How I Treat: Infections and inborn errors of immunity-Prevention, diagnosis, and treatment

Infections are a significant cause of morbidity and mortality in inborn errors of immunity (IEI). Prevention of infections relies on knowledge of the specific pathogen susceptibility of the IEI to then consider antimicrobial prophylaxis, immune modulation, and/or environmental measures. In addition, complications of IEI include bronchiectasis, which can lead to more infections due to disruption of mucociliary clearance; suppression of chronic infections and airway clearance measures are important parts of bronchiectasis management. We discuss some infections that are challenging to diagnose for patients with distinct IEI and how partnership with the microbiology laboratory can lead to more prompt diagnosis and specific management. Finally, the increasing ability to identify the molecular basis of IEI facilitates precise immunomodulation, which can attenuate the infection burden of these diseases yet can also add unique infection risks depending on the specific therapy.