Pulmonary abnormalities in patients with primary hypogammaglobulinemia

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. zi prospective 3-year follow-up was conducted in IJ 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 gn 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.