Table 3 Strategies for the treatment and management of PIDs
From: Primary immunodeficiency
| Â | 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 |