Phenotyping Adults with Non-Cystic Fibrosis Bronchiectasis: A 10-Year Cohort Study in a French Regional University Hospital Center

Abstract

Background: Data concerning phenotypes in bronchiectasis are scarce. Objective: The aim of this study was to describe the clinical, functional and microbiological phenotypes of patients with bronchiectasis. Methods: A monocentric retrospective study in a university hospital in France was conducted over 10 years (2002-2012). Non-cystic fibrosis patients with tomographic confirmation of bronchiectasis were included. The clinical, functional and microbiological data of patients were analyzed relying on the underlying etiology. Results: Of the 311 included patients, an etiology was found for 245 of them. At the time of diagnosis, the median age was 61 years and the mean FEV1 was 63% of predicted. The main causes of bronchiectasis were post-infectious (50%, mostly related to tuberculosis), chronic obstructive pulmonary disease (COPD; 13%) and idiopathic (11%). Other causes were immune deficiency (6%), asthma (4%), autoimmunity (3%), tumor (2%) and other causes (4%). The comparison of phenotypic traits shows significant differences between COPD, congenital and idiopathic groups in term of sex (p = 0.0175), tobacco status (p < 0.0001), FEV1 (p = 0.0412) and age at diagnosis (p < 0.001), Pseudomonas aeruginosa (PA) colonization (p = 0.0276) and lobectomy (0.0093). Functional follow-up was available in 30% of patients with a median duration of 2.7 years. Presence of PA was associated with a lower median FEV1 at diagnosis (43% p < 0.003) but not with a faster rate of decline in FEV1. Conclusion: Distinctive clinical, functional and microbiological features were found for idiopathic, congenital and COPD-related bronchiectasis. A prospective follow-up of these subgroups is necessary to validate their relevance in the management of bacterial colonization and specific complications of these bronchiectases.