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The Role of Inhaled Corticosteroids in the Management of Cystic Fibrosis

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Abstract

The lung disease of cystic fibrosis (CF) is characterized by a vicious cycle of airway obstruction, chronic bacterial infection, and vigorous inflammation, which ultimately results in bronchiectasis. Recognition that excessive and persistent inflammation is a key factor in lung destruction has prompted investigation into anti-inflammatory therapies. Although effective, the use of systemic corticosteroids has been limited by the unacceptable adverse effect profile. Inhaled corticosteroids (ICS) are a widely prescribed anti-inflammatory agent in CF, likely as a result of clinicians’ familiarity with these agents and their excellent safety profile at low doses in asthmatic patients. However, while multiple studies are limited by small sample size and short duration, they consistently failed to demonstrate statistically or clinically significant benefits of ICS use in CF. This review provides an overview of the inflammatory response in CF, the mechanisms of action of corticosteroids, the safety of ICS, and the literature relevant to the use of ICS in CF.

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Acknowledgements

Grant support from the National Institutes of Health Grant P30-DK27651, KL2RR024990, and the Cystic Fibrosis Foundation is gratefully acknowledged. The authors have no conflicts of interest that are directly relevant to the content of this review.

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  1. Department of Pediatrics, Division of Pulmonology, Case Western Reserve University School of Medicine, Rainbow Babies and Children’s Hospital, Cleveland, Ohio, USA

    Kristie R. Ross, James F. Chmiel & Michael W. Konstan

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  2. James F. Chmiel
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Ross, K.R., Chmiel, J.F. & Konstan, M.W. The Role of Inhaled Corticosteroids in the Management of Cystic Fibrosis. Pediatr-Drugs 11, 101–113 (2009). https://doi.org/10.2165/00148581-200911020-00002

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