Increased von Willebrand Factor Processing in COPD, Reflecting Lung Epithelium Damage, Is Associated with Emphysema, Exacerbations and Elevated Mortality Risk

References

• AgustiA, CalverleyPMA, CelliB, et al. Evaluation of COPD Longitudinally to Identify Predictive Surrogate Endpoints (ECLIPSE) investigators. Characterisation of COPD heterogeneity in the ECLIPSE cohort. Respir Res. 2010;11:122. doi:10.1186/1465-9921-11-12220831787
   PubMed Web of Science ®Google Scholar
• AlkadyO, FattouhM. Role of inflammatory biomarkers in COPD In: 5.2 Monitoring Airway Disease. European Respiratory Society:PA1010. 2016 Available from: https://erj.ersjournals.com/content/48/suppl_60/PA1010 . Accessed March 04, 2020.
   Google Scholar
• AndréP, DenisCV, WareJ, et al. Platelets adhere to and translocate on von Willebrand factor presented by endothelium in stimulated veins. Blood. 2000;96:3322–3328. doi:10.1182/blood.V96.10.332211071623
   PubMed Web of Science ®Google Scholar
• AngelisN, PorpodisK, ZarogoulidisP, et al. Airway inflammation in chronic obstructive pulmonary disease. J Thorac Dis. 2014;6(Suppl 1):S167–72. doi:10.3978/j.issn.2072-1439.2014.03.0724672691
   PubMed Web of Science ®Google Scholar
• BarakOF, MladinovS, HoilandRL, et al. Disturbed blood flow worsens endothelial dysfunction in moderate-severe chronic obstructive pulmonary disease. Sci Rep. 2017;7. doi:10.1038/s41598-017-17249-6.
   Web of Science ®Google Scholar
• BarnesPJ. Chronic obstructive pulmonary disease: effects beyond the lungs. PLoS Med. 2010;7:e1000220. doi:10.1371/journal.pmed.100022020305715
   PubMed Web of Science ®Google Scholar
• BarnesPJ. New anti-inflammatory targets for chronic obstructive pulmonary disease. Nat Rev Drug Discov. 2013;12:543–559. doi:10.1038/nrd402523977698
   PubMed Web of Science ®Google Scholar
• BártholoTP, da CostaCH, RufinoR. Evaluation of von Willebrand factor in COPD patients. J Bras Pneumol. 2014;40:373–379. doi:10.1590/S1806-3713201400040000425210959
   PubMed Web of Science ®Google Scholar
• BihletAR, KarsdalMA, SandJMB, et al. Biomarkers of extracellular matrix turnover are associated with emphysema and eosinophilic-bronchitis in COPD. Respir Res. 2017;18:22. doi:10.1186/s12931-017-0509-x28103932
   PubMed Web of Science ®Google Scholar
• BorchielliniA, FijnvandraatK, Ten CateJW, et al. Quantitative analysis of von Willebrand factor propeptide release in vivo: effect of experimental endotoxemia and administration of 1-deamino-8-D-arginine vasopressin in humans. Blood. 1996;88:2951–2958. doi:10.1182/blood.V88.8.2951.bloodjournal88829518874191
   PubMed Web of Science ®Google Scholar
• CockayneDA, ChengDT, WaschkiB, et al. Systemic biomarkers of neutrophilic inflammation, tissue injury and repair in COPD patients with differing levels of disease severity. PLoS One. 2012;7:e38629. doi:10.1371/journal.pone.003862922701684
   PubMed Web of Science ®Google Scholar
• CoppolaR, MariD, LattuadaA, FranceschiC. Von Willebrand factor in Italian centenarians. Haematologica. 2003;88:39–43.12551825
   PubMed Web of Science ®Google Scholar
• CrawleyJTB, de GrootR, XiangY, LukenBM, LaneDA, De GrootR. Unravelling the scissile bond: how ADAMTS13 recognises and cleaves von Willebrand factor. Blood. 2011;118:3212–3221. doi:10.1182/blood-2011-02-30659721715306
   PubMed Web of Science ®Google Scholar
