title: Comparison of pulmonary diseases in common variable immunodeficiency and X-linked agammaglobulinaemia.
abstract: Patients with CVID are at greater risk of developing lung complications than patients with XLA because of delayed diagnosis and possible immune dysregulation. Early diagnosis and appropriate treatment reduces the incidence of pulmonary infections in both groups of patients. However, CVID patients are prone to progressive lung disease despite optimal immunoglobulin therapy.
  
  
  OBJECTIVE
  Pulmonary disease is the most common complication in patients with common variable immunodeficiency (CVID) or X-linked agammaglobulinaemia (XLA). Pulmonary disease may progress despite immunoglobulin replacement therapy. In this study pulmonary complications were compared in patients with CVID or XLA.
  
  
  METHODS
  Pulmonary complications were evaluated in 115 patients (76 with CVID and 39 with XLA) by reviewing hospital records of chest infections, pulmonary function tests and high-resolution CT scans.
  
  
  RESULTS
  Thirty-two patients with XLA (82%) presented with 59 episodes of pneumonia before diagnosis, whereas 15 patients (38.4%) experienced pneumonia after immunoglobulin replacement therapy (1.67 vs 0.45 episodes per patient per year). Among the CVID patients, 196 episodes of pneumonia were documented in 59 patients (77.6%) before diagnosis, while 36 patients (47.3%) experienced pneumonia after therapy (1.11 vs 0.58 episodes of pneumonia per patient per year). Forty-seven (41%) patients (38 with CVID and 9 with XLA) developed chronic lung disease. The CVID patients developed more complications, including bronchiectasis and lymphoid interstitial pneumonitis, than the XLA patients.
  
  
  CONCLUSIONS
  Patients with CVID had a greater likelihood of developing lung disease, possibly due to delayed diagnosis and immune dysregulation, as compared with XLA patients. Early diagnosis of patients with primary antibody deficiencies and adequate i.v. immunoglobulin replacement therapy substantially reduces the number of pulmonary infections. However, CVID patients are prone to progression of lung disease despite optimal immunoglobulin therapy because of the nature of the disease. This important issue should be addressed in further studies.
authors.affiliation: Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. aghamohammadi@sina.tums.ac.ir
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
authors.forename: Abdolreza
authors.initials: A
authors.lastname: Allahverdi
authors.forename: Hassan
authors.initials: H
authors.lastname: Abolhassani
authors.forename: Kasra
authors.initials: K
authors.lastname: Moazzami
authors.forename: Hooman
authors.initials: H
authors.lastname: Alizadeh
authors.forename: Mohammad
authors.initials: M
authors.lastname: Gharagozlou
authors.forename: Najmoddin
authors.initials: N
authors.lastname: Kalantari
authors.forename: Vahid
authors.initials: V
authors.lastname: Sajedi
authors.forename: Alireza
authors.initials: A
authors.lastname: Shafiei
authors.forename: Nima
authors.initials: N
authors.lastname: Parvaneh
authors.forename: Masoud
authors.initials: M
authors.lastname: Mohammadpour
authors.forename: Nasser
authors.initials: N
authors.lastname: Karimi
authors.forename: Mohammad Salehi
authors.initials: MS
authors.lastname: Sadaghiani
authors.forename: Nima
authors.initials: N
authors.lastname: Rezaei
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D001987:Bronchiectasis / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D018450:Disease Progression; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D007116:Immunization, Passive; D008171:Lung Diseases / Q000453:epidemiology*; D017563:Lung Diseases, Interstitial / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011014:Pneumonia / Q000453:epidemiology; D015995:Prevalence; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012307:Risk Factors; D055815:Young Adult