Multiple faces of non-cystic fibrosis bronchiectasis: defining etiology and disease severity

Abstract

Non-cystic fibrosis bronchiectasis is a common respiratory disorder of diverse etiology, characterized by permanently dilated airways due to chronic bronchial inflammation, to date, regarded as an orphan disease. The aim of this study was to review the etiology of non-cystic fibrosis bronchiectasis and correlate it with disease severity. To this end, we performed a retrospective study of patients diagnosed with bronchiectasis using high resolution computed tomography scans, between january 2014 and december 2016 at Clinical Hospital of Constanta, Romania. The patient group consisted of 70 adults (19 women and 51 men) and the etiology of bronchiectasis was determined in 51 cases. This included: postinfective (20 patients), chronic obstructive pulmonary disese related (18 patients), asthma related (6 patients), connective tissue disease related (4 patients). Also, we identifyed less common conditions causing bronchietasis like: Primary immunodeficiency, Allergic bronchopulmonary aspergilosis and Swayer James syndrome. Furthermore, the severity of bronchiectasis was determined using Bronchiectasis Severity Index. In terms of finding an etiology, disease severity was not the most reliable marker for identifying different etiological phenotypes. Unederlying cause and prognosis of non-cystic fibrosis are both heterogenous, thus, establishing a specific etiological diagnosis and assesment of disease severity is fundamental to therapeutical management and to prevent disease progresion. Overall, non-cystic fibrosis bronchiectasis is an underestimated disease, not only in prevalence and incidence, but also in its ability to cause great morbidity and mortality.