(!) Cushing's syndrome

Question 1

Define Cushings syndrome

Answer 1

set of symptoms caused by excess circulating cortisol in the body-pathological hypercortisolism from any cause

Cushing disease is specifically when a secondary cause-pituary adenoma secreting ACTH is the cause
Other causes include adrenal adenoma, ectopic secreting ACTH tumour, and excessive oral intake

Question 2

Aetiology and risk factors of Cushings Disease

Answer 2

Cushing disease-adrenal adenoma secreting ACTH, causes 80% of cushings syndrome
But only 10% of ACTH secreting adenoma lead to Sx-most are silent
10% are adrenal adenomas, with 5% developing cortisol prod
Exogenous-taking to much steroids-most common cause

Risk factors
Women>men
Age 20-50
Exogenous-taking steroids -most common

Question 3

Epidiemology of Cushings Syndrome

Answer 3

Uncommon -7.5 in 1 million

More common in women than men

Question 4

Signs and Sx of Cushings syndrome

Answer 4

Facial plethora/red flush
Fat in weird places-supraclavicular
Lemon on stick look-weight gain and central obesity
Moon face and back hump

Easy bruising
Weak limbs
Purple striae on skin. (stretch marks)
Thin skin

depression
New/worse hypertension
headache

from Cushing disease-excess pigmentation

Hirsutism
Decreased libido
Erectile dysfunction

Diabetes miellitus
Osteoporosis

absence of pregnancy, malnutrition, alcoholism
Weird periods

Question 5

Investigations of Cushings Syndrome

Answer 5

Glucose-diabetes
pregnancy-negative
late night cortisol-higher than expected
Hypokalemia
Hypernatremia

Low dose Dexamethasone suppression test (either overnight or 48h)
–doesnt suppress if bushings syndrome

High dose supression-
Doesn’t supress if from adrenal. Suppress if ACTH causative

PIT MRI

Question 6

Management of Cushings Syndrome

Answer 6

Cushings disease-transsphenoidal resection of tumour

medical 2nd line (surgery wasn’t enough-Pasireotide is a somatostatin analogue)
3rd line-radiotherapy
Might or might not need cortisol replacement

Syndrome
unilateral adrenalectomy of the affected adrenal gland-curative and doesn’t need replace

Ectopic ACTH-cure the original cancer
but hard so
glucocorticoid receptor antagonist (mifepristone)
Osilodrostat is a potent oral inhibitor of steroidogenesis (inhibits steroid 11-beta-hydroxylase) and is generally well tolerated
Or bilateral adrenalectomy

Question 7

Complications of Cushings Syndrome

Answer 7

After surgery-lots less ACTH, or no more adrenals–adrenal insufficiency

main cause of death is cushings-CVD (from HTN and Diabetes)
HTN, Diabetes
Oestoporois

Question 8

Prognosis of Cushings syndrome

Answer 8

Untreated-50% death in 5 years–CVD deaths

If cortisol is normalised-can go down to normal population death

Pit adenoma-microtumour better than large (lots of remission)

Adrenal adenoma-after surgery, great

Ectopic-depends on cuase-if lung bad