Cystic fibrosis (CF) is a complex genetic disease affecting many organs, although 85% of the mortality is a result of lung disease. The natural history of the lung disease consists of early and persistent infection, an exaggerated inflammatory response, structural airway changes (i.e. bronchiectasis), and progressive airways obstruction, ultimately resulting in respiratory failure. As airways disease worsens, there is an increased likelihood of respiratory complications, such as pneumothorax, that may be serious. This review describes our current understanding of the pathogenesis of pneumothorax in CF and its treatment.

RECENT FINDINGS: The CF Foundation Pulmonary Therapies Committee recently published their recommendations for the treatment of hemoptysis and pneumothorax. As insufficient data exist from which a systematic review of the literature could be used to develop guidelines, the recommendations were derived from a formalized expert panel consensus process.

SUMMARY: We now have recommendations on specific care of the patient with CF who has a pneumothorax.