Cystic fibrosis in the 21st century: what every radiologist should know

Abstract

Cystic Fibrosis (CF) is the most common lethal genetic disorder in Caucasian populations, affecting roughly 70,000 individuals worldwide. This autosomal recessive disorder causes a wide spectrum of multisystemic manifestations, most of which are either directly or indirectly related to defective epithelial chloride secretion. The current median life expectancy is 44 years; however, a significant proportion of the CF population now live into the 5th decade and beyond due to advances in treatment. As life expectancy of CF patients increases, there is a newly emerging adult CF population with unique radiological manifestations spanning multiple organ systems, which often require follow-up imaging. The goal of this article is to review the multiple systemic manifestations and complications of CF on different imaging modalities and explore the appropriate radiological follow up recommended.

Keywords: CT scan; Complications; Cystic fibrosis; Diagnosis; Follow-up; MRI.