Table 4. [Treatment of Manifestations in Individuals with STAT3 Hyper IgE Syndrome]. - GeneReviews®

Manifestation/ Concern	Treatment	Considerations/Other
Eczema & recurrent boils	Topical antiseptics, e.g., dilute bleach baths ¹ & chlorhexidine; frequent swimming in chlorinated pool	Adequate skin lubrication is needed after bleach.
Anti-staphylococcal prophylaxis, e.g., w/2x/day TMP/SMX
Histamine-1 antagonists (e.g., hydroxyzine) to control pruritus	Helpful for more significant eczema
Recurrent pneumonias	Antibiotic prophylaxis, typically w/2x/day TMP/SMX	Targeting Staphylococcus aureus & other pyogenic bacteria to prevent the pneumonias & their complications
In Coccidioides endemic regions use of prophylactic antifungals (e.g., fluconazole) can be considered.	To prevent disseminated severe infection
If structural damage to the lungs (e.g., bronchiectasis &/or pneumatoceles) has occurred, the breadth of antimicrobial coverage may need to be extended, incl antifungals, as these structural abnormalities become secondarily infected w/gram-negative bacteria (e.g., Pseudomonas) or fungi (e.g., Aspergillus). In the setting of bronchiectasis, consideration of azithromycin to prevent exacerbations if no mycobacterial infection present	Sputum samples for bacteria, fungi, & mycobacteria (typically nontuberculous) should be obtained for microbiology during lung infections to help guide antimicrobial choice.
Intravenous or subcutaneous IgG replacement can be considered.	Has been used w/anecdotal improvement for some individuals, esp those who fail to make protective levels of specific antibodies following vaccination challenge; prospective, randomized controlled studies of immunoglobulin supplementation have not been performed.
Airway clearance w/bronchiectasis	Airway clearance techniques incl airway clearance devices, hypertonic saline nebulizers.
Chronic mucocutaneous candidiasis	Antifungal prophylaxis	Consider fluconazole prophylaxis if living in a Coccidioides endemic region.
Osteoporosis & Minimal trauma fractures	Optimize calcium & vitamin D intake	The role of bisphosphonates for those w/this disorder w/osteoporosis is unclear; some improvement seen in bone density but unclear improvement in fractures [Sowerwine et al 2014].
Arterial aneurysms	Optimal blood pressure mgmt
Antiplatelet or anticoagulation therapies may be considered for individuals w/significant coronary artery aneurysms to prevent myocardial infarction related to clotting w/in the aneurysm.	This must be weighed against risk of hemoptysis, a complication of fungal or bacterial lung disease in persons w/STAT3-HIES.

Manifestation/
Concern

Treatment

Considerations/Other

Eczema &
recurrent boils

Topical antiseptics, e.g., dilute bleach baths ¹ & chlorhexidine; frequent swimming in chlorinated pool

Adequate skin lubrication is needed after bleach.

Anti-staphylococcal prophylaxis, e.g., w/2x/day TMP/SMX

Histamine-1 antagonists (e.g., hydroxyzine) to control pruritus

Helpful for more significant eczema

Recurrent
pneumonias

Antibiotic prophylaxis, typically w/2x/day TMP/SMX

Targeting Staphylococcus aureus & other pyogenic bacteria to prevent the pneumonias & their complications

In Coccidioides endemic regions use of prophylactic antifungals (e.g., fluconazole) can be considered.

To prevent disseminated severe infection

If structural damage to the lungs (e.g., bronchiectasis &/or pneumatoceles) has occurred, the breadth of antimicrobial coverage may need to be extended, incl antifungals, as these structural abnormalities become secondarily infected w/gram-negative bacteria (e.g., Pseudomonas) or fungi (e.g., Aspergillus).
In the setting of bronchiectasis, consideration of azithromycin to prevent exacerbations if no mycobacterial infection present

Sputum samples for bacteria, fungi, & mycobacteria (typically nontuberculous) should be obtained for microbiology during lung infections to help guide antimicrobial choice.

Intravenous or subcutaneous IgG replacement can be considered.

Has been used w/anecdotal improvement for some individuals, esp those who fail to make protective levels of specific antibodies following vaccination challenge; prospective, randomized controlled studies of immunoglobulin supplementation have not been performed.

Airway clearance w/bronchiectasis

Airway clearance techniques incl airway clearance devices, hypertonic saline nebulizers.

Chronic mucocutaneous
candidiasis

Antifungal prophylaxis

Consider fluconazole prophylaxis if living in a Coccidioides endemic region.

Osteoporosis &
Minimal trauma
fractures

Optimize calcium & vitamin D intake

The role of bisphosphonates for those w/this disorder w/osteoporosis is unclear; some improvement seen in bone density but unclear improvement in fractures [Sowerwine et al 2014].

Arterial
aneurysms

Optimal blood pressure mgmt

Antiplatelet or anticoagulation therapies may be considered for individuals w/significant coronary artery aneurysms to prevent myocardial infarction related to clotting w/in the aneurysm.

This must be weighed against risk of hemoptysis, a complication of fungal or bacterial lung disease in persons w/STAT3-HIES.

From: STAT3 Hyper IgE Syndrome

GeneReviews^® [Internet].

Adam MP, Feldman J, Mirzaa GM, et al., editors.

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