• DonaldsonG, SeemungalT, BhowmikA, WedzichaJ. Relationship between exacerbation frequency and lung function decline in chronic obstructive pulmonary disease. Thorax. 2002;57:847–852. doi:10.1136/thorax.57.10.84712324669
   PubMed Web of Science ®Google Scholar
• FabbriLM, RabeKF. From COPD to chronic systemic inflammatory syndrome? Lancet. 2007;370:797–799. doi:10.1016/S0140-6736(07)61383-X17765529
   PubMed Web of Science ®Google Scholar
• FranchiniM. Hemostasis and aging. Crit Rev Oncol Hematol. 2006;60:144–151. doi:10.1016/j.critrevonc.2006.06.00416860994
   PubMed Web of Science ®Google Scholar
• GanWQ, ManSFP, SenthilselvanA, SinDD. Association between chronic obstructive pulmonary disease and systemic inflammation: a systematic review and a meta-analysis. Thorax. 2004;59:574–580. doi:10.1136/thx.2003.01958815223864
   PubMed Web of Science ®Google Scholar
• HoggJC. Pathophysiology of airflow limitation in chronic obstructive pulmonary disease. Lancet (London, England). 2004;364:709–721. doi:10.1016/S0140-6736(04)16900-6
   PubMed Web of Science ®Google Scholar
• HulsteinJJJ, de GrootPG, SilenceK, VeyradierA, FijnheerR, LentingPJ. A novel nanobody that detects the gain-of-function phenotype of von Willebrand factor in ADAMTS13 deficiency and von Willebrand disease type 2B. Blood. 2005;106:3035–3042. doi:10.1182/blood-2005-03-115316014562
   PubMed Web of Science ®Google Scholar
• HurstJR, VestboJ, AnzuetoA, et al. Susceptibility to exacerbation in chronic obstructive pulmonary disease. N Engl J Med. 2010;363:1128–1138. doi:10.1056/NEJMoa090988320843247
   PubMed Web of Science ®Google Scholar
• HyseniA, KempermanH, de LangeDW, KeseciogluJ, de GrootPG, RoestM. Active von Willebrand factor predicts 28-day mortality in patients with systemic inflammatory response syndrome. Blood. 2014;123:2153–2156. doi:10.1182/blood-2013-08-50809324458436
   PubMed Web of Science ®Google Scholar
• KaweckiC, LentingPJ, DenisCV. von Willebrand factor and inflammation. J Thromb Haemost. 2017;15:1285–1294. doi:10.1111/jth.2017.15.issue-728671350
   PubMed Web of Science ®Google Scholar
• LefrançaisE, Ortiz-MuñozG, CaudrillierA, et al. The lung is a site of platelet biogenesis and a reservoir for haematopoietic progenitors. Nature. 2017;544:105–109. doi:10.1038/nature2170628329764
   PubMed Web of Science ®Google Scholar
• MaclayJD, McAllisterDA, JohnstonS, et al. Increased platelet activation in patients with stable and acute exacerbation of COPD. Thorax. 2011;66:769–774. doi:10.1136/thx.2010.15752921507906
   PubMed Web of Science ®Google Scholar
• MacNeeW. Pathogenesis of chronic obstructive pulmonary disease. Proc Am Thorac Soc. 2005;2:258–66; discussion 290–1.
   Google Scholar
• ManninoD, Tal-SingerR, LomasD, et al. Plasma fibrinogen as a biomarker for mortality and hospitalized exacerbations in people with COPD. Chronic Obstr Pulm Dis J COPD Found. 2014;2:23–34. doi:10.15326/jcopdf.2.1.2014.0138
   Google Scholar
• Manon-JensenT, LangholmLL, RønnowSR, et al. End-product of fibrinogen is elevated in emphysematous chronic obstructive pulmonary disease and is predictive of mortality in the ECLIPSE cohort. Respir Med. 2019;160:105814. doi:10.1016/j.rmed.2019.10581431739246
   PubMed Web of Science ®Google Scholar
• Manon-JensenT, LangholmLL, SunS, et al. Initiation of the wound healing cascade in inflammatory bowel disease: assessment of Von Willebrand factor ADAMTS-13 processing and formation in crohn’s disease. EC Gastroenterol Dig Syst. 2019;6:143–154.
