Antimicrobial prevention in IEI
| IEI/Disease process . | Antimicrobial regimens . | Comments . |
|---|---|---|
| SCID | TMP/SMX for PJP prophylaxis starting at 1 mo; second line agents include pentamidine, atovaquone, and dapsone Fluconazole for Candida prophylaxis Antivirals dependent on risks (2) RSV monoclonal antibody seasonally IgRT within a few weeks of life, trough levels >800 mg/dl | Monitor bilirubin and blood counts on TMP/SMX Folinic acid to minimize cytopenias Monitor liver function tests on fluconazole Avoid breastfeeding if mother CMV IgG positive Irradiated blood products Avoid live vaccines (rotavirus, MMR, varicella, and BCG) |
| CGD | TMP/SMX twice daily for bacterial prophylaxis Itraconazole for fungal prophylaxis; other mold-active triazoles as alternative include posaconazole Consideration of IFNγ | Remember corticosteroid/azole interaction Environmental considerations to minimize mold/water exposures Diagnosis of lung infections preferably with biopsy Some infections (i.e., Nocardia pneumonias, S. aureus liver abscess) have better response if corticosteroids added to antimicrobials |
| HIES | TMP/SMX twice daily for bacterial prophylaxis (consider alternatives such as doxycycline if resistant organisms) Fluconazole for frequent Candida infections and in Coccidioides endemic regions Itraconazole if pneumatocele is present, posaconazole if chronic mold infection Consider IgRT | Avoid lung surgeries and lung biopsies if possible due to risk of prolonged bronchopleural fistulae Consider dupilumab for eczematoid dermatitis; can also reduce skin infections Antiseptics (dilute bleach baths, chlorhexidine) to minimize S. aureus skin colonization Low threshold to look for infection as systemic signs of inflammation are diminished |
| XLA | IgRT with optimization of trough Consider azithromycin Influenza and SARS CoV2 vaccination can maximize responses through T cell–dependent mechanisms | Low threshold to consider Campylobacter/Helicobacter with persistent fevers, lower extremity erythema/bruise-like and boggy skin changes Low threshold to look for chronic enterovirus with CNS symptoms Consider Aichi virus with nephritis, hepatitis |
| Bronchiectasis | Consider azithromycin after sputum cultures for Mycobacteria Airway clearance techniques Consider of inhaled antimicrobials | Check EKG for QTc with addition of azithromycin and consider hearing screen Periodic sputum cultures to be aware of colonization and optimal antimicrobials if needed Caution with inhaled tobramycin if renal insufficiency |
| MSMD | Prophylaxis with azithromycin after NTM infections treated Consideration of IFNγ for treatment in addition to antimicrobials with certain defects (IL12RB1 deficiency, AD IFNγR1 deficiency, NEMO, and IkBα) | Avoid BCG vaccination For complete IFNγR deficiencies, after control of NTM with combination antimicrobials, HSCT is indicated Some risk of endemic mycoses, therefore minimize environmental exposures and in Coccidioides endemic regions consider prophylactic azoles |
| Perioperative | Consideration of S. aureus therapies perioperatively with longer course if hardware placed (7–10 days) | S. aureus decolonization with nasal mupirocin, antiseptics |
| Women’s health | Many restrictions of antimicrobials during pregnancy—consider azithromycin for prophylaxis and topical antifungals if needed | Close communication with Obstetrician and IEI practitioner |
| IEI/Disease process . | Antimicrobial regimens . | Comments . |
|---|---|---|
| SCID | TMP/SMX for PJP prophylaxis starting at 1 mo; second line agents include pentamidine, atovaquone, and dapsone Fluconazole for Candida prophylaxis Antivirals dependent on risks (2) RSV monoclonal antibody seasonally IgRT within a few weeks of life, trough levels >800 mg/dl | Monitor bilirubin and blood counts on TMP/SMX Folinic acid to minimize cytopenias Monitor liver function tests on fluconazole Avoid breastfeeding if mother CMV IgG positive Irradiated blood products Avoid live vaccines (rotavirus, MMR, varicella, and BCG) |
| CGD | TMP/SMX twice daily for bacterial prophylaxis Itraconazole for fungal prophylaxis; other mold-active triazoles as alternative include posaconazole Consideration of IFNγ | Remember corticosteroid/azole interaction Environmental considerations to minimize mold/water exposures Diagnosis of lung infections preferably with biopsy Some infections (i.e., Nocardia pneumonias, S. aureus liver abscess) have better response if corticosteroids added to antimicrobials |
| HIES | TMP/SMX twice daily for bacterial prophylaxis (consider alternatives such as doxycycline if resistant organisms) Fluconazole for frequent Candida infections and in Coccidioides endemic regions Itraconazole if pneumatocele is present, posaconazole if chronic mold infection Consider IgRT | Avoid lung surgeries and lung biopsies if possible due to risk of prolonged bronchopleural fistulae Consider dupilumab for eczematoid dermatitis; can also reduce skin infections Antiseptics (dilute bleach baths, chlorhexidine) to minimize S. aureus skin colonization Low threshold to look for infection as systemic signs of inflammation are diminished |
| XLA | IgRT with optimization of trough Consider azithromycin Influenza and SARS CoV2 vaccination can maximize responses through T cell–dependent mechanisms | Low threshold to consider Campylobacter/Helicobacter with persistent fevers, lower extremity erythema/bruise-like and boggy skin changes Low threshold to look for chronic enterovirus with CNS symptoms Consider Aichi virus with nephritis, hepatitis |
| Bronchiectasis | Consider azithromycin after sputum cultures for Mycobacteria Airway clearance techniques Consider of inhaled antimicrobials | Check EKG for QTc with addition of azithromycin and consider hearing screen Periodic sputum cultures to be aware of colonization and optimal antimicrobials if needed Caution with inhaled tobramycin if renal insufficiency |
| MSMD | Prophylaxis with azithromycin after NTM infections treated Consideration of IFNγ for treatment in addition to antimicrobials with certain defects (IL12RB1 deficiency, AD IFNγR1 deficiency, NEMO, and IkBα) | Avoid BCG vaccination For complete IFNγR deficiencies, after control of NTM with combination antimicrobials, HSCT is indicated Some risk of endemic mycoses, therefore minimize environmental exposures and in Coccidioides endemic regions consider prophylactic azoles |
| Perioperative | Consideration of S. aureus therapies perioperatively with longer course if hardware placed (7–10 days) | S. aureus decolonization with nasal mupirocin, antiseptics |
| Women’s health | Many restrictions of antimicrobials during pregnancy—consider azithromycin for prophylaxis and topical antifungals if needed | Close communication with Obstetrician and IEI practitioner |