NAGEHAN EMİRALİOĞLU, Zeynelabidin ÖZTÜRK, Ebru YALÇIN, DENİZ DOĞRU ERSÖZ, HAYRİYE UĞUR ÖZÇELİK, EMİNE NURAL KİPER

Long term azithromycin therapy in patients with cystic fibrosis

Emiralioğlu N, Öztürk Z, Yalçın E, Doğru D, Özçelik U, Kiper N. Long term azithromycin therapy in patients with cystic fibrosis. Turk J Pediatr 2016; 58: 34-40.Inflammation is a central contributor to the pathogenesis of cystic fibrosis (CF) pulmonary disease; so limiting the excessive production of inflammatory mediators represents a major therapeutic strategy for slowing the decline in lung function and improving survival. The macrolide antibiotic azithromycin (AZM) has anti-inflammatory properties and immunomodulatory effects that may be beneficial in CF. The aim of this study was to document the long term use of AZM effect on pulmonary function, nutritional status and number of pulmonary exacerbations in patients with CF. Twenty four patients with CF aged 4-23 years followed at Hacettepe University Department of Pediatric Pulmonology between May 2007- December 2014 enrolled in the study from 630 CF patients. They received 10 mg/kg/day of AZM three times a week. Pulmonary function parameters, sputum cultures, body mass index (BMI) Z scores and number of pulmonary exacerbations were analyzed at different time intervals (at the visits at months 6, 9 and 12).Median age of the patients was 14.7 (range 4-23 years) years and median treatment duration was 14 months (range 6-60 months). Initially, median FEV1% was found 68% (range 30%-100%), BMI was found 17.05 (range 13.326.5) and oxygen saturation was found 95% (range 84%-99%). At the end of the 6th, 9th and 12th months of the AZM therapy; no significant differences in FEV1% and oxygen saturation parameters were found compared to the initial time, however BMI increased significantly (p=0.03), also the number of pulmonary exacerbations (p

PDF

___

1. Davies JC, Alton EW, Bush A. Cystic fibrosis. BMJ 2007; 335: 1255-1259.

2. Principi N, Blasi F, Esposito S. Azithromycin use in patients with cystic fibrosis. Eur J Clin Microbiol Infect Dis 2015; 34: 1071-1079.

3. Watt AP, Courtney J, Moore J, Ennis M, Elborn JS. Neutrophil cell death, activation and bacterial infection in cystic fibrosis. Thorax 2005; 60: 659-664.

4. Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2013; 187: 680-689.

5. Lutz L, Pereira DC, Paiva RM, et al. Macrolides decrease the minimal inhibitory concentration of antipseudomonal agents against Pseudomonas aeruginosa from cystic fibrosis patients in biofilm. BMC Microbiol 2012; 12: 196.

6. Southern KW, Barker PM, Solis-Moya A, et al. Macrolide antibiotics for cystic fibrosis. Cochrane Database Syst Rev 2012; 11: CD002203.

7. Lee TW, Brownlee KG, Conway SP, et al. Evaluation of a new definition for chronic Pseudomonas aeruginosa infection in cystic fibrosis patients. J Cyst Fibros 2003; 2: 29-34.

8. Goss CH, Burns JL. Exacerbations in cystic fibrosis. 1: epidemiology and pathogenesis. Thorax 2007; 62: 360-367.

9. Standardization of Spirometry, 1994 Update. American Thoracic Society. Am J Respir Crit Care Med 1995; 152: 1107-1136.

10. Khan TZ, Wagener JS, Bost T, et al. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med 1995; 151: 1075-1082.

11. Spagnolo P, Fabbri LM, Bush A. Long-term macrolide treatment for chronic respiratory disease. Eur Respir J 2013; 42: 239-251.

12. Wolter J, Seeney S, Bell S, et al. Effect of long term treatment with azithromycin on disease parameters in cystic fibrosis: a randomized trial. Thorax 2002; 57: 212-216.

13. Equi A, Balfour-Lynn IM, Bush A, et al. Long term azithromycin in children with cystic fibrosis: a randomized, placebo-controlled crossover trial. Lancet 2002; 360: 978-984.

14. Saiman L, Marshall BC, Mayer-Hamblett N, et al. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA 2003; 290: 1749-1756.

15. Clement A, Tamalet A, Leroux E, et al. Long term effects of azithromycin in patients with cystic fibrosis: a double blind, placebo controlled trial. Thorax 2006; 61: 895-902.

16. McCormack J, Bell S, Senini S, et al. Daily versus weekly azithromycin in cystic fibrosis patients. Eur Respir J 2007; 30: 487-495.

17. Saiman L, Anstead M, Mayer-Hamblett N, et al. Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA 2010; 303: 1707-1715.

18. Saiman L, Mayer-Hamblett N, Anstead M, et al. Openlabel, follow-on study of azithromycin in paediatric patients with CF uninfected with Pseudomonas aeruginosa. Pediatr Pulmonol 2012; 47: 641-648.

19. Ratjen F, Saiman L, Mayer-Hamblett N, et al. Effect of azithromycin on systemic markers of inflammation in cystic fibrosis patients uninfected with Pseudomonas aeruginosa. Chest 2012; 142: 1259-1266.

20. Tramper-Stranders GA, Wolfs TF, et al. Maintenance azithromycin treatment in pediatric patients with cystic fibrosis: long-term outcomes related to macrolide resistance and pulmonary function. Pediatr Infect Dis J 2007; 26: 8-12.

21. Willekens J, Eyns H, Malfroot A. How long should we maintain long-term azithromycin treatment in cystic fibrosis patients? Pediatr Pulmonol 2015; 50: 103-104.

22. Cai Y, Chai D, Wang R, et al. Effectiveness and safety of macrolides in cystic fibrosis patients: a meta-analysis and systematic review. J Antimicrob Chemother 2011; 66: 968-978.

23. Hansen CR, Pressler T, Koch C, et al. Long-term azitromycin treatment of cystic fibrosis patients with chronic Pseudomonas aeruginosa infection; an observational cohort study. J Cyst Fibros 2005; 4: 35-40.

24. Roux AL, Catherinot E, Ripoll F, et al. Multicenter study of prevalence of nontuberculous mycobacteria in patients with cystic fibrosis in france. J Clin Microbiol 2009; 47: 4124-4128.