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KEY CONCEPTS

KEY CONCEPTS

  • imageGlucocorticoid secretion from the adrenal cortex is stimulated by adrenocorticotropic hormone (ACTH) or corticotropin that is released from the anterior pituitary in response to the hypothalamic-mediated release of corticotropin-releasing hormone (CRH).

  • imageTo ensure the proper treatment of Cushing syndrome, diagnostic procedures should (1) establish the presence of hypercortisolism and (2) discover the underlying etiology of the disease.

  • imageThe rationale for treating Cushing syndrome is to reduce the morbidity and mortality resulting from disorders such as diabetes mellitus, cardiovascular disease, and electrolyte abnormalities.

  • imageThe treatment of choice for both ACTH-dependent and ACTH-independent Cushing syndrome is surgery. Pharmacologic agents are reserved for adjunctive therapy, refractory cases, or inoperable disease.

  • imagePharmacologic agents that may be used to manage the patient with Cushing syndrome include steroidogenesis inhibitors, adrenolytic agents, neuromodulators of ACTH release, and glucocorticoid-receptor blocking agents.

  • imageSpironolactone, a competitive aldosterone-receptor antagonist, is the drug of choice in bilateral adrenal hyperplasia (BAH)–dependent hyperaldosteronism.

  • imageAddison’s disease (primary adrenal insufficiency) is a state of deficiency in cortisol, aldosterone, and various androgens due to the loss of function in all regions of the adrenal cortex.

  • imageSecondary adrenal insufficiency usually results from exogenous steroid use, leading to hypothalamic–pituitary–adrenal (HPA)–axis suppression followed by a decrease in ACTH release, and low levels of androgens and cortisol.

  • imageVirilism results from the excessive secretion of androgens from the adrenal gland and often manifests as hirsutism in females.

PATIENT CARE PROCESS

Patient Care Process for Cushing Syndrome

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Collect

  • Patient characteristics (eg, age, sex, pregnant)

  • Patient medical history (personal and family)

  • Social history (eg, tobacco/ethanol use)

  • Iatrogenic causes of Cushing syndrome: current medications including corticosteroids (all routes, past and present), medroxyprogesterone acetate, megestrol acetate, gamma-hydroxybutyic acid, CYP3A4 inhibitors and inducers

  • Objective data

    • Blood pressure, heart rate, body mass index

    • Test for hypercortisolism: 24-hour UFC, midnight plasma cortisol, late-night salivary cortisol, or low-dose dexamethasone suppression test

    • Follow-up diagnostic tests to differentiate etiologies (see Fig. 93-4)

Assess

  • Presence of Cushing syndrome complications:

    • Metabolic: impaired glucose metabolism, dyslipidemia

    • Cardiovascular: hypertension, vascular damage, thrombosis, hypokalemia

    • Immunologic: bacterial, fungal, and viral infections; rebound autoimmunity Musculoskeletal: osteopenia/osteoporosis, myopathy

    • Neuropsychiatric: depression, anxiety, bipolar disorder

    • Reproductive: decreased libido, hypogonadism (men), menstrual irregularity (women)

    • Dermatologic: hirsutism, alopecia, hyperhidrosis

  • Physical exam: Peripheral obesity, fat accumulation (Buffalo Hump), rounded face (moon face), striae, ecchymosis, hyperpigmentation, acanthosis nigricans, acne, and thin skin

  • Current medications that may contribute to or worsen Cushing syndrome.

  • Results of follow-up diagnostic testing for etiology (see Fig. 93-4, and Tables 93-2 and 93-3)

  • Ability/willingness to pursue surgical/chemotherapeutic (if indicated) versus medical management

Plan*

  • Ensure proper administration of necessary corticosteroid therapy; discontinue unnecessary corticosteroid therapy, with taper if HPA axis integrity is suspect

  • Nondrug options for endogenous Cushing syndrome, depending on etiology: surgery, chemotherapy, irradiation, postoperative steroid replacement (see Table 93-4)

  • Steroid replacement regimens postoperatively for patients with adrenal adenomas (see Table 93-7)

  • Medical management when surgery is not possible or against patient wishes (see Tables 93-5 and 93-6 for ...

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