Highlights of a workshop to discuss targeting inflammation in cystic fibrosis

Journal of Cystic Fibrosis

Volumn 8, Issue 1, 2009, Pages 1-8

  Banner, Katharine H ^a   De Jonge, Hugo ^b   Elborn, Stuart ^c   Growcott, Ellena ^a   Gulbins, Erich ^d   Konstan, Mike ^e   Moss, Rick ^f   Poll, Chris ^a   Randell, Scott H ^g   Rossi, Adriano G ^h   Thomas, Lorraine ^a   Waltz, David ^a  

^a NOVARTIS INSTITUTES FOR BIOMEDICAL RESEARCH   (United States)

^b ERASMUS MEDICAL CENTER   (Netherlands)

^c BELFAST CITY HOSPITAL   (United Kingdom)

^d UNIVERSITY OF DUISBURG ESSEN   (Germany)

^e CASE WESTERN RESERVE UNIVERSITY   (United States)

^f STANFORD UNIVERSITY SCHOOL OF MEDICINE   (United States)

^g UNIVERSITY OF NORTH CAROLINA SCHOOL OF MEDICINE   (United States)

^h UNIVERSITY OF EDINBURGH   (United Kingdom)

Author keywords

Cystic fibrosis; Epithelial cell; Inflamation; Lymphocyte; Neutrophil

Indexed keywords

AMITRIPTYLINE; ANTIINFLAMMATORY AGENT; ANTIOXIDANT; AZITHROMYCIN; BIIL 284 BS; CORTICOSTEROID; CYCLIN DEPENDENT KINASE INHIBITOR; DEOXYRIBONUCLEASE; DORNASE ALFA; ELASTASE INHIBITOR; GAMMA1B INTERFERON; IBUPROFEN; LEUKOTRIENE RECEPTOR BLOCKING AGENT; MACROLIDE; NONSTEROID ANTIINFLAMMATORY AGENT; PLACEBO; PROTEINASE INHIBITOR; ROSCOVITINE; UNCLASSIFIED DRUG;

APOPTOSIS; CELL FUNCTION; CELL TYPE; CLINICAL PROTOCOL; CLINICAL TRIAL; CYSTIC FIBROSIS; DRUG EFFICACY; DRUG MECHANISM; DRUG TARGETING; EPITHELIUM CELL; HUMAN; INFLAMMATION; LUNG INJURY; LYMPHOCYTE; NEUTROPHIL; NONHUMAN; REVIEW; UNSPECIFIED SIDE EFFECT; WORKSHOP;

ANIMALS; ANTI-INFLAMMATORY AGENTS; CYSTIC FIBROSIS; EDUCATION; EPITHELIAL CELLS; HUMANS; INFLAMMATION; LYMPHOCYTES; MODELS, BIOLOGICAL; NEUTROPHILS; RESEARCH DESIGN;

EID: 57649158936     PISSN: 15691993     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.jcf.2008.10.002     Document Type: Review

References (78)

