Cushing’s Syndrome is a rare disease characterised by persistent high levels of cortisol, a hormone that triggers anti-inflammatory actions in response to stressful situations such as illness or injury.
Excessive secretion of cortisol can lead to chronic disorders, such as hypertension and glucose intolerance. Other Cushing’s Syndrome symptoms include easy bruising, facial plethora, depression, decreased libido and osteopenia. If left untreated, Cushing’s Syndrome can become life-threatening as a result of malignancy, overwhelming infection and organ failure.
High levels of cortisol may be due to endogenous triggers such as endocrine tumours (pituitary, adrenal or ectopic tumours), or exogenous sources such as high doses of corticosteroid treatment. Cortisol release may be further influenced by high levels of adrenocorticotropic hormone (ACTH), a factor that regulates cortisol release from the adrenal glands. Diagnosis of Cushings’ syndrome is therefore based on progressive signs and symptoms and multiple tests including the urinary free-cortisol test, salivary cortisol test and the low-dose dexamethasone suppression test (LDDST).
If possible and appropriate, first-line treatment is surgical resection of any primary tumours (pituitary adenoma, non-pituitary tumour secreting ACTH, or adrenal tumours). Second-line therapies include repeat transsphenoidal surgery (TSS), radiation therapy/radiosurgery, bilateral adrenalectomy and treatments such as neuromodulators of ACTH release, adrenal steroidogenesis inhibitors and glucocorticoid receptor blockers.
Related news and insights
Xeris Biopharma announced that the European Journal of Endocrinology (EJE) published the extended evaluation (EE) results of the SONICS study (NCT01838551) evaluating longer-term effects of Recorlev (levoketoconazole) on cortisol levels, biomarkers of Cushing’s syndrome (CS) comorbidities, clinical signs and symptoms of CS, and quality of life. The manuscript also reports findings from pituitary adenoma imaging in the SONICS study
Recordati Rare Diseases announces that positive results from the Phase III LINC 4 study of Isturisa were presented on March 22 at the Endocrine Society’s Annual Meeting. Results from LINC 4, the first Phase III study in patients with Cushing’s disease to include an upfront, double-blind, randomised, placebo controlled period, demonstrated that Isturisa provided rapid and sustained normalisation of mean urinary free cortisol (mUFC) levels.