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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 14, 2017.

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CONDITION/DISORDER SYNONYMS

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  • Addison disease.

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DEFINITION

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  • Hypofunction of adrenal gland caused by primary (Addison disease) or secondary adrenal insufficiency.

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ETIOLOGY

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  • Autoimmune dysfunction (eg, Graves disease)

  • Autoimmune polyendocrine syndrome.

  • Tuberculosis.

  • Bilateral adrenalectomy.

  • Medications (ketoconazole, phenytoin, rifampin, phenobarbital)

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PATHOPHYSIOLOGY

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  • Occurs when adrenal glands do not produce enough cortisol and, in some cases, aldosterone.

  • Primary adrenal insufficiency (Addison disease) usually causes:

    • Destruction of all regions of adrenal cortex.

    • Deficiencies of cortisol, aldosterone, and androgens.

    • Compensatory increases in corticotropin-releasing hormone (CRH) and adrenocorticotropic hormone (ACTH)

  • Autoimmune dysfunction causes 80–90% of cases in developed countries; tuberculosis is leading cause in developing countries.

  • Medications that inhibit cortisol synthesis (eg, ketoconazole) or accelerate cortisol metabolism (eg, phenytoin, rifampin, phenobarbital) can also cause primary adrenal insufficiency.

  • Secondary adrenal insufficiency usually results from exogenous corticosteroid use, leading to suppression of hypothalamic-pituitary-adrenal axis and decreased ACTH release, resulting in impaired androgen and cortisol production.

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EPIDEMIOLOGY

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  • Incidence approximately 1 in 100,000

  • Can affect persons of any age, gender, or ethnicity but typically presents in adults between ages 30 and 50 years.

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CLINICAL PRESENTATION

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SIGNS AND SYMPTOMS
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  • Weight loss, weakness, craving for salt, headaches, memory impairment, depression, postural dizziness, and GI symptoms.

  • Early symptoms of acute adrenal insufficiency also include myalgias, malaise, and anorexia, which can eventually lead to vomiting, fever, and shock.

  • Hyperpigmentation may involve exposed and nonexposed body parts.

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DIAGNOSIS

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LABORATORY TESTS
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  • Using the short cosyntropin stimulation test, an increase in cortisol level to 18 mcg/dL (500 nmol/L) or more rules out adrenal insufficiency.

  • Patients with Addison disease have an abnormal response to the short cosyntropin stimulation test. Plasma ACTH levels are usually 400–2000 pg/mL in primary insufficiency and normal to low (5–50 pg/mL) in secondary insufficiency.

  • A normal cosyntropin-stimulation test does not rule out secondary adrenal insufficiency.

  • Insulin hypoglycemia test, metyrapone test, and CRH stimulation test are sometimes performed.

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DESIRED OUTCOMES

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  • Limit morbidity and mortality.

  • Return patient to normal function.

  • Prevent episodes of acute adrenal insufficiency.

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TREATMENT: NONPHARMACOLOGIC THERAPY

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  • Inform patients of expected outcome, treatment complications, proper medication use and adherence, and potential side effects.

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TREATMENT: PHARMACOLOGIC THERAPY FOR ADRENAL INSUFFICIENCY

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  • Corticosteroids (Table 1)

    • Use hydrocortisone, cortisone, or prednisone at the lowest effective dose twice daily to mimic normal diurnal adrenal rhythm of cortisol production.

    • Starting total daily doses: hydrocortisone 15–25 ...

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