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Anti-Inflammatory Therapies for Cystic Fibrosis

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Treatment of Cystic Fibrosis and Other Rare Lung Diseases

Part of the book series: Milestones in Drug Therapy ((MDT))

Abstract

Cystic fibrosis (CF) lung disease is characterized by airway obstruction, chronic bacterial infection, and a self-propagating inflammatory response. These three factors are interrelated and feed into each other resulting in structural damage to the airway wall architecture, loss of lung function, and ultimately the death of the patient. Current maintenance regimens targeted toward treating the lung disease typically include airway clearance measures to relieve the airway obstruction and inhaled antibiotics to treat the chronic bacterial infection. Few patients receive chronic anti-inflammatory therapy. The first studies of anti-inflammatory drugs for CF began approximately 30 years ago with the first study of oral corticosteroids. Unfortunately, chronic systemic corticosteroid use was associated with significant side effects and therefore cannot be recommended for long-term use in treating CF airway inflammation. High-dose ibuprofen is the only anti-inflammatory drug currently recommended for the treatment of CF airway inflammation. Unfortunately, it has never been widely adopted primarily due to the requirement to obtain pharmacokinetic studies prior to initiating therapy and concerns over potential side effects. Clearly, new anti-inflammatory drugs are desperately needed in CF. Because massive neutrophil influx is the hallmark of airway inflammation in CF, any new anti-inflammatory drug must either directly or indirectly address the neutrophil and its damaging inflammatory products. In this chapter, the history of anti-inflammatory drug development in CF will be reviewed first, and then an update on current studies of anti-inflammatory drugs in the CF pipeline will be provided.

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Authors and Affiliations

  1. Department of Pediatrics, Case Western Reserve University School of Medicine, Rainbow Babies and Children’s Hospital, Cleveland, OH, 44106, USA

    Elliott C. Dasenbrook M.D., M.H.S. & James F. Chmiel M.D., M.P.H.

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  1. Elliott C. Dasenbrook M.D., M.H.S.
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  2. James F. Chmiel M.D., M.P.H.
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Correspondence to Elliott C. Dasenbrook M.D., M.H.S. .

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Editors and Affiliations

  1. Div of Pulmonary Medicine, Nippon Medical School Div of Pulmonary Medicine, Tokyo, Japan

    Arata Azuma

  2. Children's Hospital of Richmond, Virginia Commonwealth University Children's Hospital of Richmond, Richmond, Virginia, USA

    Michael S. Schechter

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Dasenbrook, E.C., Chmiel, J.F. (2017). Anti-Inflammatory Therapies for Cystic Fibrosis. In: Azuma, A., Schechter, M. (eds) Treatment of Cystic Fibrosis and Other Rare Lung Diseases. Milestones in Drug Therapy. Springer, Basel. https://doi.org/10.1007/978-3-0348-0977-1_8

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