Cushing Syndrome Treatment Plans

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Questions and Answers

What is the treatment of choice for ectopic ACTH syndrome?

Surgery and chemotherapy (correct)

Ketoconazole

Metyrapone

Mitotane

What is the maximum dose of Metyrapone?

10 g/day

6 g/day (correct)

4 g/day

8 g/day

Which medication is used to decrease GI effects in pituitary-dependent Cushing syndrome?

Ketoconazole

Food (correct)

Metyrapone

Mitotane

What is the dose of Mifepristone?

300 mg once daily Signup and view all the answers

What is the maximum dose of Cabergoline?

7 mg/week Signup and view all the answers

What is the treatment of choice for adrenal adenoma?

Surgery Signup and view all the answers

What is the dose of Pasireotide?

0.6-0.9 mg twice daily Signup and view all the answers

What is the treatment of choice for pituitary-dependent Cushing syndrome?

Surgery Signup and view all the answers

What is the primary cause of ACTH-dependent Cushing syndrome?

Pituitary adenomas Signup and view all the answers

What percentage of patients with Cushing syndrome have peripheral obesity and fat accumulation?

50% Signup and view all the answers

What is the term for excessive ACTH production resulting from an endocrine or nonendocrine tumor?

Ectopic ACTH syndrome Signup and view all the answers

What is the most common finding in patients with Cushing syndrome?

Central obesity and facial rounding Signup and view all the answers

What is the term for adrenal gland hypofunction?

Addison disease Signup and view all the answers

What is the usual cause of ACTH-independent Cushing syndrome?

Adrenal adenomas and carcinomas Signup and view all the answers

What is the percentage of ACTH-dependent Cushing syndrome cases caused by ectopic ACTH-secreting tumors and nonneoplastic corticotropin hypersecretion?

20% Signup and view all the answers

What is the term for the accumulation of fat in the dorsocervical area?

Buffalo hump Signup and view all the answers

What is the primary function of Mifepristone?

Antagonizing glucocorticoid receptors Signup and view all the answers

What is the FDA-approved indication for Mifepristone?

Treatment of Cushing syndrome in patients with type 2 diabetes or glucose intolerance Signup and view all the answers

What is the most common cause of primary hyperaldosteronism?

Bilateral adrenal hyperplasia Signup and view all the answers

What is the effect of Mifepristone on cortisol levels?

Increases cortisol levels Signup and view all the answers

What is a common side effect of Mifepristone?

Hypokalemia Signup and view all the answers

What is the purpose of close monitoring of 24-hour UFC and serum cortisol?

To identify adrenal insufficiency in patients with Cushing syndrome Signup and view all the answers

What is the category of hyperaldosteronism that is caused by bilateral adrenal hyperplasia?

Primary hyperaldosteronism Signup and view all the answers

What is the estimated percentage of patients who develop Cushing-induced osteoporosis?

60% Signup and view all the answers

Which of the following tests is used to determine the etiology of Cushing's syndrome?

Plasma ACTH Signup and view all the answers

What is the term for the type of familial hyperaldosteronism that is remediable by glucocorticoids?

Type I Signup and view all the answers

What is the goal of treatment for Cushing's syndrome?

All of the above Signup and view all the answers

Which of the following is a common psychiatric change associated with Cushing's syndrome?

None of the above Signup and view all the answers

What is the estimated percentage of patients who present with back pain?

40% Signup and view all the answers

Which of the following imaging tests is used to identify adrenal nodules and masses?

High-resolution CT scanning or MRI Signup and view all the answers

What is the estimated percentage of patients who progress to spinal compression fractures?

20% Signup and view all the answers

Which of the following tests is used to establish hypercortisolism?

All of the above Signup and view all the answers

What is the primary effect of mitotane on the adrenal cortex?

Atrophies the zona fasciculata and reticularis Signup and view all the answers

What is the primary mechanism of action of pasireotide in treating Cushing disease?

Activating somatostatin receptors to decrease ACTH secretion Signup and view all the answers

What is the primary reason for the limited use of cyproheptadine in treating Cushing disease?

It causes significant weight gain and sedation Signup and view all the answers

What is a common side effect of mitotane therapy that can be avoided by gradually increasing the dose and/or administering it with food?

Nausea and diarrhea Signup and view all the answers

What is the primary function of neurotransmitters such as serotonin, GABA, and acetylcholine in the context of Cushing disease?

