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American Journal of Respiratory and Critical Care Medicine Vol 168. pp. 918-951, (2003)
© 2003 American Thoracic Society

State of the Art

Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis

Ronald L. Gibson, Jane L. Burns and Bonnie W. Ramsey

Department of Pediatrics, University of Washington School of Medicine, Children's Hospital, and Regional Medical Center, Seattle, Washington

Correspondence and requests for reprints should be addressed to Bonnie W. Ramsey, M.D., Professor of Pediatrics, Department of Pediatrics, University of Washington School of Medicine, 2611 NE 125th Street, Suite 90, Seattle, WA 98125. E-mail: bonnie.ramsey{at}seattlechildrens.org

This comprehensive State of the Art review summarizes the current published knowledge base regarding the pathophysiology and microbiology of pulmonary disease in cystic fibrosis (CF). The molecular basis of CF lung disease including the impact of defective cystic fibrosis transmembrane regulator (CFTR) protein function on airway physiology, mucociliary clearance, and establishment of Pseudomonas aeruginosa infection is described. An extensive review of the microbiology of CF lung disease with particular reference to infection with P. aeruginosa is provided. Other pathogens commonly associated with CF lung disease including Staphylococcal aureus, Burkholderia cepacia, Stenotrophomonas maltophilia, Achromobacter xylosoxidans and atypical mycobacteria are also described. Clinical presentation and assessment of CF lung disease including diagnostic microbiology and other measures of pulmonary health are reviewed. Current recommendations for management of CF lung disease are provided. An extensive review of antipseudomonal therapies in the settings of treatment for early P. aeruginosa infection, maintenance for patients with chronic P. aeruginosa infection, and treatment of exacerbation in pulmonary symptoms, as well as antibiotic therapies for other CF respiratory pathogens, are included. In addition, the article discusses infection control policies, therapies to optimize airway clearance and reduce inflammation, and potential future therapies.

Key Words: cystic fibrosis • Pseudomonas aeruginosa • airway disease • cystic fibrosis transmembrane conductance regulator • antibiotics




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Surfactant Protein A2 (SP-A2) Variants Expressed in CHO Cells Stimulate Phagocytosis of Pseudomonas aeruginosa More than Do SP-A1 Variants
Infect. Immun., March 1, 2007; 75(3): 1403 - 1412.
[Abstract] [Full Text] [PDF]

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 PediatricsHome page
C. L. Ren
Pulmonary Manifestations in {Delta}F508/R117H
Pediatrics, March 1, 2007; 119(3): 647 - 647.
[Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
P. Moskwa, D. Lorentzen, K. J. D. A. Excoffon, J. Zabner, P. B. McCray Jr., W. M. Nauseef, C. Dupuy, and B. Banfi
A Novel Host Defense System of Airways Is Defective in Cystic Fibrosis
Am. J. Respir. Crit. Care Med., January 15, 2007; 175(2): 174 - 183.
[Abstract] [Full Text] [PDF]

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 J. Clin. Microbiol.Home page
S. Besier, C. Smaczny, C. von Mallinckrodt, A. Krahl, H. Ackermann, V. Brade, and T. A. Wichelhaus
Prevalence and Clinical Significance of Staphylococcus aureus Small-Colony Variants in Cystic Fibrosis Lung Disease
J. Clin. Microbiol., January 1, 2007; 45(1): 168 - 172.
[Abstract] [Full Text] [PDF]

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 Proc. Natl. Acad. Sci. USAHome page
L. R. Hoffman, E. Deziel, D. A. D'Argenio, F. Lepine, J. Emerson, S. McNamara, R. L. Gibson, B. W. Ramsey, and S. I. Miller
Selection for Staphylococcus aureus small-colony variants due to growth in the presence of Pseudomonas aeruginosa
PNAS, December 26, 2006; 103(52): 19890 - 19895.
[Abstract] [Full Text] [PDF]

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 Proc. Natl. Acad. Sci. USAHome page
J. F. Linares, I. Gustafsson, F. Baquero, and J. L. Martinez
Antibiotics as intermicrobial signaling agents instead of weapons
PNAS, December 19, 2006; 103(51): 19484 - 19489.
[Abstract] [Full Text] [PDF]

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 J. Immunol.Home page
J. L. Koff, M. X. G. Shao, S. Kim, I. F. Ueki, and J. A. Nadel
Pseudomonas Lipopolysaccharide Accelerates Wound Repair via Activation of a Novel Epithelial Cell Signaling Cascade
J. Immunol., December 15, 2006; 177(12): 8693 - 8700.
[Abstract] [Full Text] [PDF]

