Bronquiectasias. Fibrosis quística

Abstract

Bronchiectasis is an irreversible dilation of the bronchial lumen. The most common etiology of bronchiectasis not due to cystic fibrosis is post-infection bronchiectasis followed by those secondary to chronic respiratory diseases. The pathogenesis is based on Cole's «vicious circle hypothesis», characterized by abnormality in mucociliary clearance-infection-inflammation. It should be suspected in patients with daily bronchorrhea associated or not with cough and with repeated respiratory infections. It can be associated with systemic diseases. Therefore, a detailed medical history must be taken in addition to the pertinent additional tests. A diagnosis is made by means of a computed tomography scan of the chest, in which the criteria described by Naidich et al. are of help. During follow-up, it is essential to perform serial sputum cultures to be able to start targeted treatment early, especially treatment for Pseudomonas aeruginosa. Respiratory therapy is the pillar of treatment for improving bronchial drainage. In addition, nutritional aspects and factors related to complications must be addressed. Cystic fibrosis will be diagnosed with the determination of two mutations on the genetic study and a positive sweat test. In addition to treating the bronchiectasis, drugs targeted at treating the protein defect (CFTR modulators) have recently come onto the market.