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Adrenal Insufficiency

What is it?

Adrenal insufficiency is a relatively rare disorder caused either by destruction of tissue in the portion of the adrenal glands known as the cortex, which normally secretes the corticosteroid hormones (Addison's disease), or by atrophy of the adrenal cortex resulting from a loss of stimulation from the pituitary gland (secondary adrenal failure). Addison's disease produces symptoms only after some 90 percent of the adrenal cortices have been destroyed. Insufficiency of corticosteroids can lead to a number of health problems, including an inability to recover from even a minor infection. Complications such as protracted weakness, shock, or death may result.

Adrenal insufficiency may occur at any age and affects both sexes equally. Once invariably fatal, the disorder is now highly treatable since the advent of corticosteroid replacement therapy in the 1950s; thus, a patient's outlook is quite favorable. Steps must be taken, however, to avert adrenal crisis—a sudden, life-threatening steroid deficiency usually brought on by infection, injury, or stress—which requires immediate emergency treatment.

What Causes It?

  • Addison's disease results from the destruction of the adrenal cortices. In most cases, no underlying cause can be pinpointed, although it often appears to involve an autoimmune disorder (an attack upon healthy tissue by the body's defenses against disease).
  • A family history of autoimmune disorders, such as hypothyroidism, is associated with a higher risk of Addison's disease.
  • Anticoagulant therapy with medications such as warfarin or heparin can occasionally result in adrenal hemorrhage, leading to destruction of adrenal tissue.
  • In a few cases Addison's disease may occur as a complication of certain infections, such as tuberculosis, histoplasmosis, or cytomegalovirus (in AIDS patients).
  • Adrenal tissue may be destroyed by metastatic cancer or other disorders (see Amyloidosis and Sarcoidosis for more information).
  • Secondary adrenal insufficiency most often results from a pituitary tumor that destroys a part of the pituitary gland or from surgical removal or radiation of the pituitary.
  • Long-term treatment with steroids leads to atrophy of the adrenal cortex; recovery of adrenal function may take months after steroid treatment is stopped.

Prevention

  • There is no way to prevent Addison's disease.

Diagnosis

  • Patient history and physical evaluation.
  • Blood and urine tests are taken to measure hormone levels.

How to Treat It

  • Lifelong hormone replacement is the key to treatment. Corticosteroids such as cortisol, prednisone, and dexamethasone are prescribed. Because the drugs promote increased secretion of stomach acid, doses should be taken with meals, milk, or antacids.
  • A mineralocorticoid such as fludrocortisone is often needed to help control salt and mineral balance and thus prevent dehydration and hypotension (unusually low blood pressure) in those with Addison's disease.
  • The dose of corticosteroids must be increased during stressful times, such as infections, gastrointestinal illness, dental extractions, or surgery.
  • Patients should wear or carry medical alert identification with them at all times.
  • Adrenal crisis requires emergency medical treatment, which includes intravenous fluid and hydrocortisone infusions.

When to Call a Doctor

  • See a doctor for any persistent unexplained weakness, weight loss, or stomach upset.
  • EMERGENCY If you have been diagnosed with Addison's disease and experience symptoms of severe lethargy, nausea, vomiting, dizziness, or dehydration, get immediate medical assistance. These may be indications of adrenal crisis.