Cushing syndrome

The clinical manifestation of pathologic hypercortisolism from any cause.

Cushing disease, which is hypercortisolism caused by an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma, is the most common cause of Cushing syndrome, and is responsible for 70% to 80% of cases.

Though uncommon, the prevalence of endogenous Cushing syndrome is greater than previously thought.

It may be difficult to distinguish patients with mild Cushing syndrome from those with the metabolic syndrome (central obesity with insulin resistance, and hypertension). Features more specific to Cushing syndrome include proximal muscle weakness, supraclavicular fat pads, facial plethora, violaceous striae, easy bruising, and premature osteoporosis.

After exclusion of exogenous corticosteroid use, patients with suspected Cushing syndrome should be tested for hypercortisolism with 1 of 4 high-sensitivity tests (late-night salivary cortisol; 1 mg overnight low-dose dexamethasone suppression testing, 24-hour urinary free cortisol; or 48-hour 2 mg dexamethasone suppression testing).

At least one additional test should be used to confirm hypercortisolism in patients with a positive initial screening test.

Once endogenous hypercortisolism is confirmed, plasma ACTH should be measured. If ACTH is suppressed, diagnostic testing should focus on the adrenal glands. If ACTH is not suppressed, pituitary or ectopic disease should be sought.

In the vast majority of cases of endogenous Cushing syndrome, surgical resection of the pituitary adenoma or adrenal adenoma that is causing hypercortisolism is the primary treatment of choice.

Cushing syndrome is the clinical manifestation of pathologic hypercortisolism from any cause. Patients often display weight gain with central obesity, facial rounding and plethora, proximal muscle weakness, and thinning of the skin. They also develop metabolic complications including diabetes mellitus, dyslipidemia, metabolic bone disease, and hypertension. Cushing syndrome can be caused by adrenocorticotropic hormone (ACTH)-secreting pituitary tumors (termed Cushing disease), by autonomous adrenal cortisol overproduction, and, rarely, by ectopic ACTH-secreting tumors.[1]Lacroix A, Feelders RA, Stratakis CA, et al. Cushing's syndrome. Lancet. 2015 Aug 29;386(9996):913-27. http://www.thelancet.com/journals/lancet/article/PIIS0140-6736(14)61375-1/fulltext http://www.ncbi.nlm.nih.gov/pubmed/26004339?tool=bestpractice.com