title: Pulmonary abnormalities in patients with primary hypogammaglobulinemia.
abstract: BACKGROUND
  Pulmonary complications are common in patients with primary hypogammaglobulinemia. Intravenous immunoglobulin replacement therapy has been thought to reduce the occurrence of pulmonary complications, yet they do occur.
  
  
  OBJECTIVE
  The purpose of this study was to evaluate pulmonary abnormalities in 22 patients with primary hypogammaglobulinemia (18 with common variable immunodeficiency, 4 with X-linked agammaglobulinemia) and to conduct a prospective 3-year follow-up study to assess the possible progression of pulmonary abnormalities.
  
  
  METHODS
  Pulmonary changes were evaluated with use of pulmonary imaging (chest radiographs, high-resolution computed tomography), and pulmonary function testing.
  
  
  RESULTS
  High-resolution computed tomography revealed pulmonary abnormalities in 21 patients. Bronchiectasis was present in 16 patients, whereas chest radiographs revealed bronchiectasis in only 3 patients. Pulmonary function testing showed obstruction in 5 patients. A prospective 3-year follow-up was conducted in 14 patients. It showed silent progression of bronchiectasis in 5 of the 14 patients, all of whom were receiving intravenous immunoglobulin replacement therapy and had preinfusion serum IgG concentrations of 5 g/L or more.
  
  
  CONCLUSIONS
  Pulmonary abnormalities develop in most patients with primary hypogammaglobulinemia. A new finding is that silent and asymptomatic progression of pulmonary changes may occur in patients despite an adequate immunoglobulin replacement therapy. High-resolution computed tomography is the method of choice in monitoring pulmonary changes.
authors.affiliation: Department of Pediatrics, Turku University Hospital, Turku, Finland.
authors.forename: L
authors.initials: L
authors.lastname: Kainulainen
authors.forename: M
authors.initials: M
authors.lastname: Varpula
authors.forename: K
authors.initials: K
authors.lastname: Liippo
authors.forename: E
authors.initials: E
authors.lastname: Svedström
authors.forename: J
authors.initials: J
authors.lastname: Nikoskelainen
authors.forename: O
authors.initials: O
authors.lastname: Ruuskanen
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications* / Q000628:therapy; D000368:Aged; D001987:Bronchiectasis / Q000150:complications / Q000175:diagnosis; D002648:Child; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008168:Lung / Q000002:abnormalities* / Q000000981:diagnostic imaging; D008173:Lung Diseases, Obstructive / Q000150:complications / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D013902:Radiography, Thoracic; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed