33
Table 10A. Management of Lung Disease
Manifestation
Associated
IEI
Intervention/
management
Strength of
recommendation
Certainty
of evidence
Granulomatous
and
lymphocytic
interstitial
lung disease
(GLILD)
Antibody
deficiency
Combination
treatment: B-cell−
depleting therapy
(e.g., rituximab)
+ antimetabolite
therapy
(mycophenolate
mofetil or
azathioprine)
Strong Moderate
High-dose
corticosteroids
Conditional Moderate
Pneumatocele
aer lung
infection
STAT3-
HIES
Antimicrobial
treatment
Strong Moderate
Avoid surgical
approach if possible
Bronchiectasis Antibody
deficiencies
Avoid surgical
approach if possible
Strong Moderate
Periodic, at least
annual, imaging of
lungs with high-
resolution computed
tomography (CT)
scan
Maintain high trough
serum IgG levels
Airway secretion
clearance measures
