title: Epidemiology and outcome of invasive fungal diseases in patients with chronic granulomatous disease: a multicenter study in France.
abstract: BACKGROUND
  Chronic granulomatous disease (CGD) is a rare inherited phagocytic disorder resulting in an increased susceptibility to infections including invasive fungal diseases (IFDs) and inflammatory complications. This study is aimed at assessing the incidence, prevalence, and outcome of IFDs among CGD patients followed in France.
  
  
  METHODS
  CGD patients were identified through the French national registry for primary immunodeficiencies (PID) held by the French national reference Centre of PID (Centre de Référence Déficits Immunitaires Héréditaires), which comprises a total of 3083 patients including 155 with CGD followed between 1976 and 2008. A questionnaire was filled out for each episode of IFD. Information retrieved included a description of the IFD using the 2008 European Organization for Research and Treatment of Cancer/Mycoses Study Group IFD definition criteria.
  
  
  RESULTS
  Of CGD patients, 42.6% (66/155) developed at least 1 episode of IFD. Overall incidence of IFD was 0.040/patient-years (1862 patient-years of total follow-up). IFD incidence was found to be significant while receiving itraconazole prophylaxis compared with no prophylaxis (0.027 vs. 0.053 IFD/patient-years; P < 0.01). Median age at IFD diagnosis was 6.5 years (3.3-11.3). The most common fungal genus was Aspergillus sp. accounting for 40% of all IFDs. Of the IFDs, 42.5% were proven, 30.0% probable, and 27.5% possible. Of all IFD episodes, 52.5% were treated by antifungal monotherapy, mostly by amphotericin B. Survival was reduced in IFD patients compared with those without it (log-rank 0.04).
  
  
  CONCLUSIONS
  IFDs are a frequent and life-threatening complication in CGD patients. Itraconazole significantly reduces its incidence and should be recommended in absence of better alternatives.
authors.affiliation: Centre de Référence Déficits Immunitaires Héréditaires, Hôpital Necker-Enfants Malades, AP-HP, Paris, France.
authors.forename: Julien
authors.initials: J
authors.lastname: Beauté
authors.forename: Gaëlle
authors.initials: G
authors.lastname: Obenga
authors.forename: Loïc
authors.initials: L
authors.lastname: Le Mignot
authors.forename: Nizar
authors.initials: N
authors.lastname: Mahlaoui
authors.forename: Marie-Elisabeth
authors.initials: ME
authors.lastname: Bougnoux
authors.forename: Richard
authors.initials: R
authors.lastname: Mouy
authors.forename: Marie-Anne
authors.initials: MA
authors.lastname: Gougerot-Pocidalo
authors.forename: Vincent
authors.initials: V
authors.lastname: Barlogis
authors.forename: Felipe
authors.initials: F
authors.lastname: Suarez
authors.forename: Fanny
authors.initials: F
authors.lastname: Lanternier
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
authors.forename: Marc
authors.initials: M
authors.lastname: Lecuit
authors.forename: Stéphane
authors.initials: S
authors.lastname: Blanche
authors.forename: Alain
authors.initials: A
authors.lastname: Fischer
authors.forename: Olivier
authors.initials: O
authors.lastname: Lortholary
mesh_terms: D000293:Adolescent; D000935:Antifungal Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D005602:France / Q000453:epidemiology; D006105:Granulomatous Disease, Chronic / Q000453:epidemiology* / Q000382:microbiology* / Q000628:therapy; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007223:Infant; D017964:Itraconazole / Q000627:therapeutic use; D008297:Male; D009181:Mycoses / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology*; D012042:Registries; D011795:Surveys and Questionnaires; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000935:Antifungal Agents; D017964:Itraconazole