Medical therapy of hypercortisolism (Cushing's syndrome)
- Lynnette K Nieman, MD
Lynnette K Nieman, MD
- Section Editor — Adrenal Disease
- Senior Investigator
- Bethesda, MD
The hypercortisolism of Cushing's syndrome is primarily treated surgically, regardless of its cause (ie, due to corticotropin [ACTH]-producing pituitary tumor [Cushing's disease], ectopic ACTH secretion by a nonpituitary tumor, or cortisol secretion by an adrenal adenoma or carcinoma). However, when surgery is delayed, contraindicated, or unsuccessful, medical therapy is often required. Adrenal enzyme inhibitors are the most commonly used drugs, but adrenolytic agents, drugs that target the pituitary, and glucocorticoid-receptor antagonists also have been used.
The pharmacologic management of hypercortisolemia in Cushing's syndrome will be reviewed here. An overview of the treatment options for Cushing's syndrome and additional details about drugs that inhibit cortisol synthesis are discussed separately. (See "Overview of the treatment of Cushing's syndrome" and "Pharmacology and toxicity of adrenal enzyme inhibitors and adrenolytic agents" and "Persistent or recurrent Cushing's disease: Surgical adrenalectomy".)
The main indications for medical therapy of Cushing's syndrome include (see "Overview of the treatment of Cushing's syndrome"):
●Management of hypercortisolism when surgery is contraindicated
●Control of hypercortisolism in preparation for surgery
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- SUGGESTED APPROACH
- Replacement glucocorticoid therapy
- Monitoring efficacy of treatment
- ADRENAL ENZYME INHIBITORS
- Oral drugs
- - Ketoconazole
- - Metyrapone
- Intravenous etomidate
- ADRENOLYTIC AGENTS
- - Dose
- - Side effects
- OTHER AGENTS
- Drugs that target pituitary
- - Cabergoline
- - Pasireotide
- - Combination therapy
- Glucocorticoid-receptor antagonists (mifepristone)
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS