title: Infections in 252 patients with common variable immunodeficiency.
abstract: BACKGROUND
  Common variable immunodeficiency is characterized by recurrent infections and defective immunoglobulin production.
  
  
  METHODS
  The DEFI French national study prospectively enrolled adult patients with primary hypogammaglobulinemia. Clinical events before inclusion were retrospectively analyzed at that time.
  
  
  RESULTS
  From April 2004 through April 2007, 341 patients were enrolled, 252 of whom had received a diagnosis of common variable immunodeficiency; of those, 110 were male, 142 were female, and 228 were white. The median age at first symptoms was 19 years. The median age at common variable immunodeficiency diagnosis was 33.9 years. The median delay for diagnosis was 15.6 years for the 138 patients with initial symptoms before 1990 and 2.9 years for the 114 patients with initial symptoms from 1990 to the time of the study. The most frequent initial symptoms were upper respiratory tract infections: bronchitis (in 38% of patients), sinusitis (36%), pneumonia (31%), and/or bronchiectasis (14%). Overall, 240 patients had respiratory symptoms. Pneumonia was reported in 147 patients; Streptococcus pneumoniae and Haemophilus influenzae were documented in 46 and 17 cases, respectively. Recurrent or chronic diarrhea was reported in 118 patients. Giardia (35 cases), Salmonella (19), and Campylobacter (19) infections were more frequent in patients with undetectable serum immunoglobulin A (P<.001). Sixteen patients developed opportunistic infections. Persistent infections and requirement for antibiotics despite immunoglobulin substitution correlated with severe defect of memory switched B cells (P=.003) but not with immunoglobulin G trough levels (P=.55).
  
  
  CONCLUSIONS
  Although reduced within the past decade, the delay of diagnosis of common variable immunodeficiency remains unacceptable. Recurrence of upper respiratory tract infection or pneumonia should lead to systematic evaluation of serum immunoglobulin.
authors.affiliation: Department of Clinical Immunology, Hôpital Saint-Louis, Paris, France.
authors.forename: Eric
authors.initials: E
authors.lastname: Oksenhendler
authors.forename: Laurence
authors.initials: L
authors.lastname: Gérard
authors.forename: Claire
authors.initials: C
authors.lastname: Fieschi
authors.forename: Marion
authors.initials: M
authors.lastname: Malphettes
authors.forename: Gael
authors.initials: G
authors.lastname: Mouillot
authors.forename: Roland
authors.initials: R
authors.lastname: Jaussaud
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Viallard
authors.forename: Martine
authors.initials: M
authors.lastname: Gardembas
authors.forename: Lionel
authors.initials: L
authors.lastname: Galicier
authors.forename: Nicolas
authors.initials: N
authors.lastname: Schleinitz
authors.forename: Felipe
authors.initials: F
authors.lastname: Suarez
authors.forename: Pauline
authors.initials: P
authors.lastname: Soulas-Sprauel
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: Arnaud
authors.initials: A
authors.lastname: Jaccard
authors.forename: Anaelle
authors.initials: A
authors.lastname: Gardeur
authors.forename: Ioannis
authors.initials: I
authors.lastname: Théodorou
authors.forename: Claire
authors.initials: C
authors.lastname: Rabian
authors.forename: Patrice
authors.initials: P
authors.lastname: Debré
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002648:Child; D017074:Common Variable Immunodeficiency / Q000150:complications*; D003141:Communicable Diseases / Q000453:epidemiology / Q000209:etiology*; D003967:Diarrhea / Q000453:epidemiology / Q000209:etiology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D008875:Middle Aged; D011014:Pneumonia / Q000453:epidemiology / Q000209:etiology; D015995:Prevalence; D012141:Respiratory Tract Infections / Q000453:epidemiology / Q000209:etiology; D013997:Time Factors
chemical_list: D000900:Anti-Bacterial Agents; D007074:Immunoglobulin G