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Addison disease

Definition

Addison disease is a disorder that occurs when the adrenal glands do not produce enough hormones.

Alternative Names

Adrenocortical hypofunction; Chronic adrenocortical insufficiency; Primary adrenal insufficiency

Causes

The adrenal glands are small hormone-releasing organs located on top of each kidney. They are made up of an outer portion, called the cortex, and an inner portion, called the medulla.

The cortex produces 3 hormones:

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

Addison disease results from damage to the adrenal cortex. The damage causes the cortex to produce hormone levels that are too low.

This damage may be caused by the following:

  • The immune system mistakenly attacking the adrenal glands (autoimmune disease)
  • Infections such as tuberculosis, HIV, or fungal infections
  • Hemorrhage into the adrenal glands
  • Tumors

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

Certain rare genetic defects may also cause adrenal insufficiency.

Symptoms

Symptoms of Addison disease include:

  • Chronic diarrhea, nausea, and vomiting
  • Darkening of the skin in some places
  • Dehydration
  • Dizziness when standing up
  • Paleness
  • Extreme weakness, fatigue, and slow, sluggish movement
  • Mouth lesions on the inside of a cheek (buccal mucosa)
  • Salt craving
  • Weight loss with reduced appetite

Exams and Tests

Laboratory tests may show:

Additional laboratory tests may be ordered.

Other tests may include:

Treatment

Treatment with replacement corticosteroids will control the symptoms of this disease. These medicines usually need to be taken for life.

Never skip doses of your medicine for this condition because life-threatening reactions may occur.

Your health care provider may increase your dosage for a short time because of:

  • Infection
  • Injury
  • Stress
  • Surgery

During an extreme form of adrenal insufficiency, called adrenal crisis, you must inject hydrocortisone right away. Treatment for low blood pressure is usually needed as well.

Some people with Addison disease are taught to give themselves an emergency injection of hydrocortisone during stressful situations. Always carry medical ID (card, bracelet, or necklace) that says you have adrenal insufficiency. The ID should also say the type of medicine and dosage you need in case of an emergency.

Outlook (Prognosis)

With hormone therapy, many people with Addison disease are able to lead a nearly normal life.

Possible Complications

Complications can occur if you take too little or too much adrenal hormone.

Complications also may result from the following related illnesses:

When to Contact a Medical Professional

Call your provider if:

  • You are unable to keep your medicine down due to vomiting.
  • You have stress such as infection, injury, trauma, or dehydration. You may need to have your medicine adjusted.
  • Your weight increases over time.
  • Your ankles begin to swell.
  • You develop new symptoms.
  • On treatment, you develop signs of a disorder called Cushing syndrome

If you have symptoms of adrenal crisis, give yourself an emergency injection of your prescribed medicine. If it is not available, go to the nearest emergency room or call 911.

Symptoms of adrenal crisis include:

  • Abdominal pain
  • Difficulty breathing
  • Dizziness or lightheadedness
  • Low blood pressure
  • Reduced level of consciousness

References

Barthel A, Willenberg HS, Gruber M, Bornstein SR. Adrenal insufficiency. In: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Adult and Pediatric. 7th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 102.

Napier C, Pearce SH. Current and emerging therapies for Addison's disease. Curr Opin Endocrinol Diabetes Obes. 2014;21(3):147-153. PMID: 24755997 www.ncbi.nlm.nih.gov/pubmed/24755997.

Nieman LK. Adrenal cortex. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 227.

Review Date:10/28/2015
Reviewed By:Brent Wisse, MD, Associate Professor of Medicine, Division of Metabolism, Endocrinology & Nutrition, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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