We planned to review the evidence about the effect of treatments to fight fungal infections that cause allergic bronchopulmonary aspergillosis (ABPA) in people with cystic fibrosis.
ABPA is an allergic lung reaction to a type of fungus (usually Aspergillus fumigatus) in some people with cystic fibrosis. It causes a cough and wheezing, and sometimes fever. If left untreated, ABPA can lead to long-term lung damage. It is usually treated with a high dose of corticosteroids (also known as steroids). However, it has not been proven that corticosteroids can prevent lung function from deteriorating in the long term. Also, long-term use of corticosteroids is linked to some serious side effects. Treating the fungus that causes ABPA may be an alternative to using high doses of corticosteroids to combat the allergic reaction. This is an update of a previously published review.
The evidence is current to 28 September 2021.
We did not find any trials that we could include in the review.
There is currently no evidence to recommend the use of antifungal treatment in people with cystic fibrosis and ABPA.
We need trials to assess the effects of corticosteroids in this population in the long and the short term.
At present, there are no randomised controlled trials that evaluate the use of antifungal therapies for the treatment of ABPA in people with cystic fibrosis, although one trial is currently ongoing.
Trials with clear outcome measures are needed to properly evaluate the use of corticosteroids in people with ABPA and cystic fibrosis.
Allergic bronchopulmonary aspergillosis (ABPA) is an allergic reaction to colonisation of the lungs with the fungus Aspergillus fumigatus, and affects around 10% of people with cystic fibrosis. ABPA is associated with an accelerated decline in lung function. High doses of corticosteroids are the main treatment for ABPA; although the long-term benefits are not clear, and their many side effects are well-documented. A group of compounds, the azoles, have activity against A fumigatus, and have been proposed as an alternative treatment for ABPA. Of this group, itraconazole is the most active. A separate antifungal compound, amphotericin B, has been used in aerosolised form to treat invasive infection with A fumigatus, and may have potential for the treatment of ABPA. Antifungal therapy for ABPA in cystic fibrosis needs to be evaluated. This is an update of a previously published review.
The review aimed to test the hypotheses that antifungal interventions for the treatment of ABPA in cystic fibrosis:
1. improve clinical status compared to placebo or standard therapy (no placebo); and
2. do not have unacceptable adverse effects.
If benefit was demonstrated, we planned to assess the optimal type, duration, and dose of antifungal therapy.
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register, which comprises references identified from comprehensive electronic database searches, handsearches of relevant journals, and abstract books of conference proceedings. Date of the most recent search of the Group's Trials Register was 28 September 2021.
We searched ongoing trials registries, most recently on 11 March 2022.
Earlier, we also approached pharmaceutical companies regarding possible unpublished trials.
Published or unpublished randomised controlled trials, in which antifungal treatments were compared to either placebo or no treatment, or where different doses of the same treatment were used in the treatment of ABPA in people with cystic fibrosis.
The searches identified six trials; none of which met the inclusion criteria for the review.
We included no completed randomised controlled trials. There is currently one ongoing trial, which we may find eligible for a future update.