Table 3

Strategies for the treatment and management of PIDs.

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

Ig, immunoglobulin; IV: intravenous; SC, subcutaneous; CID, combined immunodeficiency; SCID, severe combined immunodeficiency; IFNγ, interferon-gamma; BMT, bone marrow transplantation; CGD, chronic granulomatous disease; HSCT, hematopoietic stem cell transplantation

McCusker and Warrington Allergy, Asthma & Clinical Immunology 2011 7(Suppl 1):S11   doi:10.1186/1710-1492-7-S1-S11