1. Riordan J.R., Rommens J.M., Kerem B., Alon N., Rozmahel R., Grzelczak Z., et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245 4922 (1989) 1066-1073
2. M.D. Bethesda, Patient registry 2006 annual data report 2007; Cytsic Fibrosis Foundation.
3. Gibson R.L., Burns J.L., and Ramsey B.W. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med 168 (2003) 918-951
4. Konstan M.W., and Berger M. Current understanding of the inflammatory process in cystic fibrosis: onset and etiology. Pediatr Pulmonol 24 2 (1997) 137-142
5. Muhlebach M.S., Stewart P.W., Leigh M.W., and Noah T.L. Quantitation of inflammatory responses to bacteria in young cystic fibrosis and control patients. Am J Respir Crit Care Med 160 1 (1999) 186-191
6. Govan J.R., and Nelson J.W. Microbiology of lung infection in cystic fibrosis. Br Med Bull 48 4 (1992) 912-930
7. Balfour-Lynn I.M. The protease-antiprotease battle in the cystic fibrosis lung. J R Soc Med 92 Suppl 37 (1999) 23-30
8. Hampton M.B., Kettle A.J., and Winterbourn C.C. Inside the neutrophil phagosome: Oxidants myeloperoxidase and bacterial killing. Blood 92 9 (1998) 3007-3301
9. http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=retrieve&dopt=full report&list_uids=1991&log$=databasead&logdbfrom=protein.
10. Borregaard N., and Coweland J.B. Granules of the human neutrophilic polymorphonuclear leukocyte. Blood 89 (1997) 3503-3521
11. Brinkmann V., Reichard U., Goosmann C., Fauler B., Uhlemann Y., Weiss D.S., et al. Neutrophil extracellular traps kill bacteria. Science 303 5663 (2004) 1532-1535
12. Brinkmann V., and Zychlinsky A. Beneficial suicide: why neutrophils die to make NETs. Nat Rev Microbiol 5 8 (2007) 577-582
13. Walmsley S.R., Print C., Farahi N., Peyssonnaux C., Johnson R.S., Cramer T., et al. Hypoxia-induced neutrophil survival is mediated by HIF-1α-dependent NFκB. J Exp Med 200 1 (2005) 105-115
14. Konstan M.W., and Davis P.B. Pharmacological approaches for the discovery and development of new anti-inflammatory agents for the treatment of cystic fibrosis. Adv Drug Deliv Rev 54 11 (2002) 1409-1423
15. Chmiel J.F., and Konstan M.W. Inflammation and anti-inflammatory therapies for cystic fibrosis. Clin Chest Med 28 2 (2007) 331-346
16. Dovey M., Aitken M.L., Emerson J., McNamara S., Waltz D.A., and Gibson R.L. Oral corticosteroid therapy in cystic fibrosis patients hospitalized for pulmonary exacerbation: a pilot study. Chest 132 4 (2007) 1212-1218
17. Eigen H., Rosenstein B.J., FitzSimmons S., and Schidlow D.V. A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group. J Pediatr 126 4 (1995) 515-523
18. Konstan M.W., Byard P.J., Hoppel C.L., and Davis P.B. Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med 332 13 (1995) 848-854
19. Balfour-Lynn I.M., Klein N.J., and Dinwiddie R. Randomised controlled trial of inhaled corticosteroids (fluticasone propionate) in cystic fibrosis. Arch Dis Child 77 2 (1997) 124-130
20. McColl A., Michlewska S., Dransfield I., and Rossi A.G. Effects of glucocorticoids on apoptosis and clearance of apoptotic cells. ScientificWorldJournal 7 (2007) 1165-1181
21. Konstan M.W., Schluchter M.D., Xue W., and Davis P.B. Clinical use of Ibuprofen is associated with slower FEV1 decline in children with cystic fibrosis. Am J Respir Crit Care Med 176 11 (2007) 1084-1089
22. Konstan M.W., Doring G., Lands L.C., Hilliard K.A., Koker P., Bhattacharya S., et al. Results of a phase II clinical trial of BIIL 284 BS (an LTB4 receptor antagonist) for the treatment of CF lung disease. Pediatr Pulmonol Supplement 28 (2005) 125-126
23. Sagel S.D., Chmiel J.F., and Konstan M.W. Sputum biomarkers of inflammation in cystic fibrosis lung disease. Proc Am Thorac Soc 4 4 (2007) 406-417
