Abstract
Cystic fibrosis (CF) lung disease is characterized by the development of progressive
bronchiectasis and impaired lung function with severe airflow obstruction. CF patients
suffer from shortened life expectancy, primarily driven by respiratory failure. The
mechanism by which CF lung disease develops is the result of an interplay of multiple
intrinsic and extrinsic factors including genotype, abnormalities in mucus composition
and movement, chronic inflammation, and chronic airway infection. Although all CF
patients are at increased risk for pulmonary complications including hemoptysis, pneumothorax,
pulmonary hypertension, and chronic hypoxic and hypercapnic respiratory failure, the
risk of developing these complications increases with progression of lung disease.
The focus of this article is to summarize the pathophysiology, epidemiology, and management
of these key pulmonary complications.
Keywords
cystic fibrosis - complications - hemoptysis - pneumothorax - pulmonary hypertension
- respiratory failure
