Bronchiectasis in children with primary immune deficiency diseases

Background: Primary immune deficiencies are a common cause of bronchiectasis in children. Aim of the study: The aim of this study was to evaluate the incidence and characteristic radiological findings of bronchiectasis in children with primary immune deficiencies. Material and methods: A retrospective review of medical records and pulmonary imaging (chest radiographs and high resolution computed tomography scans) of forty-one patients with primary immune deficiency diseases, aged between 6 months and 18 years was undertaken. Results: Based on high resolution computer tomography (HRCT) imaging, the diagnosis of bronchiectasis was established in 5 patients (12.2%), with Bruton's agammaglobulinemia (XLA), common variable immunodeficiency (CVID) and the hyperimmunoglobulin E syndrome (HIES). In 3 children (7.2%), two with IgG subclass deficiencies (IgGsD) and in one with HIES, pre-bronchiectasis was demonstrated. The HRCT features of bronchiectasis were predominatingly detected in the basilar segments of the lower lobes, followed by the right middle lobe and the lingula. The median age of 5 children with bronchiectasis visualized in HRCT imaging was 13 years. Pyogenic bacteria were recovered in the respiratory tract of all the children with bronchiectasis, with Streptococcus pneumoniae most frequently identified. Conclusions: Bronchiectasis is a frequent pulmonary complication and should be actively looked for in children with primary immune deficiencies and lung infections.