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

Clin Imaging. 2022 Apr:84:118-129. doi: 10.1016/j.clinimag.2022.02.004. Epub 2022 Feb 12.

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.

Publication types

  • Review

MeSH terms

  • Adult
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Cystic Fibrosis* / complications
  • Cystic Fibrosis* / diagnostic imaging
  • Cystic Fibrosis* / genetics
  • Humans
  • Radiography
  • Radiologists

Substances

  • Cystic Fibrosis Transmembrane Conductance Regulator