Clinical presentation of lung disease in cystic fibrosis

Although cystic fibrosis (CF) is a multiorgan disease, the extent of CF lung disease is decisive for the course and survival of patients. The optimization of symptomatic therapies has led to a significant improvement in the life expectancy of those affected in recent decades. Regular monitoring of the course of CF lung disease with microbiological, pulmonary function, and imaging examinations is essential for early detection of problems and individualized therapy. With new, causal therapy options in the form of cystic fibrosis transmembrane conductance regulator (CFTR) modulators and early diagnosis through newborn screening, a further normalization of life expectancy and quality of life of CF patients can be expected.