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Review
. 2019 Nov;25(6):636-645.
doi: 10.1097/MCP.0000000000000616.

Prevention of chronic infection with Pseudomonas aeruginosa infection in cystic fibrosis

Affiliations
Review

Prevention of chronic infection with Pseudomonas aeruginosa infection in cystic fibrosis

Edith T Zemanick et al. Curr Opin Pulm Med. 2019 Nov.

Abstract

Purpose of review: This review provides an update on definitions of chronicity of infection, approaches to airway sampling to detect infection, strategies for Pseudomonas aeruginosa eradication, impact of cystic fibrosis transmembrane regulator protein (CFTR) modulators and future challenges for clinical trials.

Recent findings: Rates of P. aeruginosa have decreased over the past two decades with establishment of effective eradication protocols. Definitions of chronic P. aeruginosa infection have required adaptation for healthier populations. Although molecular (PCR) approaches to early P. aeruginosa detection are sensitive, to date, earlier diagnosis has not impacted on clinical outcomes. Despite eradication regimens, some people with early P. aeruginosa fail to clear their infection. Most people also experience a recurrence and eventual transition to chronic infection. Several recent studies sought to address this gap. CFTR modulators (predominantly ivacaftor) demonstrated reduced P. aeruginosa density, although infection may persist or recur demonstrating the need for continued antiinfective therapies in the modulator era.

Summary: Future studies of approaches to P. aeruginosa eradication will be complex due to expanded availability and ongoing competitive clinical trials of CFTR modulators. Studies to address optimal eradication therapy, particularly in adults, will be required, though adequate recruitment to power these studies may prove challenging.

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Conflict of interest statement

Conflicts of interest

S.C.B. has received travel support for participation in investigator meetings and institutional support for participation in Symposia (Vertex, Gilead, Novartis). He has been a site PI for studies sponsored by Vertex, Galapagos, Flatley and Pharmaxis. He has been global PI for a study sponsored by Galapagos. E.T.Z. has been a site PI for studies sponsored by Vertex, Nivalis and Savara.

Figures

FIGURE 1.
FIGURE 1.
Median FEV1% predicted for people with cystic fibrosis in the United States by age (1998–2018). Source of data: Cystic fibrosis patients under care at CF Foundation accredited care centre in the United States, who consented to have their data entered. Reproduced with permission from [7].
FIGURE 2.
FIGURE 2.
P. aeruginosa infection status based on Leeds criteria of people with cystic fibrosis in the United States by age (2017). Source of data: Cystic fibrosis patients under care at CF Foundation accredited care centre in the United States, who consented to have their data entered. Reproduced with permission from [7].

References

    1. Cystic Fibrosis Foundation Patient Registry. 2017 annual data report. Bethesda, Maryland: Cystic Fibrosis Foundation; 2018. https://www.cff.org/Research/Researcher-Resources/Patient-Registry/2017-... [Accessed 3 July 2019].
    1. Elborn JS. Cystic fibrosis. Lancet 2016; 388:2519–2531. - PubMed
    1. Hoch H, Sontag MK, Scarbro S, et al. Clinical outcomes in US infants with cystic fibrosis from 2001 to 2012. Pediatr Pulmonol 2018; 53:1492–1497.

      This study demonstrated decreased P. aeruginosa culture positivity in infants following expansion of NBS in the U.S.

    1. Salsgiver EL, Fink AK, Knapp EA, et al. Changing epidemiology of the respiratory bacteriology of patients with cystic fibrosis. Chest 2016; 149:390–400. - PMC - PubMed
    1. Wainwright CE, Vidmar S, Armstrong DS, et al. Effect of bronchoalveolar lavage-directed therapy on Pseudomonas aeruginosa infection and structural lung injury in children with cystic fibrosis: a randomized trial. JAMA 2011; 306:163–171. - PubMed

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