title: Pulmonary complications in patients with antibody deficiency.
abstract: OBJECTIVE
  The aim of this study was to evaluate pulmonary complications in patients with primary antibody deficiency (X-linked agammaglobulinaemia [XLA] and common variable immunodeficiency [CVID]).
  
  
  METHODS
  Thirty patients over six years of age regularly followed in a reference out-patient clinic on primary immunodeficiency were studied. All of them have been treated with intravenous immunoglobulin (IVIG) replacement therapy. Pulmonary complications were evaluated analysing clinical data (medical records review), lung function test (spirometry) and pulmonary imaging (chest computed tomography [CCT]).
  
  
  RESULTS
  Patients with normal CCT (N = 14) and those with abnormal CCT (N = 16) have shown no differences regarding the age at onset of symptoms, age of diagnosis, and duration of IVIG treatment. The mean number of pneumonia episodes before IVIG replacement was significantly higher among patients with abnormal CCT (4 vs 7 episodes, p = 0.008). CCT abnormalities observed in 16 patients were: bronchiectasis (12/16); peribronchial thickening (3/16); air trapping (5/16); lung volume reduction (4/16); atelectasis (2/16), follicular bronchiolitis and ground-glass abnormality (2/16) and parenchyma nodule (1/16). Lung function tests showed ventilatory disturbance in 18/30: obstructive pattern in 38.8%, restrictive pattern in 44.4%, and mix pattern in 16.7%. There were no significant differences in lung function between those with and without CCT abnormalities. Negative significant correlations were observed between lung function and number of episodes of pneumonia. Chronic persistent cough was associated with a reduction in lung function.
  
  
  CONCLUSIONS
  Pulmonary complications are not rare in patients with antibody deficiencies and they must be monitored.
authors.affiliation: Division of Allergy, Clinical Immunology and Rheumatology, Department of Pediatrics, Federal University of São Paulo, São Paulo, Brazil.
authors.forename: Beatriz T
authors.initials: BT
authors.lastname: Costa-Carvalho
authors.forename: Gustavo F
authors.initials: GF
authors.lastname: Wandalsen
authors.forename: Guilherme
authors.initials: G
authors.lastname: Pulici
authors.forename: Carolina Sanchez
authors.initials: CS
authors.lastname: Aranda
authors.forename: Dirceu
authors.initials: D
authors.lastname: Solé
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications* / Q000188:drug therapy; D002648:Child; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000188:drug therapy; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008171:Lung Diseases / Q000453:epidemiology* / Q000209:etiology*; D008297:Male; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed; D055815:Young Adult
chemical_list: D016756:Immunoglobulins, Intravenous