Pulmonary complications in cystic fibrosis Complicaciones pulmonares en fibrosis quística

Abstract

Cystic fibrosis is an autosomal recessive disease caused by mutations in chromosome 7, and compromises multiple organs. One of the most common complications is the respiratory tract infection due to Pseudomonas aeruginosa.
Objectives: To present the recommendations on the management of the pulmonary complications reported in clinical practice guidelines, of good quality according to AGREE II, with the aim of reducing morbidity and improving the quality of life of the patients.
Methods: A review was performed on cystic fibrosis clinical practice guidelines, in English and Spanish, published between 2005 and 2013, using the AGREE II tool to evaluate their quality.
Results: The search produced a total of 126 guidelines, of which 6 were evaluated, and 4 obtained a score ≥ 60% in the rigour and independence domain according to the AGREE II. The recommendations for the management of pulmonary exacerbations due to Pseudomonas aeruginosa were taken from these, as well as those for those of the airways, pneumothorax, haemoptysis, and allergic bronchopulmonary aspergillosis.
Conclusions: The best inhaled antibiotic for chronic use is tobramycin. Dornase alpha is the best mucolytic. The use of corticosteroids is controversial, but is useful in patients who suffer from asthma and/or allergic bronchopulmonary aspergillosis. The best quality clinical practice guidelines are currently from Ecuador.