title: Invasive fungal infection in chronic granulomatous disease:  insights into pathogenesis and management.
abstract: OBJECTIVE
  Invasive fungal infections (IFIs) remain a major cause of death in patients with chronic granulomatous disease (CGD). We discuss the new insights into the pathogenesis, diagnosis, prevention, and management of invasive fungal infections in patients with CGD.
  
  
  RESULTS
  CGD has the highest prevalence of IFIs among the immunodeficiencies. Infections typically involve the lung, and the most commonly isolated pathogen is Aspergillus spp. However, IFIs due to rare opportunistic filamentous fungi are increasingly reported. Most IFIs are diagnosed on routine chest imaging, and serum markers such as galactomannan and 1,3-β-D-glucan are of limited value in CGD. Routine use of itraconazole for prophylaxis continues to be recommended, although posaconazole may be an alternative. Management of IFIs is typically centered on prolonged courses of antifungal therapy. Surgery may be required for complete resolution, especially in the setting of osteomyelitis or infections due to Aspergillus nidulans or other poorly responsive molds. Hematopoietic stem cell transplantation (HSCT) cures CGD and may be appropriate in select patients with refractory IFIs.
  
  
  CONCLUSIONS
  Management of IFIs in CGD has significantly improved over the last decade. Earlier diagnosis of IFIs, accurate identification of pathogens, and development of reliable susceptibility testing are areas for future emphasis. HSCT is a promising therapy, even during refractory infections in CGD.
authors.affiliation: Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892-1684, USA.
authors.forename: E Liana
authors.initials: EL
authors.lastname: Falcone
authors.forename: Steven M
authors.initials: SM
authors.lastname: Holland
mesh_terms: D000935:Antifungal Agents / Q000627:therapeutic use; D006105:Granulomatous Disease, Chronic / Q000150:complications*; D006801:Humans; D017964:Itraconazole / Q000627:therapeutic use; D009181:Mycoses / Q000175:diagnosis / Q000473:pathology* / Q000517:prevention & control / Q000628:therapy; D013514:Surgical Procedures, Operative / Q000379:methods; D014230:Triazoles / Q000627:therapeutic use
chemical_list: D000935:Antifungal Agents; D014230:Triazoles; D017964:Itraconazole; C101425:posaconazole