24. Mayer-Hamblett N., Aitken M.L., Accurso F.J., Kronmal R.A., Konstan M.W., Burns J.L., et al. Association between pulmonary function and sputum biomarkers in cystic fibrosis. Am J Respir Crit Care Med 175 8 (2007) 822-828
25. McElvaney N.G., Hubbard R.C., Birrer P., Chernick M.S., Caplan D.B., Frank M.M., et al. Aerosol alpha 1-antitrypsin treatment for cystic fibrosis. Lancet 337 8738 (1991) 392-394
26. Cantin A.M., White T.B., Cross C.E., Forman H.J., Sokol R.J., and Borowitz D. Antioxidants in cystic fibrosis. Conclusions from the CF antioxidant workshop, Bethesda, Maryland, November 11-12, 2003. Free Radic Biol Med 42 1 (2007) 15-31
27. Suri R. The use of human deoxyribonuclease (rhDNase) in the management of cystic fibrosis. BioDrugs 19 3 (2005) 135-144
28. Shah P.L., and Hodson M.E. Dornase alfa. BioDrugs 8 6 (1997) 439-445
29. Rossi A.G., Sawatzky D.A., Walker A., Ward C., Sheldrake T.A., Riley N.A., et al. Cyclin-dependent kinase inhibitors enhance the resolution of inflammation by promoting inflammatory cell apoptosis. Nat Med 12 9 (2006) 1056-1064
30. Donaldson S.H., and Boucher R.C. Sodium channels and cystic fibrosis. Chest 132 5 (2007) 1631-1636
31. Mall M., Grubb B.R., Harkema J.R., O'Neal W.K., and Boucher R.C. Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice. Nat Med 10 5 (2004) 487-493
32. Zähringer U., Lindner B., Inamura S., Heine H., and Alexander C. TLR2 - promiscuous or specific? A critical re-evaluation of a receptor expressing apparent broad specificity. Immunobiology 213 3-4 (2008) 205-224
33. Hartl D., Latzin P., Hordijk P., Marcos V., Rudolph C., Woischnik M., et al. Cleavage of CXCR1 on neutrophils disables bacterial killing in cystic fibrosis lung disease. Nat Med 13 12 (2007) 1423-1430
34. Gewirtz T.A., Navas T.A., Lyons S., Godowski P.J., and Madara J.L. Cutting Edge: bacterial flagellin activates basolaterally expressed TLR5 to induce epithelial pro-inflammatory gene expression. J Immunol 167 (2001) 1882-1887
35. Tsan M.F., and Gao B. Endogenous ligands of Toll-like receptors. J Leukoc Biol 76 (2004) 514-519
36. Gay N.J., and Gangloff M. Structure and function of Toll receptors and their ligands. Annu Rev Biochem 76 (2007) 141-165
37. Wu Q., Lu Z., Verghese M.W., and Randell S.H. Airway epithelial cell tolerance to Pseudomonas aeruginosa. Respir Res 6 (2005) 26
38. Pantano C., Ather J.L., Alcorn J.F., Poynter M.E., Brown A.L., and Guala A.S. Nuclear factor-kappaB activation in airway epithelium induces inflammation and hyperresponsiveness. Am J Respir Crit Care Med 177 9 (2008) 959-969
39. Cheng D.S., Han W., Chen S.M., Sherrill T.P., Chont M., and Park G.Y. Airway epithelium controls lung inflammation and injury through the NF-kappa B pathway. J Immunol 178 10 (2007) 6504-6513
40. Sadikot R.T., Zeng H., Joo M., Everhart M.B., Sherrill T.P., Li J., et al. Targeted immunomodulation of the NF-kappaB pathway in airway epithelium impacts host defense against Pseudomonas aeruginosa. J Immunol 176 8 (2006) 4923-4930
41. Azzawi M., Johnston P.W., Majumdar S., Kay A.B., and Jeffery P.K. T lymphocytes and activated eosinophils in airway mucosa in fatal asthma and cystic fibrosis. Am Rev Respir Dis 145 6 (1992) 1477-1482
42. Hubeau C., Lorenzato M., Couetil J.P., Hubert D., Dusser D., Puchelle E., et al. Quantitative analysis of inflammatory cells infiltrating the cystic fibrosis airway mucosa. Clin Exp Immunol 124 1 (2001) 69-76
43. Bubien J.K. CFTR may play a role in regulated secretion by lymphocytes: a new hypothesis for the pathophysiology of cystic fibrosis. Pflugers Arch 443 Suppl 1 (2001) S36-S39
