Adrenal function in cystic fibrosis

Maria Socorro Rayas*, Andrea Kelly, Kara S. Hughan, Tanicia Daley, David Zangen

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

2 Scopus citations


Cystic fibrosis (CF) is not known to directly affect the adrenal gland, but commonly used CF therapies do impact the function of the hypothalamic-pituitary-adrenal (HPA) axis. By binding to the glucocorticoid receptor, medications such as inhaled and oral corticosteroids can enhance the systemic effects of cortisol and result in iatrogenic Cushing syndrome. Prolonged use suppresses the body's ability to make cortisol, resulting in iatrogenic adrenal insufficiency upon medication discontinuation. Chronic use of inhaled and oral corticosteroids can negatively affect bone health, growth, and glucose metabolism. This chapter provides practical guidelines regarding the screening, diagnosis, and treatment of iatrogenic adrenal insufficiency. As the guidelines are mainly derived from the asthma literature, this chapter also highlights the need for studies to evaluate the impact of CF therapies on adrenal function and other CF-endocrinopathies.

Original languageAmerican English
Pages (from-to)S74-S81
JournalJournal of Cystic Fibrosis
StatePublished - Oct 2019

Bibliographical note

Funding Information:
The authors would like to acknowledge the Cystic Fibrosis Foundation for the grant support through the Emerging Leaders in CF Program.

Publisher Copyright:
© 2019 European Cystic Fibrosis Society


  • ACTH stimulation test
  • Corticosteroids
  • Cortisol
  • Cystic fibrosis
  • Iatrogenic Cushing syndrome
  • Iatrogenic adrenal insufficiency


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