Is there a role for inhaled anti-inflammatory drugs in cystic fibrosis treatment?

ABSTRACT

Introduction: Cystic fibrosis (CF) is a congenital life-limiting, orphan disease affecting 1/2500 – 1/3000 people worldwide with the greatest prevalence in Europe, North America and Australia. The primary reason underpinning the cause of morbidity and mortality of CF patients is associated with recurrent pulmonary inflammation and infection that leads to chronic, progressive lung deterioration and ultimately death of CF patients.

Areas covered: This review aims to explore the potential role for inhaled anti-inflammatory drugs as a more successful treatment option for CF, in comparison with current oral delivery. Specifically, the focus is on ibuprofen, the only nonsteroidal anti-inflammatory drug approved for chronic use in CF. The need for inhalation therapy has also been highlighted with an insight on the reasons and challenges associated with developing an inhalation therapy of nonsteroidal anti-inflammatory drugs (NSAIDs).

Expert opinion: There is a fundamental need to direct research towards development of anti-inflammatory drugs to control inflammation rather than just targeting infection. Development of an inhalable preparation of ibuprofen alone or in combination with an antibiotic holds the potential to be the most effective treatment option among the existing array of therapies available for CF.

KEYWORDS:

• Cystic fibrosis
• ibuprofen
• inhaled therapy
• NSAID

Article highlights

• Anti-inflammatory drugs may reduce the inflammatory cascade, ameliorating the symptoms and reducing the long-term decline of pulmonary function in CF patients.

• Antibiotics continue to remain the mainstay of CF treatment, with very little focus given to anti-inflammatory agents.

• Oral NSAIDs are associated with several adverse events, particularly related to gastrointestinal bleeding and dose-related issues, consequently not widely used for CF treatment.

• The pulmonary route represents a versatile, highly promising and, until recently, less exploited route of drug delivery for NSAIDs for CF.

• An inhalable form of ibuprofen, alone or in combination with an antibiotic, could hold the potential to revolutionize the therapeutic approaches for CF, and may also reduce the treatment burden of the CF community in the long term.

Declaration of interest

Z Sheikh is a recipient of University of Sydney International Scholarship (Strategic). The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed. Peer reviewers on this manuscript have no relevant financial or other relationships to disclose

Additional information

Funding

This paper is not funded.