Table 3 Strategies for the treatment and management of PIDs
From: Primary immunodeficiency
| Supportive | Definitive | |
|---|---|---|
| CIDs/SCID |
Ig replacement therapy (IV or SC) Antibiotic prophylaxis Antifungal prophylaxis Aggressive management of established infections Infectious precautions when hospitalized Withhold all live vaccines |
BMT HSCT Gene therapy a possibility for some SCIDs |
| B-cell disorders |
Ig replacement therapy (IV or SC) Antibiotic prophylaxis Antifungal prophylaxis depending upon etiology Hearing assessment Assessment of pulmonary status and function Close monitoring for co-morbidities | Gene therapy is a potential future treatment in some patients |
| Innate disorders |
Antibiotic prophylaxis Antifungal prophylaxis Cytokine replacement (IFNγ) for CGD Vaccinations (e.g., meningococcal) Ig replacement is sometimes indicated |
BMT, e.g., for CGD Gene therapy is a potential future treatment |