Antibiotic treatment for Burkholderia cepacia complex in people with cystic fibrosis experiencing a pulmonary exacerbation

Abstract

Background

Chronic pulmonary infection is a hallmark of lung disease in cystic fibrosis. Infections dominated by organisms of the Burkholderia cepacia complex, a group of at least 18 closely‐related species of gram‐negative bacteria, are particularly difficult to treat. These infections may be associated with a fulminant necrotising pneumonia. Burkholderia cepacia complex bacteria are resistant to many common antibiotics and able to acquire resistance against many more. Following patient segregation in cystic fibrosis medical care, the more virulent epidemic strains are not as frequent, and new infections are more likely to be with less virulent environmentally‐acquired strains. Although evidence‐based guidelines exist for treating respiratory exacerbations involving Pseudomonas aeruginosa, these cannot be extended to Burkholderia cepacia complex infections. This review, which is an update of a previous review, aims to assess the available trial evidence for the choice and application of treatments for these infections.

Objectives

To assess the effectiveness and safety of different antibiotic regimens in people with cystic fibrosis experiencing an exacerbation and chronically infected with organisms of the Burkholderia cepacia complex.

Search methods

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.

Date of latest search: 29 May 2019.

Selection criteria

Randomised and quasi‐randomised controlled trials of treatments for exacerbations of pulmonary symptoms in people with cystic fibrosis chronically infected with organisms of the Burkholderia cepacia complex.

Data collection and analysis

No relevant trials were identified.

Main results

No trials were included in this review.

Authors' conclusions

Burkholderia cepacia complex infections present a significant challenge for people with cystic fibrosis and their clinicians. The incidence is likely to increase as the cystic fibrosis population ages; and managing and treating these infections will become more important. There is a lack of trial evidence to guide decision making and no conclusions can be drawn from this review about the optimal antibiotic regimens for people with cystic fibrosis who have chronic Burkholderia cepacia complex infections. Clinicians must continue to assess each person individually, taking into account in vitro antibiotic susceptibility data, previous clinical responses and their own experience. Multicentre randomised clinical trials are needed to assess the effectiveness of different antibiotic regimens in people with cystic fibrosis infected with organisms of the Burkholderia cepacia complex.

Plain language summary

Which antibiotics are best to treat worsening symptoms in people with cystic fibrosis with persistent Burkholderia cepacia complex lung infection?

Review question

We looked for evidence of which antibiotics are best to treat a flare up of symptoms in people with cystic fibrosis with persistent Burkholderia cepacia complex lung infection.

Background

Cystic fibrosis is a common inherited condition where the lungs often become blocked with mucus. This harms the lungs' defences and often results in chronic, persistent infections that cannot be cleared by antibiotics. People with cystic fibrosis often need courses of antibiotics to reduce their symptoms (for instance cough, excess mucus and breathlessness) when these flare up or worsen. Such episodes are called exacerbations, and are usually treated with intravenous antibiotics (given through a drip into a vein). One group of bacteria that can infect the lungs of people with cystic fibrosis is called the Burkholderia cepacia complex. These closely‐related bacteria are found widely in the environment and do not cause infections in healthy people who do not have cystic fibrosis. They are particularly hard to treat as they are resistant to many commonly‐used antibiotics. Currently doctors do not know which antibiotics are best at treating these infections; which combinations of antibiotics should be used; how long the antibiotics should be used for; or whether there are additional treatments that might also help. This is an update of a previously published review.

Search date

The evidence is current to: 29 May 2019.

Study characteristics

We did not find any trials in people with exacerbations of cystic fibrosis who were infected with Burkholderia cepacia complex bacteria, where they were given different treatments at random.

Key results

More research is needed to find out which treatments are best for improving lung function and survival and for reducing side effects and length of time spent in hospital for people infected with Burkholderia cepacia complex bacteria experiencing an exacerbation.