Cushing Syndrome

Definitions

Cushing syndrome: Elevated cortisol levels from any cause (exogenous or endogenous, ACTH-dependent or independent)

Cushing disease: Hypercortisolism specifically due to an ACTH-secreting pituitary adenoma

Signs/Symptoms

  • Hypertension
  • Hyperglycemia
  • Abdominal striae
  • Lipid re-distribution
    • Central obesity
    • “Moon” facies
    • “Buffalo hump” on back of neck
  • Fatigue/weakness

Workup

Management:

Cushing’s Disease (ACTH-secreting pituitary adenoma) (1)

1st Line

  • Surgical resection (works in ~2/3 of patients) is first line

2nd Line

  • Pituitary radiotherapy (may induce pituitary insufficiency)
  • Adrenal surgery (induces adrenal insufficiency)
  • Medical therapy
    • Ketoconazole (inhibits steroid production) is the most commonly used drug
    • Mifepristone (glucocorticoid receptor antagonist) mainly for glucose intolerance
    • Cabergoline (dopamine agonist)
    • Pasireotide (somatostatin analog)

Adrenal hyperplasia/tumors

  • Referral for adrenalectomy

Ectopic ACTH production (2)

  • Tumor excision
  • Medical therapy if surgery is not possible or if the source of ACTH secretion is unknown
    • Metyrapone, mitotane, ketoconazole, or etomidate (steroidogenesis inhibitors)

References:

  1. Pivonello, R., De Leo, M., Cozzolino, A., & Colao, A. (2015, August). The treatment of cushing’s disease. Retrieved March 31, 2021, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4523083/
  2. Sakihara, S., Kageyama, K., Yamagata, S., Terui, K., Daimon, M., & Suda, T. (2014). A case of ectopic acth syndrome treated with intermittent administration of dopamine agonists. Retrieved March 31, 2021, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3965275/

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