Purpose of review: This review will discuss the challenges of defining a pulmonary exacerbations in cystic fibrosis (CF) and the key pathogens which contribute. It will discuss the treatment options currently available and the importance of preventing pulmonary exacerbations.
Recent findings:The basis for treatment of pulmonary exacerbations remains unchanged over the past 15 years and whilst there have been trials exploring alternative antibiotics, there has been little change. However, there are ongoing studies that are expected to establish a platform for identifying best practices. Chronic CF therapies have been shown to reduce pulmonary exacerbations. In the era of new CFTR (CF transmembrane conductance regulator) modulator therapies, the number of pulmonary exacerbations are expected to be even fewer. However, it is unclear whether the other chronic therapies can be discontinued without losing their benefits in reducing exacerbations.
Summary:Although there is no universal definition of a pulmonary exacerbation in CF, proposed definitions have many similarities. We have outlined the current recommendations for treatment of pulmonary exacerbations, including the duration and location of treatments. We have also summarised the key therapies used for prevention of pulmonary exacerbations in CF.
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