Recurrent exacerbations affect FEV1 decline in adult patients with cystic fibrosis

SummaryObstructive lung disease is the major cause of morbidity and mortality in cystic fibrosis (CF). To identify risk factors contributing to FEV1 decline in CF patients, we carried out a retrospective analysis of clinical and pulmonary function data in a population of CF patients followed up for 5 years and studied the correlation between clinical data and FEV1 decline.Fifty-one adult CF patients were studied. The FEV1 decline was related to the following clinical characteristics: CFTR genotype, age, gender, weight, height, age at diagnosis, baseline FEV1, pancreatic function, presence of airway infection, pancreatic insufficiency and diabetes, number of exacerbations/year and intravenous (i.v.) antibiotic courses/year.Both the number of exacerbations/year and the number of i.v. antibiotic courses/year were strongly related to the FEV1 decline. Patients with airway infection or with diabetes had significantly lower FEV1 values during the study as compared with non-infected patients or patients without diabetes; however, both the presence of airway infection or diabetes did not affect the FEV1 decline.These results suggest that the aggressive treatment of disease exacerbations is crucial for delaying lung function decline in CF.