High resolution computed tomography and pulmonary function in common variable immunodeficiency

Abstract

Patients with common variable immunodeficiency (CVID) have impaired production of immunoglobulins and hence recurrent airway infections, which in turn may lead to radiological changes and impaired lung function. Uncertainty exists about the nature and frequency of the radiological and the physiological abnormalities, and how they relate to each other. We reassessed high resolution computed tomography (HRCT) images in 65 patients, reported results from previously measured lung function tests, and studied relations between radiology, function and clinical variables. Airway obstruction, ventilatory restriction and impaired gas diffusion was found in 40, 34 and 21% of the patients, respectively. HRCT abnormalities were present in 94% of the subjects, mild changes being the most common. Bronchial wall thickening, found in two thirds of the patients, was related to airway obstruction and impaired gas diffusion. Linear and/or irregular opacities, the most frequent interstitial abnormality, was related to impaired gas diffusion. Bronchiectasis was found in more than half, but only severe bronchiectasis was related to airway obstruction. Since bronchial wall thickening and linear and/or irregular opacities are both frequent and important determinants of impaired pulmonary function, more attention should be given to these features in the follow up of CVID patients.

High-resolution computed tomography and pulmonary function in children with common variable immunodeficiency

“…For air trapping, we demonstrated that visual, as well as quantitative, HRCT measurements correlate significantly with PFTs and disease duration. For visual scoring of air trapping, previous studies have also found a significant correlation with PFTs in CVID patients [20]. These data and our data indicate that HRCT-diagnosed air trapping is related to relevant airway obstruction in these patients.…”

Human Plasma-derived Polymeric IgA and IgM Antibodies Associate with Secretory Component to Yield Biologically Active Secretory-like Antibodies

“…In patients with primary or secondary immunodeficiency, replacement therapy with IgG effectively prevents severe systemic infection. However, despite appropriate IgG supplementation, frequently occurring chronic infection/inflammation of the respiratory and gastrointestinal mucosae has been linked to low serum IgA levels (42)(43)(44). The inherent structural features of SIgA would make it a logical agent to fill this therapeutic gap in combination with standard therapy (e.g.…”

Small airways diseases, excluding asthma and COPD: an overview

“…In a cohort of 65 patients, correlation was found between bronchial wall thickening, mucoid impaction and mosaic lung attenuation on the one hand and airway obstruction (FEV1 and FEV1/FVC) on the other [167]. Low diffusing capacity of the lung for carbon monoxide (DLCO) was associated with bronchial wall thickening and linear opacities.…”