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Addison’s disease

Definition

Addison's disease is a hormone deficiency (not enough hormone) caused by damage to the outer layer of the adrenal gland (the part known as the adrenal cortex).

Alternative Names

Adrenocortical hypofunction; Chronic adrenocortical insufficiency; Primary adrenal insufficiency

Causes, incidence, and risk factors

The adrenal glands are small hormone-secreting organs 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 3 types of hormones: glucocorticoid hormones, mineralocorticoid hormones, and sex hormones.

• The glucocorticoid hormones (such as cortisol) maintain glucose (sugar) control, suppress (decrease) immune response, and help the body respond to stress.
• The mineralocorticoid hormones (such as aldosterone) regulate sodium and potassium balance.
• The sex hormones, androgens (male) and estrogens (female) affect sexual development and reproduction.

Addison's disease results from damage to the adrenal cortex. The damage causes decreased production of the hormones produced by the cortex. This damage may be caused by the following:

• The immune system mistakenly attacking the gland (autoimmune disease)
• Infections such as tuberculosis, HIV, or fungal infections
• Hemorrhage, blood loss
• Tumors
• Use of blood-thinning drugs (anticoagulants)

Risk factors for the autoimmune type of Addison's disease include other autoimmune diseases:

• Type I diabetes
• Hypoparathyroidism
• Hypopituitarism
• Pernicious anemia
• Testicular dysfunction
• Graves' disease
• Chronic thyroiditis
• Dermatis herpetiformis
• Vitiligo
• Myasthenia gravis

These may be caused by certain genetic defects.

Symptoms

• Extreme weakness
• Fatigue
• Unintentional weight loss
• Nausea and vomiting
• Chronic diarrhea
• Loss of appetite
• Darkening of the skin - patchy skin color
  • Unnaturally dark color in some locations
  • Paleness may also occur
• Mouth lesions on the inside of a cheek (buccal mucosa) - pigmentation
• Slow, sluggish movement
• Changes in 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.
• Sex hormone levels will probably remain normal, because these hormones are also made by the ovaries or testes (and in larger amounts).

This disease may also alter the results of the following tests:

• Renin
• Potassium test
• Cortisol, urine
• CO2
• Aldosterone
• ACTH
• 24 hour urinary aldosterone excretion rate
• 17-ketosteroids
• 17-hydroxycorticosteroids
• Cortrosyn stimulation test (Cortrosyn is a man-made form of part of the ACTH hormone)
• Blood eosinophil count (a special white blood cell)

Treatment

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

Times of stress, infection, or injury may require increased doses of medications.

Adrenal crisis is an extreme form of symptoms of adrenal insufficiency, brought on by physical stress. Hydrocortisone must be injected immediately to sustain life. Supportive treatment for low blood pressure is usually necessary as well.

Some people with Addison's disease are taught to give themselves an emergency injection of hydrocortisone during stressful situations. 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 skip doses of medication for this condition, as life-threatening reactions may occur. If you are unable to keep the medication down due to vomiting, notify your health care provider, go to the emergency room, or call the local emergency number (such as 911) immediately.

Also report sudden weight gain or fluid retention to your health care provider.

Expectations (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 (chronic thyroiditis)
• Hypoparathyroidism
• Pernicious anemia
• Ovarian hypofunction or testicular failure

Calling your health care provider

Call your health care provider if you have been diagnosed with Addison's disease, and stress such as infection, injury, trauma, or dehydration occurs. Medication adjustments may be needed.

Call your provider if your weight increases over time, your ankles begin to swell, or other new symptoms develop.

If symptoms of adrenal crisis (low blood pressure, diminished consciousness, difficulty breathing, abdominal pain) occur, give yourself an emergency injection of your prescribed medication as instructed or -- if this is not available -- go to the nearest emergency room or call 911.

Review Date:8/8/2006
Reviewed By:Robert Hurd, MD, Department of Biology, College of Arts and Sciences, Xavier University, Cincinnati, OH. Review provided by VeriMed Healthcare Network

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