44. Chen J.H., Schulman H., and Gardner P. A cAMP-regulated chloride channel in lymphocytes that is affected in cystic fibrosis. Science 243 4891 (1989) 657-660
45. McDonald T.V., Nghiem P.T., Gardner P., and Martens C.L. Human lymphocytes transcribe the cystic fibrosis transmembrane conductance regulator gene and exhibit CF-defective cAMP-regulated chloride current. J Biol Chem 267 5 (1992) 3242-3248
46. Dong Y.J., Chao A.C., Kouyama K., Hsu Y.P., Bocian R.C., Moss R.B., et al. Activation of CFTR chloride current by nitric oxide in human T lymphocytes. EMBO J 14 12 (1995) 2700-2707
47. Moss R.B., Bocian R.C., Hsu Y.P., Dong Y.J., Kemna M., Wei T., et al. Reduced IL-10 secretion by CD4+ T lymphocytes expressing mutant cystic fibrosis transmembrane conductance regulator (CFTR). Clin Exp Immunol 106 2 (1996) 374-388
48. Moss R.B., Hsu Y.P., and Olds L. Cytokine dysregulation in activated cystic fibrosis (CF) peripheral lymphocytes. Clin Exp Immunol 120 3 (2000) 518-525
49. Moser C., Kjaergaard S., Pressler T., Kharazmi A., Koch C., and Hoiby N. The immune response to chronic Pseudomonas aeruginosa lung infection in cystic fibrosis patients is predominantly of the Th2 type. APMIS 108 5 (2000) 329-335
50. Hartl D., Griese M., Kappler M., Zissel G., Reinhardt D., Rebhan C., et al. Pulmonary T(H)2 response in Pseudomonas aeruginosa-infected patients with cystic fibrosis. J Allergy Clin Immunol 117 1 (2006) 204-211
51. Moss R.B., Mayer-Hamblett N., Wagener J., Daines C., Hale K., Ahrens R., et al. Randomized, double-blind, placebo-controlled, dose-escalating study of aerosolized interferon gamma-1b in patients with mild to moderate cystic fibrosis lung disease. Pediatr Pulmonol 39 3 (2005) 209-218
52. Ye P., Rodriguez F.H., Kanaly S., Stocking K.L., Schurr J., Schwarzenberger P., et al. Requirement of interleukin 17 receptor signaling for lung CXC chemokine and granulocyte colony-stimulating factor expression, neutrophil recruitment, and host defense. J Exp Med 194 4 (2001) 519-527
53. Dubin P.J., McAllister F., and Kolls J.K. Is cystic fibrosis a TH17 disease?. Inflamm Res 56 6 (2007) 221-227
54. McAllister F., Henry A., Kreindler J.L., Dubin P.J., Ulrich L., Steele C., et al. Role of IL-17A, IL-17F, and the IL-17 receptor in regulating growth-related oncogene-alpha and granulocyte colony-stimulating factor in bronchial epithelium: implications for airway inflammation in cystic fibrosis. J Immunol 175 1 (2005) 404-412
55. Weber A.J., Soong G., Bryan R., Saba S., and Prince A. Activation of NF-kappaB in airway epithelial cells is dependent on CFTR trafficking and Cl- channel function. Am J Physiol Lung Cell Mol Physiol 281 1 (2001) L71-L78
56. Kammouni W., Figarella C., Marchand S., and Merten M. Altered cytokine production by cystic fibrosis tracheal gland serous cells. Infect Immun 65 12 (1997) 5176-5183
57. Li J., Johnson X.D., Iazvovskaia S., Tan A., Lin A., and Hershenson M.B. Signaling intermediates required for NF-kappa B activation and IL-8 expression in CF bronchial epithelial cells. Am J Physiol Lung Cell Mol Physiol 284 2 (2003) L307-L315
58. Bonfield T.L., Konstan M.W., and Berger M. Altered respiratory epithelial cell cytokine production in cystic fibrosis. J Allergy Clin Immunol 104 1 (1999) 72-78
59. Kube D., Sontich U., Fletcher D., and Davis P.B. Proinflammatory cytokine responses to P. aeruginosa infection in human airway epithelial cell lines. Am J Physiol Lung Cell Mol Physiol 280 3 (2001) L493-L502
60. Aldallal N., McNaughton E.E., Manzel L.J., Richards A.M., Zabner J., Ferkol T.W., et al. Inflammatory response in airway epithelial cells isolated from patients with cystic fibrosis. Am J Respir Crit Care Med 166 9 (2002) 1248-1256
