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Cystic Fibrosis
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my personal edition > cystic fibrosis > news
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DGReview
Macrolides Benefit Patients With Cystic Fibrosis Although More Studies Are Needed
A DGReview of :"Macrolide antibiotic therapy in patients with cystic fibrosis"
Swiss Medical Weekly
07/30/2003
By Emma Hitt, PhD
The macrolide antibiotic azithromycin has shown promise in the treatment of cystic fibrosis (CF), although further studies are needed to confirm the benefit of macrolides in these patients.
In his report, Martin H.Schöni, MD, with the Department of Paediatrics at the University of Berne, Switzerland, summarised some of the latest trials addressing the use of macrolides in treating patients with CF.
According to Dr. Schöni, more than 1000 genetic mutations in the cystic fibrosis transmembrane regulator (CFTR) protein have been identified, although an explanation for the pulmonary phenotype has not yet emerged. However, the "level of inflammation directly correlates with progression and outcome of the illness," he notes, and CFTR dysfunction may predispose to bacterial adherence resulting in an augmented inflammatory response.
The anti-inflammatory benefits of macrolides in CF patients may involve several mechanisms, including modulation of inflammatory pathways; antibacterial effects through inhibition of alginate and biofilm formation and inhibition of adherence of Pseudomonas aeruginosa to the bronchial wall; and upregulation of chloride transport by the CFTR.
In his article, Dr. Schöni outlines data from clinical trials in macrolide therapy for diffuse panbronchiolitis (DPB) and CF. DBP is a chronic inflammatory pulmonary disease, seen mainly in Japan, that may be associated with some rare mutations of the CFTR gene.
In DBP patients with P. aeruginosa infection, the benefits of low-dose treatment with 400 mg to 600 mg erythromycin daily for at least 6 months was "impressive, resulting in an increase of 10-year survival from 12.4% to more than 90%."
In CF patients, from 1997 to 2003, 10 studies were conducted including 339 patients treated with macrolides (8 studies with azithromycin, 1 with erythromycin, 1 with clarithromycin).
In these studies, FEV1 and/or FVC increased from between 4.8% to 11% of predicted values from baseline; however, in general, the presence and density of P. aeruginosa did not change, suggesting that an anti-inflammatory effect was probably responsible for the clinical improvements in patients with CF and chronic P. aeruginosa infection.
So far, researchers of various trials have made only non-specific recommendations about the use of azithromycin in patients with CF. In general, though, they suggest that azithromycin is an option in patients older than 6 years of age with chronic P. aeruginosa infection and may be used as an adjunct to the other proven CF therapies, such as tobramycin and ibuprofen.
"The data presented in peer reviewed journals are, however, too sparse to already justify firm recommendations for the general use of azithromycin, erythromycin or clarithromycin on a long-term basis for the treatment of chronic lung disease in CF," Dr. Schöni concludes.
Swiss Med Week 2003;133: 297-301.
"Macrolide antibiotic therapy in patients with cystic fibrosis"
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