Pulmonary abnormalities in patients with primary hypogammaglobulinemia

J Allergy Clin Immunol. 1999 Nov;104(5):1031-6. doi: 10.1016/s0091-6749(99)70085-0.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Agammaglobulinemia / complications*
  • Agammaglobulinemia / therapy
  • Aged
  • Bronchiectasis / complications
  • Bronchiectasis / diagnosis
  • Child
  • Common Variable Immunodeficiency / complications*
  • Common Variable Immunodeficiency / therapy
  • Female
  • Follow-Up Studies
  • Humans
  • Lung / abnormalities*
  • Lung / diagnostic imaging
  • Lung Diseases, Obstructive / complications
  • Lung Diseases, Obstructive / diagnosis
  • Male
  • Middle Aged
  • Prospective Studies
  • Radiography, Thoracic
  • Respiratory Function Tests
  • Tomography, X-Ray Computed