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SOURCE

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Source: Hwang AY, Smith SM, Gums JG. Adrenal gland disorders. In: DiPiro JT, Talbert RL, Yee GC, Matzke GR, Wells BG, Posey LM, eds. Pharmacotherapy: A Pathophysiologic Approach. 10th ed. New York, NY: McGraw-Hill; 2017. http://accesspharmacy.mhmedical.com/content.aspx?bookid=1861&sectionid=134128262. Accessed March 7, 2017.

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CONDITION/DISORDER SYNONYM

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  • Hypercortisolism.

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DEFINITION

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  • Clinical features resulting from chronic exposure to excess glucocorticoids of any cause.

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ETIOLOGY

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  • Pituitary adenomas (Cushing disease)

  • Ectopic adrenocorticotropic hormone (ACTH)-secreting tumors.

  • Adrenal adenomas or carcinomas.

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PATHOPHYSIOLOGY

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  • Results from effects of supraphysiologic levels of glucocorticoids caused by exogenous administration or endogenous overproduction by adrenal gland (adrenocorticotropic hormone [ACTH]-dependent) or by abnormal adrenocortical tissues (ACTH-independent)

  • Ectopic ACTH syndrome is excessive ACTH production resulting from endocrine or nonendocrine tumor, usually of pancreas, thyroid, or lung.

  • ACTH-independent Cushing syndrome usually caused by adrenal adenomas and carcinomas.

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EPIDEMIOLOGY

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  • Annual incidence of Cushing syndrome: 13 cases per million people.

  • Approximately 70% of cases due to Cushing disease, 15% to ectopic ACTH-secreting tumors, and 15% to adrenal tumors.

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RISK FACTORS

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  • Chronic corticosteroid use.

  • Age 20–50 years.

  • Female sex.

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CLINICAL PRESENTATION

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SIGNS AND SYMPTOMS
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  • Central obesity and facial rounding (90% of patients)

  • Peripheral obesity and fat accumulation (50%)—fat accumulation in dorsocervical area (buffalo hump); moon facies.

  • Myopathy (65%) or muscular weakness (58%)

  • Striae along lower abdomen that may have red to purple color.

  • Hypertension (75–85%)

  • Glucose intolerance (60%)

  • Psychiatric changes (55%)

  • Osteoporosis (50–60%)—typically presenting with back pain.

  • Gonadal dysfunction with amenorrhea in up to 75% of women.

  • Excess androgen secretion with hirsutism in 80% of women.

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DIAGNOSIS

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LABORATORY TESTS
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  • 24-hour urinary free cortisol (UFC), midnight plasma cortisol, late-night (11 PM) salivary cortisol, and/or low-dose dexamethasone suppression test (DST) can identify hypercortisolism.

  • Plasma ACTH test; metyrapone stimulation test; corticotropin-releasing hormone (CRH) stimulation test; and inferior petrosal sinus sampling may be performed.

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IMAGING
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  • High-resolution CT and MRI may identify pituitary and adrenal masses.

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DIFFERENTIAL DIAGNOSIS
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  • Primary and secondary aldosteronism.

  • Iatrogenic Cushing due to exogenous factors.

  • Pseudo-Cushing due to other disease states mimicking Cushing symptoms.

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DESIRED OUTCOMES

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  • Limit morbidity and mortality.

  • Return patient to normal functional state by removing source of hypercortisolism without causing pituitary or adrenal deficiencies.

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TREATMENT: GENERAL APPROACH

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  • See Table 1 for treatment plans based on etiology.

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Table Graphic Jump Location
TABLE 1.Possible Treatment Plans in Cushing Syndrome Based on Etiology

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