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 J. Clin. Microbiol.Home page
S. E. Sharp and C. Searcy
Comparison of Mannitol Salt Agar and Blood Agar Plates for Identification and Susceptibility Testing of Staphylococcus aureus in Specimens from Cystic Fibrosis Patients
J. Clin. Microbiol., December 1, 2006; 44(12): 4545 - 4546.
[Abstract] [Full Text] [PDF]

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 ThoraxHome page
J K Block, K L Vandemheen, E Tullis, D Fergusson, S Doucette, D Haase, Y Berthiaume, N Brown, P Wilcox, P Bye, et al.
Predictors of pulmonary exacerbations in patients with cystic fibrosis infected with multi-resistant bacteria
Thorax, November 1, 2006; 61(11): 969 - 974.
[Abstract] [Full Text] [PDF]

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 Antimicrob. Agents Chemother.Home page
X. Qin, Y. Razia, J. R. Johnson, J. R. Stapp, D. R. Boster, T. Tsosie, D. L. Smith, C. R. Braden, K. Gay, F. J. Angulo, et al.
Ciprofloxacin-resistant gram-negative bacilli in the fecal microflora of children.
Antimicrob. Agents Chemother., October 1, 2006; 50(10): 3325 - 3329.
[Abstract] [Full Text] [PDF]

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 J Antimicrob ChemotherHome page
O. Burkhardt, C. Lehmann, R. Madabushi, V. Kumar, H. Derendorf, and T. Welte
Once-daily tobramycin in cystic fibrosis: better for clinical outcome than thrice-daily tobramycin but more resistance development?
J. Antimicrob. Chemother., October 1, 2006; 58(4): 822 - 829.
[Abstract] [Full Text] [PDF]

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 Mol. Pharmacol.Home page
K. Zaman, S. Carraro, J. Doherty, E. M. Henderson, E. Lendermon, L. Liu, G. Verghese, M. Zigler, M. Ross, E. Park, et al.
S-Nitrosylating Agents: A Novel Class of Compounds That Increase Cystic Fibrosis Transmembrane Conductance Regulator Expression and Maturation in Epithelial Cells
Mol. Pharmacol., October 1, 2006; 70(4): 1435 - 1442.
[Abstract] [Full Text] [PDF]

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 MicrobiologyHome page
A. Emam, A. R. Yu, H.-J. Park, R. Mahfoud, J. Kus, L. L. Burrows, and C. A. Lingwood
Laboratory and clinical Pseudomonas aeruginosa strains do not bind glycosphingolipids in vitro or during type IV pili-mediated initial host cell attachment.
Microbiology, September 1, 2006; 152(Pt 9): 2789 - 2799.
[Abstract] [Full Text] [PDF]

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 PediatricsHome page
K. J. Smith, O. Elidemir, M. K. Dishop, K. W. Eldin, N. Tatevian, and R. H. Moore
Intravenous Injection of Pharmaceutical Tablets Presenting as Multiple Pulmonary Nodules and Declining Pulmonary Function in an Adolescent With Cystic Fibrosis
Pediatrics, September 1, 2006; 118(3): e924 - e928.
[Abstract] [Full Text] [PDF]

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 ThoraxHome page
G A Tramper-Stranders, C K van der Ent, M G Slieker, S W J Terheggen-Lagro, F Teding van Berkhout, J L L Kimpen, and T F W Wolfs
Diagnostic value of serological tests against Pseudomonas aeruginosa in a large cystic fibrosis population
Thorax, August 1, 2006; 61(8): 689 - 693.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
D. L. Chen, T. W. Ferkol, M. A. Mintun, J. E. Pittman, D. B. Rosenbluth, and D. P. Schuster
Quantifying Pulmonary Inflammation in Cystic Fibrosis with Positron Emission Tomography
Am. J. Respir. Crit. Care Med., June 15, 2006; 173(12): 1363 - 1369.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
P. K. Lam, D. E. Griffith, T. R. Aksamit, S. J. Ruoss, S. M. Garay, C. L. Daley, and A. Catanzaro
Factors Related to Response to Intermittent Treatment of Mycobacterium avium Complex Lung Disease
Am. J. Respir. Crit. Care Med., June 1, 2006; 173(11): 1283 - 1289.
[Abstract] [Full Text] [PDF]

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 Antimicrob. Agents Chemother.Home page
D. H. Kwon and C.-D. Lu
Polyamines Increase Antibiotic Susceptibility in Pseudomonas aeruginosa.
Antimicrob. Agents Chemother., May 1, 2006; 50(5): 1623 - 1627.
[Abstract] [Full Text] [PDF]

