Abstract
Our local register of patients with primary immunodeficiencies includes 162 cases with confirmed antibody deficiency. Among them there are 95 children with median age of 8.7 years and 67 adults with median age of 28.6 years. The register covers the population of Sverdlovsk region of Russian population with 4.8 mln inhabitants. Non-cystic fibrosis bronchiectasis was diagnosed in 20 out of 162 patients (12.3%). Non-cystic fibrosis bronchiectasis was detected more frequently in patients with agammaglobulinemia (in 12 out of 14 cases, 85.7%) and common variable immunodeficiency (in 8 out of 20 cases, 34.8%) in comparison to selective IgA deficiency (none out of 124 cases) (p<0.001 in both cases). The only patient with thymoma with immunodeficiency was free of bronchiectasis. Among 20 patients with bronchiectasis there were 4 children aged from 6 to 14 years (median 7.2) and 16 adults aged from 18 to 62 years (median 38.6). Thus, our data revealed high rate of non-cystic fibrosis bronchiectasis in agammaglobulinemia and common variable immunodeficiency patients. Further research is needed to determine the etiology of bronchiectasis in these groups of patients, including genetic predisposition and microbial landscape.
- © 2014 ERS