Basic and clinical immunologyGranulomatous-lymphocytic lung disease shortens survival in common variable immunodeficiency☆
Section snippets
Study population
After institutional review board approval, the records of 85 patients with a diagnosis of hypogammaglobulinemia seen between 1985 and 2001 at this center were retrospectively reviewed. Demographic, physiologic, radiographic, and immunologic data were abstracted from the medical records. Sixty-nine patients who met the criteria for the definition of CVID11., 12. made up the final cohort of patients. The diagnosis of CVID was made by demonstrating a reduction greater than 2 SDs of the mean in IgG
Pulmonary disorders
The primary pulmonary disorders identified in the entire group are outlined in Table I. Twenty-nine (42%) patients had neither clinically significant chronic respiratory symptoms nor radiologic evidence of pulmonary disease (group 1). Twenty-three (33%) patients had chronic respiratory symptoms without diffuse chest radiographic abnormalities (group 2). All these subjects had airways disease (ie, bronchiectasis or asthma). In line with previous studies, bronchiectasis was the most frequently
Discussion
The purpose of this study was to further characterize the prevalence, clinical findings, and natural history of the noninfectious diffuse parenchymal lung disease complications of CVID. Our most important finding is that patients with GLILD exhibited a reduced survival when compared with patients without this disorder. In the absence of GLILD, median survival was nearly 30 years, whereas in its presence the median survival was reduced by more than 50%.
ILD was common in our patients with CVID.
Acknowledgements
We thank Gabriele Cheatham for secretarial assistance.
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K. K. Brown and J. M. Routes contributed equally to this article.
Supported by a grant from the American Academy of Allergy, Asthma and Immunology and the Immunodeficiency Foundation (to JMR); the Andrew Goodman Fellowship Grant (CAB); and National Heart, Lung, and Blood Institute SCOR HL67671 (to KKB and CDC).