Alternate Names: Adrenocortical hypofunction; Chronic adrenocortical insufficiency; Adrenal insufficiency

Causes and Risks:

The adrenal glands are located on top of each kidney. They consist of the outer portion (called the cortex), and the inner portion (called the medulla). The cortex produces three types of hormones: androgens and estrogens, glucocorticoid hormones, and mineralocorticoid hormones.

The androgens and estrogens affect sexual development and reproduction. The glucocorticoid hormones (such as cortisol) maintain glucose regulation, suppress the immune response, and provide for the response to stress. The mineralocorticoid hormones (such as aldosterone) regulate sodium and potassium balance.

Risk factors include rare familial autoimmune diseases and other diseases. These include: premature cessation of menstruation, type I diabetes, hypoparathyroidism, hypopituitarism, pernicious anemia, testicular dysfunction, Graves' disease, chronic thyroiditis, candidiasis, dermatis herpetiformis, vitiligo and myasthenia gravis.

Other risk factors are anticoagulant use and AIDS.

Symptoms:

• Weakness - extreme
• Fatigue - muscle weakness
• Unintentional weight loss
• Nausea
• Vomiting
• Chronic diarrhea
• Loss of appetite
• Darkening of the skin - skin color, patchy
  • Color is unnaturally dark in some locations
  • Paleness may also occur
• Mouth lesions on the inside of a cheek (buccal mucosa) - pigmentation
• Slow, sluggish, lethargic movement
• Changes in the blood pressure or heart rate
• Salt craving

Signs and Tests:

• Blood pressure is low.
• Cortisol level is low.
• Serum sodium is low.
• A potassium test may show increased potassium.
• An abdominal x-ray may show adrenal calcification.
• An abdominal CT scan may show adrenal calcification, enlargement or atrophy.

• Renin
• Potassium test
• Cortisol, urine
• CO2
• Aldosterone
• ACTH
• 24 hour urinary aldosterone excretion rate
• 17-ketosteroids
• 17-hydroxycorticosteroids
• Cortrosyn stimulation test
• DHEAS

Treatment:

Replacement therapy with corticosteroids will control the symptoms of this disease. However, these drugs must usually be continued for life. Usually a combination of glucocorticoids (cortisone or hydrocortisone) and mineralocorticoids (fludrocortisone) are given.

Medication may need to be increased during times of stress, infection, or injury.

In adrenal crisis, an intravenous or intramuscular injection of hydrocortisone must be given immediately. Supportive treatment for low blood pressure is usually necessary.

Some people with Addison’s disease are taught to give themselves an emergency injection of hydrocortisone in times of stress. It is important for the individual with Addison’s disease to always carry a medical identification card that states the type of medication and the proper dose needed in case of an emergency.

Never omit medication. If unable to retain medication due to vomiting, notify the health care provider. Also report sudden weight gain or fluid retention to the health care provider.

Prognosis: With adequate replacement therapy, most people with Addison’s disease are able to lead normal lives.

Complications: Complications may result from the following associated illnesses:

• Diabetes
• Thyrotoxicosis
• Hashimoto’s thyroiditis
• Hypoparathyroidism
• Pernicious anemia
• Ovarian hypofunction or testicular failure

Call your health care provider if Addison’s disease has been diagnosed and stress such as infection, injury, or dehydrating illness develops (medication adjustment may be indicated).

Call for an appointment with your health care provider if weight increases progressively, the ankles begin to swell, or other new symptoms develop.