Oral corticosteroids for cystic fibrosis pulmonary exacerbation: seeking the future in the past

Extract

Pulmonary exacerbations remain the most important cause of morbidity, loss of lung function, and reduced quality of life in patients with cystic fibrosis (CF), and are characterised by chronic bacterial airway infection and persistent neutrophilic inflammation [1]. Historically, treatments for CF have predominantly concentrated on combating infections, and anti-inflammatory therapy has been neglected. Chronic neutrophil-dominant airway inflammation is a major contributor to the permanent loss of lung function and progression of disease [2]. Corticosteroids are potent and widely used anti-inflammatory agents in acute exacerbations of other chronic respiratory diseases [3, 4]; however, to date, data on the efficacy of corticosteroids from randomised controlled trials (RCTs) involving CF patients have been limited to clinically stable children and vary in dosing and duration of treatment. Definitive evidence for oral corticosteroid treatment in CF patients with pulmonary exacerbations is still lacking, and whether the therapy was associated with superior clinical outcomes is unclear.