Emerging Aspergillus Species Almost Exclusively Associated With Primary Immunodeficiencies

Invasive aspergillosis (IA) is the most serious mold infection encountered in patients with iatrogenic immunosuppression. IA is also a major cause of mortality and morbidity in individuals with primary immunodeficiency (PID). Although is the most common etiologic agent of IA reported in PID patients, followed by , multiple poorly recognized species such as . , and have been reported almost exclusively from patients with inborn defects in host antifungal defense pathways. Infection in PID patients exhibits patterns of disease progression distinct from those in iatrogenic immunosuppression. Specifically, the disease can be extrapulmonary and chronic with a tendency to disseminate in a contiguous manner across anatomical planes. It is also more refractory to standard antifungal therapy. This synopsis summarizes our understanding of emerging rare species that primarily affect patients with PIDs but not those with acquired immunodeficiencies.