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Review
. 2016 Aug;63(4):617-36.
doi: 10.1016/j.pcl.2016.04.003.

Cystic Fibrosis: Microbiology and Host Response

Edith T Zemanick  1 , Lucas R Hoffman  2
Affiliations
Review

Cystic Fibrosis: Microbiology and Host Response

Edith T Zemanick et al. Pediatr Clin North Am. 2016 Aug.

Abstract

The earliest descriptions of lung disease in people with cystic fibrosis (CF) showed the involvement of 3 interacting pathophysiologic elements in CF airways: mucus obstruction, inflammation, and infection. Over the past 7 decades, our understanding of CF respiratory microbiology and inflammation has evolved with the introduction of new treatments, increased longevity, and increasingly sophisticated laboratory techniques. This article reviews the current understanding of infection and inflammation and their roles in CF lung disease. It also discusses how this constantly evolving information is used to inform current therapeutic strategies, measures and predictors of disease severity, and research priorities.

Keywords: Biomarker; Cystic fibrosis; Infection; Inflammation; Microbiology; Neutrophil.

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Figures

Figure 1
Figure 1
Epidemiology of traditional CF pathogens in 2013, from the US CF Patient Registry. (a) Percent of patients reported to be culture-positive for the indicated bacteria in 2013, by age group. (b) Overall prevalence in CF patients per year of these bacteria. Cystic fibrosis patients under care at CF Foundation-accredited care centers in the United States, who consented to have their data entered. Courtesy of CF Foundation, Broomall, PA.
Figure 1
Figure 1
Epidemiology of traditional CF pathogens in 2013, from the US CF Patient Registry. (a) Percent of patients reported to be culture-positive for the indicated bacteria in 2013, by age group. (b) Overall prevalence in CF patients per year of these bacteria. Cystic fibrosis patients under care at CF Foundation-accredited care centers in the United States, who consented to have their data entered. Courtesy of CF Foundation, Broomall, PA.
Figure 2
Figure 2
Pathophysiology of lung disease in CF. (a) Traditional view of pathology of lung disease in CF. Inflammation is triggered by chronic bacterial infection. (b) Potential alternative mechanism for airway inflammation in CF. Underlying CFTR dysfunction contributes to altered host-response. From: Rao S and Grigg J, New insights into pulmonary inflammation in cystic fibrosis Arch Dis Child. 2006 Sep; 91(9): 786–788; with permission.
Figure 2
Figure 2
Pathophysiology of lung disease in CF. (a) Traditional view of pathology of lung disease in CF. Inflammation is triggered by chronic bacterial infection. (b) Potential alternative mechanism for airway inflammation in CF. Underlying CFTR dysfunction contributes to altered host-response. From: Rao S and Grigg J, New insights into pulmonary inflammation in cystic fibrosis Arch Dis Child. 2006 Sep; 91(9): 786–788; with permission.
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