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ESSENTIALS OF DIAGNOSIS

  • Deficiency of cortisol and mineralocorticoid from destruction of the adrenal cortex.

  • Weakness, vomiting, diarrhea; abdominal pain, arthralgias; amenorrhea.

  • Increased skin pigmentation, especially of creases, pressure areas, and nipples.

  • Hypovolemic hypotension, small heart.

  • Hyponatremia; hyperkalemia (may be absent with vomiting and diarrhea); hypoglycemia; eosinophilia.

  • Elevated plasma ACTH level; cosyntropin unable to stimulate serum cortisol to 20 mcg/dL (550 nmol/L) or more.

  • Acute adrenal crisis: above manifestations become critical, with fever, shock, confusion, coma, death.

GENERAL CONSIDERATIONS

Primary adrenal insufficiency (Addison disease) is caused by dysfunction or absence of the adrenal cortices. It is distinct from secondary adrenal insufficiency caused by deficient secretion of ACTH. (See Anterior Hypopituitarism.)

Addison disease is an uncommon disorder with a prevalence of about 90–140 cases per million and an annual incidence of about 5–6 cases per million in the United States. Addison disease refers to a chronic deficiency of cortisol caused by adrenocortical insufficiency; plasma ACTH and alpha-MSH levels are consequently elevated, causing pigmentation that ranges from none to strikingly dark. Patients with destruction of the adrenal cortices or with classic 21-hydroxylase deficiency also have mineralocorticoid deficiency, typically with hyponatremia, volume depletion, and hyperkalemia. In contrast, mineralocorticoid deficiency is not present in patients with familial corticosteroid deficiency, Allgrove syndrome, or secondary adrenal insufficiency.

Acute adrenal (Addisonian) crisis is an emergency caused by insufficient cortisol. Crisis may occur in the course of treatment of chronic adrenal insufficiency, or it may be the presenting manifestation of adrenal insufficiency. Acute adrenal crisis is more commonly seen in primary adrenal insufficiency than in secondary adrenal insufficiency. It is usually precipitated by one of the following: (1) Severe stress (eg, infection, trauma, surgery, hyperthyroidism, or prolonged fasting), or minor stress (vaccinations) in patients with latent or treated adrenal insufficiency; (2) Hyperthyroidism or prescription of thyroid hormone to patients with untreated adrenal insufficiency; (3) Nonadherence to glucocorticoid replacement or sudden withdrawal of adrenocortical hormone in patients with chronic primary or secondary adrenal insufficiency (eFigure 26–23); (4) Bilateral adrenalectomy or removal of a functioning adrenal tumor that had suppressed the other adrenal gland; (5) Sudden destruction of the pituitary gland (pituitary necrosis) or damage to both adrenals (by trauma, hemorrhage, anticoagulant therapy, thrombosis, infection or, rarely, metastatic carcinoma); (6) Administration of intravenous etomidate (used for rapid anesthesia induction or intubation).

eFigure 26–23.

Pattern of plasma ACTH and cortisol values in patients recovering from prior long-term daily treatment with large doses of corticosteroids. (Courtesy of R Ney. Reproduced, with permission, from Ganong WF. Review of Medical Physiology, 19th ed. Copyright © 1999 by The McGraw-Hill Companies, Inc.)

ETIOLOGY

Autoimmunity is the most common cause of Addison disease in industrialized countries, accounting for about 90% of spontaneous cases; adrenal function decreases over several years as it progresses to overt adrenal insufficiency. ...

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