Exercise testing in adults with cystic fibrosis

Cystic fibrosis (CF), the most frequent genetic disease in the Caucasian population, is characterised by absent or incorrect function of the channel that regulates chloride exchange at the cell surface. The lungs are particularly involved as the very thick and tenacious mucus leads to progressive airflow limitation, respiratory infections, bronchiectasis, lung destruction and, ultimately, respiratory failure. Despite the remarkable advances in treatment which translated into improved survival, most patients present with progressively poorer exercise-based evaluations in the forthcoming years. In particular, CPET is useful to determine the mechanisms of exercise intolerance in individual patients who may have treatment and prognostic implications. Moreover, the test is frequently valuable to assess the effects of therapeutic interventions, such as exercise-based rehabilitation programmes.