 | 5.3 (1, >5 y) | .47 | 50.0 (5, ongoing) | <.0001 |
| Receiving therapy at diagnosis (% [n]) | 0 | 0 | 0 | — | 70.0 (7) | <.0001 |
| Immunosuppression (around SARS-CoV-2 vaccine) | ||||||
| Yes: any, ≤1 mo before (% [n]) | 25.0 (3) | 23.8 (5) | 36.8 (7) | .64 | 40.0 (4) | .49 |
| Yes: any, ≤1 mo after (% [n]) | 16.7 (2) | 23.8 (5) | 31.6 (6) | .65 | 20.0 (2) | .74 |
| Yes: B-cell depletion, ≤6 mo before to ≤1 mo after (% [n]) | 0 | 0 | 15.8 (3) | .0003 | 50.0 (5) | <.0001 |
IgR, Immunoglobulin replacement; PAD, predominant antibody deficiency; PID, primary immunodeficiency.