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J Allergy Clin Immunol. 1999 Nov;104(5):1031-6.

Pulmonary abnormalities in patients with primary hypogammaglobulinemia.

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1
Department of Pediatrics, Turku University Hospital, Turku, Finland.

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.

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PMID:
10550749
[Indexed for MEDLINE]

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