   Google Scholar
• van MourikJA, Romani de WitT. Von Willebrand factor propeptide in vascular disorders. Thromb Haemost. 2001;86:164–171. doi:10.1055/s-0037-161621411487004
   PubMed Web of Science ®Google Scholar
• NightingaleT, CutlerD. The secretion of von Willebrand factor from endothelial cells; an increasingly complicated story. J Thromb Haemost. 2013;11:192–201. doi:10.1111/jth.12225
   PubMed Web of Science ®Google Scholar
• PerotinJ-M, AdamD, Vella-BoucaudJ, et al. Delay of airway epithelial wound repair in COPD is associated with airflow obstruction severity. Respir Res. 2014;15:151. doi:10.1186/s12931-014-0151-925427655
   PubMed Web of Science ®Google Scholar
• PimandaJ, HoggP. Control of von Willebrand factor multimer size and implications for disease. Blood Rev. 2002;16:185–192. doi:10.1016/S0268-960X(02)00017-612163004
   PubMed Web of Science ®Google Scholar
• PoberJS, SessaWC. Evolving functions of endothelial cells in inflammation. Nat Rev Immunol. 2007;7:803–815. doi:10.1038/nri217117893694
   PubMed Web of Science ®Google Scholar
• PolatliM, ÇakirA, CildagO, BolamanAZ, YeniseyC, YeniceriogluY. Microalbuminuria, von Willebrand factor and fibrinogen levels as markers of the severity in COPD exacerbation. J Thromb Thrombolysis. 2008;26:97–102. doi:10.1007/s11239-007-0073-117622488
   PubMed Web of Science ®Google Scholar
• PolosaR, MalerbaM, CacciolaRR, et al. Effect of acute exacerbations on circulating endothelial, clotting and fibrinolytic markers in COPD patients. Intern Emerg Med. 2013;8:567–574. doi:10.1007/s11739-011-0636-121660493
   PubMed Web of Science ®Google Scholar
• SagheerS, RodgersS, YacoubO, DauerR, McraeS, DuncanE. Comparison of von Willebrand factor (VWF) activity levels determined by HemosIL AcuStar assay and HemosIL LIA assay with ristocetin cofactor assay by aggregometry. Haemophilia. 2016;22:e200–e207. doi:10.1111/hae.2016.22.issue-327076201
   PubMed Web of Science ®Google Scholar
• SandJMB, KnoxAJ, LangeP, et al. Accelerated extracellular matrix turnover during exacerbations of COPD. Respir Res. 2015;16:69. doi:10.1186/s12931-015-0225-326062683
   PubMed Web of Science ®Google Scholar
• SchneiderSW, NuscheleS, WixforthA, et al. Shear-induced unfolding triggers adhesion of von Willebrand factor fibers. Proc Natl Acad Sci U S A. 2007;104:7899–7903. doi:10.1073/pnas.060842210417470810
   PubMed Web of Science ®Google Scholar
• Soler-CataluñaJJ, Martínez-GarcíaMÁ, SánchezPR, SalcedoE, NavarroM, OchandoR. Disease patients with chronic obstructive pulmonary severe acute exacerbations and mortality in topic collections severe acute exacerbations and mortality in patients with chronic obstructive pulmonary disease. Thorax. 2005;60:925–931. doi:10.1136/thx.2005.04052716055622
   PubMed Web of Science ®Google Scholar
• VestboJ, AndersonW, CoxsonHO, et al. Evaluation of COPD longitudinally to identify predictive surrogate end-points (ECLIPSE). Eur Respir J. 2008;31:869–873. doi:10.1183/09031936.0011170718216052
   PubMed Web of Science ®Google Scholar
• VestboJ, EdwardsLD, ScanlonPD, et al.; ECLIPSE Investigators. Changes in forced expiratory volume in 1 second over time in COPD. N Engl J Med. 2011;365:1184–1192. doi:10.1056/NEJMoa110548221991892
   PubMed Web of Science ®Google Scholar
• VestboJ, HurdSS, AgustíAG, et al. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease GOLD executive summary. Am J Respir Crit Care Med. 2013;187:347–365. doi:10.1164/rccm.201204-0596PP22878278
   PubMed Web of Science ®Google Scholar
• WagnerDD. Cell biology of von Willebrand factor. Annu Rev Cell Biol. 1990;6:217–246. doi:10.1146/annurev.cb.06.110190.0012452275814
   PubMedGoogle Scholar