To promote pituitary ACTH production Signup and view all the answers

What is the primary indication for pasireotide therapy?

Treatment of adults with Cushing disease for whom pituitary surgery is not an option or has not been curative Signup and view all the answers

What is a common CNS side effect of mitotane therapy?

Lethargy and somnolence Signup and view all the answers

What is the effect of mitotane on the zona glomerulosa during acute therapy?

It is minimally affected Signup and view all the answers

Study Notes

Treatment of Cushing Syndrome

Treatment plans are based on etiology, including surgery, chemotherapy, and medications such as Metyrapone, Ketoconazole, Mitotane, Mifepristone, Cabergoline, and Pasireotide.
Treatment options vary depending on the underlying cause, including ectopic ACTH syndrome, pituitary dependent Cushing syndrome, and adrenal adenoma or carcinoma.

Etiology of Cushing Syndrome

Cushing syndrome can result from exogenous administration of glucocorticoids or endogenous overproduction by the adrenal gland.
ACTH-dependent Cushing syndrome (80% of cases) is usually caused by overproduction of ACTH by the pituitary gland, causing bilateral adrenal hyperplasia.
Pituitary adenomas account for about 85% of ACTH-dependent cases, while ectopic ACTH secreting tumors and nonneoplastic corticotropin hypersecretion cause the remaining 20% of cases.
ACTH-independent Cushing syndrome is usually caused by adrenal adenomas and carcinomas.

Clinical Presentation

Common findings in Cushing syndrome include central obesity, facial rounding, and peripheral obesity.
Other symptoms may include myopathy, muscular weakness, abdominal striae, hypertension, glucose intolerance, psychiatric changes, gonadal dysfunction, and amenorrhea and hirsutism in women.
Up to 60% of patients develop Cushing-induced osteoporosis, and about 40% present with back pain.

Diagnosis

Hypercortisolism can be established with one or more of the following tests: 24-hour urinary free cortisol, midnight plasma cortisol, late-night salivary cortisol, and/or low-dose dexamethasone suppression test.
Other tests to determine etiology include plasma ACTH, adrenal vein catheterization, metyrapone stimulation test, adrenal, chest, or abdominal computed tomography (CT), corticotropin-releasing hormone (CRH) stimulation test, inferior petrosal sinus sampling, and pituitary magnetic resonance imaging (MRI).

Goals of Treatment

Goals of treatment include limiting morbidity and mortality, removing the source of hypercortisolism, and minimizing pituitary or adrenal deficiencies.
Mitotane degenerates cells within the zona fasciculata and reticularis, resulting in atrophy of the adrenal cortex.

Neuromodulators of ACTH Release

Pituitary secretion of ACTH is normally mediated by neurotransmitters such as serotonin, γ-aminobutyric acid (GABA), acetylcholine, and catecholamines.
Agents that target these transmitters have been proposed for treatment of Cushing disease, including cyproheptadine, bromocriptine, cabergoline, valproic acid, octreotide, lanreotide, pasireotide, rosiglitazone, and tretinoin.
Pasireotide (Signifor) is approved for treatment of adults with Cushing disease for whom pituitary surgery is not an option or has not been curative.

Glucocorticoid Receptor Blocking Agents

Mifepristone (Korlym) is a progesterone and glucocorticoid receptor antagonist that inhibits dexamethasone suppression and increases endogenous cortisol and ACTH levels in normal subjects.
Evidence suggests that mifepristone is highly effective in reversing the manifestations of hypercortisolism (hyperglycemia, hypertension, and weight gain).

Evaluation of Therapeutic Outcomes

Close monitoring of 24-hour UFC and serum cortisol is essential to identify adrenal insufficiency in patients with Cushing syndrome.
Monitor steroid secretion with all drug therapy (except mifepristone) and give corticosteroid replacement if needed.

Hyperaldosteronism

Hyperaldosteronism involves excess aldosterone secretion and is categorized as either primary (stimulus arising from within the adrenal gland) or secondary (stimulus from extra-adrenal etiologies).
Primary hyperaldosteronism (PA) is usually caused by bilateral adrenal hyperplasia and aldosterone-producing adenoma (Conn syndrome).

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Description

This quiz covers the treatment plans for Cushing syndrome based on etiology. It includes information on nondrug and drug treatment options, including dosing ranges for medications such as Metyrapone.