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63. Hill AT, Bayley D, Stockley RA. The interrelationship of sputum inflammatory markers in patients with chronic bronchitis. Am J
Respir Crit Care Med 1999;160:893-8. DOI PubMed
64. McElvaney NG, Nakamura H, Birrer P, et al. Modulation of airway inflammation in cystic fibrosis. In vivo suppression of interleukin-
8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor. J Clin Invest
1992;90:1296-301. DOI PubMed PMC
65. Rao NV, Marshall BC, Gray BH, Hoidal JR. Interaction of secretory leukocyte protease inhibitor with proteinase-3. Am J Respir Cell
Mol Biol 1993;8:612-6. DOI PubMed
66. Shapiro SD. Proteolysis in the lung. Eur Respir J Suppl 2003;44:30s-2s. DOI PubMed
67. Oliveira S, Rosowski EE, Huttenlocher A. Neutrophil migration in infection and wound repair: going forward in reverse. Nat Rev
Immunol 2016;16:378-91. DOI PubMed PMC
68. Isogai Z, Ono RN, Ushiro S, et al. Latent transforming growth factor beta-binding protein 1 interacts with fibrillin and is a microfibril-
associated protein. J Biol Chem 2003;278:2750-7. DOI PubMed
69. Whitters D, Stockley R. Immunity and bacterial colonisation in bronchiectasis. Thorax 2012;67:1006-13. DOI PubMed
70. Stevens T, Ekholm K, Gränse M, et al. AZD9668: pharmacological characterization of a novel oral inhibitor of neutrophil elastase. J
Pharmacol Exp Ther 2011;339:313-20. DOI PubMed
71. Mereo BioPharma Press Release. Mereo biopharma announces positive top-line efficacy and safety data from “ASTRAEUS” phase 2
trial of alvelestat in alpha-1 antitrypsin deficiency- associated emphysema. Available from: https://www.mereobiopharma.com/news-
and-events/news/2022/may/mereo-biopharma-announces-positive-top-line-efficacy-and-safety-data-from-astraeus-phase-2-trial-of-
alvelestat-in-alpha-1-antitrypsin-deficiency-associated-emphysema/ [Last accessed on 9 Dec 2022].
72. Woolhouse IS, Bayley DL, Lalor P, Adams DH, Stockley RA. Endothelial interactions of neutrophils under flow in chronic obstructive
pulmonary disease. Eur Respir J 2005;25:612-7. DOI PubMed
73. White JR, Judithann ML, Peter RY, et al. Identification of a potent, selective non-peptide CXCR2 antagonist that inhibits interleukin-
8-induced neutrophil migration. J Biol Chem ;1998.273:10095-8. DOI PubMed
74. Lazaar AL, Miller BE, Donald AC, et al; for 205724 Investigators. CXCR2 antagonist for patients with chronic obstructive pulmonary
disease with chronic mucus hypersecretion: a phase 2b trial. Respir Res 2020;21:149. DOI PubMed PMC
75. Tonnesen MG, Anderson DC, Springer TA, Knedler A, Avdi N, Henson PM. Adherence of neutrophils to cultured human
microvascular endothelial cells. Stimulation by chemotactic peptides and lipid mediators and dependence upon the Mac-1, LFA-1,
p150,95 glycoprotein family. J Clin Invest 1989;83:637-46. DOI PubMed PMC
76. Hoover RL, Karnovsky MJ, Austen KF, Corey EJ, Lewis RA. Leukotriene B4 action on endothelium mediates augmented
neutrophil/endothelial adhesion. Proc Natl Acad Sci USA 1984;81:2191-3. DOI PubMed PMC
77. Bhatt L, Roinestad K, Van T, Springman EB. Recent advances in clinical development of leukotriene B4 pathway drugs. Semin
Immunol 2017;33:65-73. DOI PubMed
78. Gompertz S, Stockley RA. A randomized, placebo-controlled trial of a leukotriene synthesis inhibitor in patients with COPD. Chest
2002;122:289-94. DOI PubMed
79. Adkison AM, Raptis SZ, Kelley DG, Pham CT. Dipeptidyl peptidase I activates neutrophil-derived serine proteases and regulates the
development of acute experimental arthritis. J Clin Invest 2002;109:363-71. DOI PubMed PMC
80. Murphy MP, McEnery T, McQuillan K, et al. α(1) antitrypsin therapy modulates the neutrophil membrane proteome and secretome.
Eur Respir J 2020;55:1901678. DOI PubMed
81. Sørensen OE, Clemmensen SN, Dahl SL, et al. Papillon-lefèvre syndrome patient reveals species-dependent requirements for
neutrophil defenses. J Clin Invest 2014;124:4539-48. DOI PubMed PMC
82. Chalmers JD, Haworth CS, Metersky ML, et al; WILLOW Investigators. Phase 2 trial of the DPP-1 inhibitor brensocatib in
bronchiectasis. N Engl J Med 2020;383:2127-37. DOI PubMed