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 Antimicrob. Agents Chemother.Home page
Y. Nalca, L. Jansch, F. Bredenbruch, R. Geffers, J. Buer, and S. Haussler
Quorum-Sensing Antagonistic Activities of Azithromycin in Pseudomonas aeruginosa PAO1: a Global Approach.
Antimicrob. Agents Chemother., May 1, 2006; 50(5): 1680 - 1688.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
S. Sethi, J. Maloney, L. Grove, C. Wrona, and C. S. Berenson
Airway Inflammation and Bronchial Bacterial Colonization in Chronic Obstructive Pulmonary Disease
Am. J. Respir. Crit. Care Med., May 1, 2006; 173(9): 991 - 998.
[Abstract] [Full Text] [PDF]

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 J. Immunol.Home page
R. T. Sadikot, H. Zeng, M. Joo, M. B. Everhart, T. P. Sherrill, B. Li, D.-s. Cheng, F. E. Yull, J. W. Christman, and T. S. Blackwell
Targeted Immunomodulation of the NF-{kappa}B Pathway in Airway Epithelium Impacts Host Defense against Pseudomonas aeruginosa.
J. Immunol., April 15, 2006; 176(8): 4923 - 4930.
[Abstract] [Full Text] [PDF]

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 Antimicrob. Agents Chemother.Home page
M. D. Macia, N. Borrell, M. Segura, C. Gomez, J. L. Perez, and A. Oliver
Efficacy and Potential for Resistance Selection of Antipseudomonal Treatments in a Mouse Model of Lung Infection by Hypermutable Pseudomonas aeruginosa
Antimicrob. Agents Chemother., March 1, 2006; 50(3): 975 - 983.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Cell Mol. Bio.Home page
T. J. Moraes, J. Plumb, R. Martin, E. Vachon, V. Cherepanov, A. Koh, C. Loeve, J. Jongstra-Bilen, J. H. Zurawska, J. V. Kus, et al.
Abnormalities in the Pulmonary Innate Immune System in Cystic Fibrosis
Am. J. Respir. Cell Mol. Biol., March 1, 2006; 34(3): 364 - 374.
[Abstract] [Full Text] [PDF]

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 Am. J. Physiol. Cell Physiol.Home page
A. Swiatecka-Urban, S. Moreau-Marquis, D. P. MacEachran, J. P. Connolly, C. R. Stanton, J. R. Su, R. Barnaby, G. A. O'Toole, and B. A. Stanton
Pseudomonas aeruginosa inhibits endocytic recycling of CFTR in polarized human airway epithelial cells
Am J Physiol Cell Physiol, March 1, 2006; 290(3): C862 - C872.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
L. A. Kalish, D. A. Waltz, M. Dovey, G. Potter-Bynoe, A. J. McAdam, J. J. LiPuma, C. Gerard, and D. Goldmann
Impact of Burkholderia dolosa on Lung Function and Survival in Cystic Fibrosis
Am. J. Respir. Crit. Care Med., February 15, 2006; 173(4): 421 - 425.
[Abstract] [Full Text] [PDF]

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 ChestHome page
V. Starosta, E. Rietschel, K. Paul, U. Baumann, and M. Griese
Oxidative Changes of Bronchoalveolar Proteins in Cystic Fibrosis.
Chest, February 1, 2006; 129(2): 431 - 437.
[Abstract] [Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
A. B. de Gonzalez and J. M. Samet
What Are the Cancer Risks from Using Chest Computed Tomography to Manage Cystic Fibrosis?
Am. J. Respir. Crit. Care Med., January 15, 2006; 173(2): 139 - 140.
[Full Text] [PDF]

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 Am. J. Respir. Crit. Care Med.Home page
P. A. de Jong, J. R. Mayo, K. Golmohammadi, Y. Nakano, M. H. Lequin, H. A. W. M. Tiddens, J. Aldrich, H. O. Coxson, and D. D. Sin
Estimation of Cancer Mortality Associated with Repetitive Computed Tomography Scanning
Am. J. Respir. Crit. Care Med., January 15, 2006; 173(2): 199 - 203.
[Abstract] [Full Text] [PDF]

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 ThoraxHome page
P A de Jong, A Lindblad, L Rubin, W C J Hop, J C de Jongste, M Brink, and H A W M Tiddens
Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis
Thorax, January 1, 2006; 61(1): 80 - 85.
[Abstract] [Full Text] [PDF]


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