Cystic fibrosis (CF) is a heritable progressive disease caused by mutations in the CTFR gene that results in the build up of thick and sticky mucus in various organs. Because the CTFR gene is highly expressed in the lungs, this organ is highly affected by such mutations.

Common respiratory system symptoms in CF include airway obstruction, chronic bacterial infection, and recurrent inflammation that can lead to bronchiectasis, or damaged and widened airways.

Oral corticosteroids are useful in treating lung inflammation in CF, but can have considerable side effects. Inhaled corticosteroids have fewer side effects since they act locally in the airways, and they are often used to treat CF. Clinicians also tend to prescribe them because they are familiar with inhaled corticosteriods, as they are often used by asthmatic patients.

A clear benefit in CF patients, however, has not been established. A systematic review that analyzed 13 clinical trials concluded that evident is not sufficient to support inhaled corticosteroids use in helping to ease lung inflammation in adults or children with CF.

How inhaled corticosteroids work

As a response to viral or bacterial infection, the body recruits neutrophils — a type of immune cell — to the airways. This inflammatory response is intended to fight the infection, but a prolonged and excessive response can damage the lungs, which is what happens in CF.

Inhaled corticosteroids work by binding to corticosteroid receptors in the membrane (the outer layer) of neutrophils in the airways. The corticosteroid-receptor complex then activates anti-inflammatory genes and blocks pro-inflammatory genes, to reduce the inflammatory response. To be effective, it is crucial that the corticosteroids reach the site of inflammation.

Ways to administer corticosteroids

Different options exist to administer corticosteroids for inhalation. A spacer device is a commonly used option that reduces the amount of medication deposited into the mouth. It can be used by patients of all ages, and is a safe way to deliver high doses of the medication.

Another option is to inhale the dry powder. This method is suitable for patients age 5 or older.

Corticosteroids can also be directly sprayed into the mouth with a meter-dose inhaler. This inhaler can be difficult to use, and is not suitable for children.

Inhaled corticosteroids that might be prescribed to CF patients include fluticasone, beclomethasone, and budesonide.

Side effects

Regular use of corticosteroids may slow growth in children.

Rarely, corticosteroids can interfere with the production of particular hormones. This can result in a wide range of problems, one example being low blood sugar.

The inhalation of corticosteroids can facilitate a fungal infection, which results in oral thrush. Rinsing the mouth after inhalation helps reduce this risk.

Hoarseness of the voice has been reported with inhaled corticosteroid use, but does not appear to be a common side effect.

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Cystic Fibrosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.