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Original Research: Genetic and Developmental Disorders |

Systematic Review of Blood Biomarkers in Cystic Fibrosis Pulmonary ExacerbationsBlood Biomarkers in Cystic Fibrosis Exacerbations

Alborz Hakimi Shoki, BSc; Nicole Mayer-Hamblett, PhD; Pearce G. Wilcox, MD; Don D. Sin, MD, FCCP; Bradley S. Quon, MD
Author and Funding Information

From the University of Sydney Medical School (Mr Shoki), the University of Sydney, Sydney, NSW, Australia; the Department of Pediatrics (Dr Mayer-Hamblett), University of Washington, Seattle, WA; James Hogg Research Centre (Drs Wilcox, Sin, and Quon), St. Paul’s Hospital, Vancouver, BC, Canada; and the Division of Respiratory Medicine (Drs Wilcox, Sin, and Quon), Department of Medicine, University of British Columbia, Vancouver, BC, Canada.

Correspondence to: Bradley S. Quon, MD, Division of Respiratory Medicine, 8B Providence Wing – 1081 Burrard St, Vancouver, BC, V6Z 1Y6, Canada; e-mail: bradley.quon@hli.ubc.ca


Funding/Support: Dr Quon was supported by a Canadian Institute of Health Research (CIHR) Fellowship Award funded by the University of British Columbia and the Government of Canada.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2013;144(5):1659-1670. doi:10.1378/chest.13-0693
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Background:  Biomarkers reflective of disease activity in cystic fibrosis (CF) have the potential to improve patient care, particularly during CF pulmonary exacerbations (CFPEs). Although blood-based biomarkers have been studied in CFPE for nearly 3 decades, none have been integrated into routine clinical practice. To facilitate progress in this area, we performed a systematic review evaluating blood-based biomarkers during CFPE.

Methods:  MEDLINE, EMBASE, and CENTRAL were searched to identify relevant studies published from January 1995 to August 2012. We included all full-text studies examining systemic (blood-based) biomarkers to aid in the diagnosis of CFPE, predict outcomes of CFPE, and/or monitor the response to CFPE treatment.

Results:  Seventy-eight unique blood-based biomarkers have been studied to date, mainly inflammatory cytokines, acute phase reactants, and markers of oxidative stress. C-reactive protein (CRP) consistently correlated with disease activity, with a statistically significant increase from stable to exacerbation state in five of six studies, and changes in response to CFPE treatment, with a statistically significant decrease from the beginning to the end of CFPE treatment in 18 of 20 studies. Other promising biomarkers of CFPE disease activity include neutrophil elastase antiproteinase complex, IL-6, myeloperoxidase (MPO), lactoferrin, and calprotectin.

Conclusions:  Although there are several blood-based biomarkers with evidence for application within the CFPE setting, CRP has been the most widely studied biomarker demonstrating the potential for clinical usefulness. Further validation studies and clinical trials are required to determine whether blood-based biomarkers can be used to ultimately improve health outcomes in the setting of a CFPE.

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