Respiratory dysfunction in patients with common variable hypogammaglobulinemia

Patients with common variable hypogammaglobulinemia (CVH) are susceptible to recurrent respiratory infections. We studied the prevalence and severity of respiratory dysfunction in 32 patients with CVH by measuring routine pulmonary function tests (PFT) and interpreting chest radiographs using a quantitative score system. Pulmonary symptoms were determined by patient interview and standard questionnaire. The mean age of 29 living patients was 28 +/- 2.2 yr; there were 22 males and 10 females. The mean age at diagnosis was 17.3 +/- 2.4 yr. Sinopulmonary symptoms were present in all 32 patients and included chronic sinusitis, otitis, bronchitis, recurrent pneumonia, and bronchiectasis. Complete PFT in 25 patients revealed restrictive, obstructive, and normal patterns in 10, 6, and 9 patients, respectively. Nine patients had dyspnea on exertion. Recent chest radiographs in 32 patients showed evidence of recurrent pulmonary infection, including air trapping, linear markings, and pleural abnormalities. Panlobular emphysema, documented in one case at autopsy, was suggested in 5 other patients by the presence of lower lobe bullous lesions and marked lower lobe hyperlucency on chest radiographs. The forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1) had remained stable in 19 adults (18 receiving adequate immunoglobulin replacement therapy) for 6.9 +/- 0.7 yr. The expected increase of the FEV1 and FVC with growth was normal in 6 of 8 children (all 8 receiving adequate therapy). Chest radiograph scores deteriorated only slightly from 20.3 +/- 0.6 to 18.4 +/- 0.8 (25 = normal) during the 6.9 +/- 0.7 yr in 30 patients (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)