Ageing with cystic fibrosis: Classical and emerging comorbidities in adults with cystic fibrosis

Cystic fibrosis (CF) is a genetic disease with pulmonary involvement being pre-dominant and often leading to respiratory failure and premature death. Non-pulmonary complications related to CF transmembrane conductance regulator (CFTR) defect are numerous and account for significant morbidity (exocrine pancreatic insufficiency, diabetes, CF-related liver disease, chronic sinusitis, osteoporosis). Improvement in patients' care led to a continuous increase in life expectancy, with a subsequent increase in the number of adult CF patients world-wide. Increased life expectancy comes with increased prevalence of CF-related comorbidities, but also with new emerging complications directly related to ageing (chronic kidney disease, cardiovascular risk factors, cancers). CFTR modulators might also contribute to modify the face of CF epidemiology and prognosis. Ageing with CF has become a challenge for CF patients and caregivers. This review summarizes classic and emerging comorbidities in the context of current growth and ageing of the CF population. It also addresses potential roles of CFTR modulators. (C) 2018 Elsevier Masson SAS. All rights reserved.