Abstract
Background: non-cystic fibrosis bronchiectasis (NCFB) are very frequently condition in patients with primary antibody deficiency. There are a few hypotheses of this co-existence: clinical (infections of respiratory tract, late identification of disease, absence of regular immunoglobulin replacement therapy, etc.) and genetic factors.The aim of work: to analyze clinical factors of bronchiectasis formation in primary antibody deficiency patients.Patients and methods: our local register of patients with primary immunodeficiencies includes 198 cases with confirmed antibody deficiency. NCFB was diagnosed in 22 out of 198 patients (11.1%). NCFB was detected more frequently in patients with agammaglobulinemia (12 out of 14 cases, 85.7%) and common variable immunodeficiency (10 out of 24 cases, 41.6%) in comparison to selective IgA deficiency (none out of 152 cases) (p<0.001 in both cases). The only patient with thymoma with immunodeficiency was free of bronchiectasis.Results: there is no correlation between the duration of primary immunodeficiency disorder, presence of regular immunoglobulin replacement therapy and NCFB formation in patients with primary antibody deficiency (Spearman's rank correlation coefficient r= 0.24 and r= 0.17, correspondingly).Conclusions: clinical factors of co-existence NCFB and primary antibody deficiency are not comprehensive.