The challenges of developing effective anti-inflammatory agents in cystic fibrosis

Journal of Cystic Fibrosis

Volumn 14, Issue 2, 2015, Pages 164-166

  Sagel, Scott D ^a  

^a University of Colorado Denver and Children s Hospital Colorado   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ACETYLCYSTEINE; ANTIINFLAMMATORY AGENT; GLUTATHIONE; LEUKOTRIENE B4 RECEPTOR ANTAGONIST; SILDENAFIL; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR;

ANTIINFLAMMATORY ACTIVITY; CYSTIC FIBROSIS; EDITORIAL; LUNG DISEASE; LUNG FUNCTION; BLOOD; CLINICAL TRIAL (TOPIC); COMPLICATION; GENETICS; HUMAN; INFLAMMATION; METABOLISM; NEUTROPHIL; PATHOPHYSIOLOGY; PROCEDURES; TARGETED GENE REPAIR;

ANTI-INFLAMMATORY AGENTS; CLINICAL TRIALS AS TOPIC; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; HUMANS; INFLAMMATION; NEUTROPHILS; TARGETED GENE REPAIR;

EID: 84924341379     PISSN: 15691993     EISSN: 18735010     Source Type: Journal    
DOI: 10.1016/j.jcf.2014.12.004     Document Type: Editorial

References (22)

1. Eigen H., Rosenstein B.J., FitzSimmons S., Schidlow D.V. A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group. J Pediatr 1995, 126:515-523.
2. Konstan M.W., Byard P.J., Hoppel C.L., Davis P.B. Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med 1995, 332:848-854.
3. Lands L.C., Milner R., Cantin A.M., Manson D., Corey M. High-dose ibuprofen in cystic fibrosis: Canadian safety and effectiveness trial. J Pediatr 2007, 151:249-254.
4. Oermann C.M., Sockrider M.M., Konstan M.W. The use of anti-inflammatory medications in cystic fibrosis: trends and physician attitudes. Chest 1999, 115:1053-1058.
5. Lai H.C., FitzSimmons S.C., Allen D.B., Kosorok M.R., Rosenstein B.J., Campbell P.W., et al. Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis. N Engl J Med 2000, 342:851-859.
6. Konstan M.W., VanDevanter D.R., Rasouliyan L., Pasta D.J., Yegin A., Morgan W.J., et al. Trends in the use of routine therapies in cystic fibrosis: 1995-2005. Pediatr Pulmonol 2010, 45:1167-1172.
7. Rowe S.M., Heltshe S.L., Gonska T., Donaldson S.H., Borowitz D., Gelfond D., et al. Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis. Am J Respir Crit Care Med 2014, 190:175-184.
8. Calabrese C., Tosco A., Abete P., Carnovale V., Basile C., Magliocca A., et al. Randomized, single blind, controlled trial of inhaled glutathione vs placebo in patients with cystic fibrosis. J Cyst Fibros 2015, 14(2).
9. Skov M., Pressler T., Lykkesfeldt J., Poulsen H.E., Jensen P.O., Johansen H.K., et al. The effect of short-term, high-dose oral N-acetylcysteine treatment on oxidative stress markers in cystic fibrosis patients with chronic P. aeruginosa infection - a pilot study. J Cyst Fibros 2015, 14(2).
10. Conrad C., Lymp J., Thompson V., Dunn C., Davies Z., Chatfield B., et al. Long-term treatment with oral N-acetylcysteine: affects lung function but not sputum inflammation in cystic fibrosis subjects. A phase II randomized placebo-controlled trial. J Cyst Fibros 2015, 14(2).
11. Taylor-Cousar J.L., Wiley C., Felton L.A., St C.C., Jones M., Curran-Everett D., et al. Pharmacokinetics and tolerability of oral sildenafil in adults with cystic fibrosis lung disease. J Cyst Fibros 2015, 14(2).
12. Roum J.H., Buhl R., McElvaney N.G., Borok Z., Crystal R.G. Systemic deficiency of glutathione in cystic fibrosis. J Appl Physiol 1993, 75:2419-2424.
13. Griese M., Kappler M., Eismann C., Ballmann M., Junge S., Rietschel E., et al. Inhalation treatment with glutathione in patients with cystic fibrosis. A randomized clinical trial. Am J Respir Crit Care Med 2013, 188:83-89.
14. Ramsey B.W., Davies J., McElvaney N.G., Tullis E., Bell S.C., Drevinek P., et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 2011, 365:1663-1672.
15. Aitken M.L., Bellon G., De B.K., Flume P.A., Fox H.G., Geller D.E., et al. Long-term inhaled dry powder mannitol in cystic fibrosis: an international randomized study. Am J Respir Crit Care Med 2012, 185:645-652.
16. Poschet J.F., Timmins G.S., Taylor-Cousar J.L., Ornatowski W., Fazio J., Perkett E., et al. Pharmacological modulation of cGMP levels by phosphodiesterase 5 inhibitors as a therapeutic strategy for treatment of respiratory pathology in cystic fibrosis. Am J Physiol Lung Cell Mol Physiol 2007, 293:L712-L719.
17. Lubamba B., Huaux F., Lebacq J., Marbaix E., Dhooghe B., Panin N., et al. Immunomodulatory activity of vardenafil on induced lung inflammation in cystic fibrosis mice. J Cyst Fibros 2012, 11:266-273.
18. Heeckeren A., Walenga R., Konstan M.W., Bonfield T., Davis P.B., Ferkol T. Excessive inflammatory response of cystic fibrosis mice to bronchopulmonary infection with Pseudomonas aeruginosa. J Clin Invest 1997, 100:2810-2815.
19. Stoltz D.A., Meyerholz D.K., Pezzulo A.A., Ramachandran S., Rogan M.P., Davis G.J., et al. Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth. Sci Transl Med 2010, 2:29ra31.
20. Sun X., Sui H., Fisher J.T., Yan Z., Liu X., Cho H.J., et al. Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. J Clin Invest 2010, 120:3149-3160.
21. Konstan M.W., Doring G., Heltshe S.L., Lands L.C., Hilliard K.A., Koker P., et al. A randomized double blind, placebo controlled phase 2 trial of BIIL 284 BS (an LTB4 receptor antagonist) for the treatment of lung disease in children and adults with cystic fibrosis. J Cyst Fibros 2014, 13:148-155.
22. Doring G., Bragonzi A., Paroni M., Akturk F.F., Cigana C., Schmidt A., et al. BIIL 284 reduces neutrophil numbers but increases P. aeruginosa bacteremia and inflammation in mouse lungs. J Cyst Fibros 2014, 13:156-163.