Treatments to cure long-term infections with Burkholderia cepacia in people with cystic fibrosis

Review question

We reviewed the evidence for antibiotic treatment to cure early infection with Burkholderia cepacia complex in people with cystic fibrosis and prevent it becoming permanent.

Background

Cystic fibrosis is an inherited disease and people who have this disease produce large amounts of thick mucus which is difficult to clear. This mucus blocks up their lungs and digestive systems. People with cystic fibrosis suffer from lots of chest infections, which cause scarring of their airways. Eventually, they develop infections that can't be cured with antibiotics, so their lungs always contain lots of bugs, this is described as being chronically infected. One of these bugs, Burkholderia cepacia, causes a lot of problems for people with cystic fibrosis because it is very difficult to treat and makes their lung disease deteriorate faster than it otherwise would. This is an updated version of the review.

Search date

We last searched for any evidence on 14 July 2016.

Study characteristics

We looked for studies of treatments which could eliminate Burkholderia cepacia from the lungs of people with cystic fibrosis. We did not find any relevant studies. This review highlights an urgent need for more research into new ways of treating long-term Burkholderia cepacia infection in people with cystic fibrosis.

Authors' conclusions: 

The authors have concluded that there was an extreme lack of evidence in this area of treatment management for people with cystic fibrosis. Without further comprehensive studies, it is difficult to draw conclusions about a safe and effective management strategy for Burkholderia cepacia complex eradication in cystic fibrosis. Thus, while the review could not offer clinicians evidence of an effective eradication protocol for Burkholderia cepacia complex, it has highlighted an urgent need for exploration and research in this area, specifically the need for well-designed multi-centre randomised controlled studies of a variety of (novel) antibiotic agents.

Read the full abstract...
Background: 

Chronic infection with Burkholderia cepacia complex species remains a significant problem for clinicians treating people with cystic fibrosis. Colonisation with Burkholderia cepacia complex species is linked to a more rapid decline in lung function and increases morbidity and mortality. There remain no objective guidelines for strategies to eradicate Burkholderia cepacia complex in cystic fibrosis lung disease, as these are inherently resistant to the majority of antibiotics and there has been very little research in this area. This review aims to examine the current treatment options for people with cystic fibrosis with acute of Burkholderia cepacia complex and to identify an evidence-based strategy that is both safe and effective. This is an updated version of the review.

Objectives: 

To identify whether treatment of Burkholderia cepacia complex infections can achieve eradication, or if treatment can prevent or delay the onset of chronic infection. To establish whether following eradication, clinical outcomes are improved and if there are any adverse effects.

Search strategy: 

We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched the reference lists of relevant articles and reviews.

Last search: 14 July 2016.

We also searched electronic clinical trials registers for the USA and Europe.

Date of last search: 14 July 2016.

Selection criteria: 

Randomised or quasi-randomised studies in people with cystic fibrosis of antibiotics or alternative therapeutic agents used alone or in combination, using any method of delivery and any treatment duration, to eradicate Burkholderia cepacia complex infections compared to another antibiotic, placebo or no treatment.

Data collection and analysis: 

Two authors independently assessed for inclusion in the review the eligibility of 50 studies (70 references) identified by the search of the Group's Trial Register and the other electronic searches.

Main results: 

No studies looking at the eradication of Burkholderia cepacia complex species were identified.

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