Chapter 51 - Prevention and Management of Infections

Abstract

The hallmark of primary immunodeficiencies is infection susceptibility. While it is known that acute and chronic infections lead to morbidity, such as bronchiectasis, the proven role of prophylactic antimicrobials is poorly studied and remains largely driven by anxiety, belief, and experience, the latter usually derived from more common diseases, like HIV. In common infection susceptibility states, guidelines exist and, increasingly, are evidence-based. Studies abound for prophylactic antibiotics in fever and neutropenia after chemotherapy, for urinary tract infection prevention in the setting of vesico-ureteral reflux, and for antifungal prophylaxis in premature infants. Primary immunodeficiencies, on the other hand, are rare, and for the most part lack evidence-based guidelines. Therefore, approaches from more common immunodeficiencies, like HIV or transplant, are often used. However, this is necessarily likely to be inappropriate, since each immunodeficiency has its own specific infection susceptibility. This chapter discusses antibiotic prophylaxis across several of the more common primary immunodeficiencies, taking into account their specific susceptibility, available data, and prudence.