61. O'Brien G.J., Riddell G., Elborn J.S., Ennis M., and Skibinski G. Staphylococcus aureus enterotoxins induce IL-8 secretion by human nasal epithelial cells. Respir Res 7 (2006) 115
62. Tirouvanziam R., de Bentzmann S., Hubeau C., Hinnrasky J., Jacquot J., Peault B., et al. Inflammation and infection in naive human cystic fibrosis airway grafts. Am J Respir Cell Mol Biol 23 2 (2000) 121-127
63. Rogers C.S., Hao Y., Rokhlina T., Samuel M., Stoltz D.A., Li Y., et al. Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer. J Clin Invest 118 4 (2008) 1571-1577
64. Guilbault C., Martin P., Houle D., Boghdady M.L., Guiot M.C., MArion D., et al. Cystic fibrosis lung disease following infection with Pseudomonas aeruginosa in Cftr knockout mice using novel non-invasive direct pulmonary infection technique. Lab Anim 39 3 (2005) 336-352
65. Legssyer R., Huaux F., Lebacq J., Delos M., Marbaix E., Lebecque P., et al. Azithromycin reduces spontaneous and induced inflammation in DeltaF508 cystic fibrosis mice. Respir Res 7 (2006) 134
66. Mall M.A., Harkema J.R., Trojanek J.B., Treis D., Livraghi A., Schubert S., et al. Development of chronic bronchitis and emphysema in beta-epthithelial Na+ channel-overexpressing mice. Am J Respir Crit Care Med 177 7 (2008) 730-742
67. Teichgraber V., Ulrich M., Endlich N., Riethmuller J., Wilker B., De Oliveira-Munding C.C., et al. Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis. Nat Med 14 4 (2008) 382-391
68. Blackwell T.S., Yull F.E., Chen C.L., Venkatakrishnan A., Blackwell T.R., Hicks D.J., et al. Multiorgan nuclear factor kappa B activation in a transgenic mouse model of systemic inflammation. Am J Respir Crit Care Med 162 3 Pt 1 (2000) 1095-1101
69. Everhart M.B., Han W., Sherrill T.P., Arutiunov M., Polosukhin V.V., Burke J.R., et al. Duration and intensity of NF-kappaB activity determine the severity of endotoxin-induced acute lung injury. J Immunol 176 8 (2006) 4995-5005
70. Sollano J., Kirsch J., Bala M., Chambers M., and Harpole L. The economics of drug discovery and the ultimate valuation of pharmacotherapies in the marketplace. Clin Pharmacol Ther 84 2 (2008) 263-266
71. Biomarkers and surrogate endpoints: preferred definitions and conceptual framework. Clin Pharmacol Ther 69 3 (2001) 89-95
72. Auerbach H.S., Williams M., Kirkpatrick J.A., and Colten H.R. Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet 2 8457 (1985) 686-688
73. Konstan M.W., Krenicky J.E., Finney M.R., Kirchner H.L., Hilliard K.A., Hilliard J.B., et al. Effect of ibuprofen on neutrophil migration in vivo in cystic fibrosis and healthy subjects. J Pharmacol Exp Ther 306 3 (2003) 1086-1091
74. Levy H., Kalish L.A., Huntington I., Weller N., Gerard C., Silverman E.K., et al. Inflammatory markers of lung disease in adult patients with cystic fibrosis. Pediatr Pulmonol 42 3 (2007) 256-262
75. Downey D.G., Martin S.L., Dempster M., Moore J.E., Keogan M.T., Starcher B., et al. The relationship of clinical and inflammatory markers to outcome in stable patients with cystic fibrosis. Pediatr Pulmonol 42 3 (2007) 216-220
76. Saiman L., Mayer-Hamblett N., Campbell P., and Marshall B.C. Heterogeneity of treatment response to azithromycin in patients with cystic fibrosis. Am J Respir Crit Care Med 172 8 (2005) 1008-1012
77. Giamarellos-Bourboulis E.J. Macrolides beyond the conventional antimicrobials: a class of potent immunomodulators. Int J Antimicrob Agents 31 1 (2008) 12-20
78. Shinkai M., Henke M.O., and Rubin B.K. Macrolide antibiotics as immunomodulatory medications: proposed mechanisms of action. Pharmacol Ther 117 3 (2008) 393-405