Addison's Disease

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Addison's Disease

Topic Overview

What is Addison's disease?

Addison's disease develops when the adrenal glands, which are above the kidneys, are not able to make enough of the hormones cortisol and, sometimes, aldosterone.

Cortisol affects almost every organ in the body and is needed for your body to work as it should. The adrenal glands release cortisol to help the body cope with stress from illness, injury, surgery, childbirth, or other reasons. Aldosterone helps the body hold on to the salt it needs, and it keeps your blood pressure steady.

Normally, cortisol levels increase through a chain reaction of hormones:

  1. First, the hypothalamus in the brain makes a hormone called CRH (corticotropin-releasing hormone).
  2. Then, CRH tells the pituitary gland to make another hormone called ACTH (adrenocorticotropic hormone).
  3. ACTH then tells the adrenal glands to make cortisol. Cortisol affects almost every area of the body. It is especially important in regulating blood pressure and metabolism.

When your adrenal gland cannot make enough cortisol, you have Addison’s disease.

What causes Addison's disease?

Addison's disease most often occurs when the body's immune system kills off the part of the adrenal glands that makes cortisol and aldosterone. It can also form when the adrenal glands are harmed by:

  • Infections, such as tuberculosis, HIV, and other bacterial or fungal infections.
  • Cancer that has spread to the adrenal glands. This is mostly seen in lung cancer.
  • Bleeding into the adrenal glands as a side effect of using blood thinners.
  • Some types of surgery or radiation treatments.
  • The use of certain medicines, such as high doses of ketoconazole.
  • Injury to the adrenal glands.

People can get Addison's disease at any age. Addison’s disease can also form if you take a steroid medicine for a long time and then suddenly stop using it.

What are the symptoms?

The most common symptoms are:

  • Extreme tiredness (fatigue).
  • Weakness.
  • Losing weight without trying.

You may also have other symptoms, such as:

  • Skin that looks darker than normal.
  • Loss of appetite.
  • Diarrhea.
  • Feeling light-headed.
  • Feeling sick to your stomach or vomiting.
  • Craving salt.

And if you have diabetes, you may have low blood sugar more often, and it may be more severe than usual.

Symptoms usually start slowly. You may not even notice them until a stressful event such as a severe infection, trauma, surgery, or dehydration causes an adrenal crisis. An adrenal crisis means that your body can't make enough cortisol to cope with the stress.

In a few cases, Addison's disease gets worse quickly. These people may already be in an adrenal crisis when they see a doctor.

Symptoms of an adrenal crisis include:

  • Severe vomiting and diarrhea.
  • Sudden pain in the belly, low back, or legs.
  • A high fever.
  • Feeling very weak or light-headed.
  • Feeling restless, confused, or fearful.
  • Having trouble staying awake.

Call your doctor right away if you have these symptoms. If an adrenal crisis is not treated, you could die of shock from a steep drop in blood pressure.

How is Addison's disease diagnosed?

To diagnose Addison’s disease, the doctor will:

  • Ask you questions about your symptoms and past health. He or she will want to know if this disease runs in your family and if you have had cancer or tuberculosis or have been infected with HIV.
  • Do a physical examination. The doctor will check to see if your skin colour is darker than normal, if you have low blood pressure, or if you are dehydrated.
  • Order lab tests. Blood tests can show if you have high potassium or low sodium levels. These levels can be a sign of Addison’s disease. You may also have a blood test to check your levels of cortisol and ACTH.

If the diagnosis is still not clear, you may have an ACTH stimulation test, which helps show how your hormone levels react to stress. You will be given a man-made form of ACTH to see if your body makes enough cortisol. Further tests will show if you have Addison’s disease or another problem.

Your doctor may suggest some imaging tests, such as an MRI, after the problem area has been found.

If your doctor thinks you have Addison’s disease, he or she may start treatment right away, even before you get your test results. If the test results later show that you don't have the disease, your doctor can stop the treatment.

How is it treated?

If you have Addison's disease, you will need to take medicine for the rest of your life to replace the cortisol and aldosterone your body can't make on its own. You may take just one medicine, or you may need more than one. You may need to increase your dose during times of stress or illness. And you may need to add extra salt to your food during hot and humid weather to replace salt lost in your sweat.

If you have an adrenal crisis, you will need treatment in a hospital.

How do you manage life with Addison’s disease?

Finding out that you have Addison’s disease can be scary. But if you get treatment and follow your doctor’s advice, you can lead a long and healthy life. Here are some things you can do at home to help you manage the disease:

  • Take your medicine exactly as your doctor tells you to.
  • Don't restrict salt in your diet.
  • If you have an adrenal crisis, get help right away. Have a shot of emergency medicine ready at your home, work, or school, and in the car. Know when and how to give the medicine. Have instructions written out, and teach someone else how to give you the medicine in case you can't give it to yourself.
  • Wear a medical ID tag (such as a medical alert bracelet). That way, health professionals know to give you a shot of cortisol if you are injured or ill and cannot speak for yourself.
  • Be prepared to deal with stress or illness. Talk with your doctor about when you might need to increase the dose of your medicine during minor illness or times of stress. Call your doctor right away if you have signs of a severe infection.

Frequently Asked Questions

Learning about Addison's disease:

Being diagnosed:

Getting treatment:

Symptoms

Addison's disease develops when the adrenal glands, located above the kidneys, are not able to produce enough of the hormones cortisol and aldosterone. In most cases, the adrenal glands fail slowly and symptoms begin gradually. But symptoms may appear rapidly if the adrenal glands are destroyed suddenly, such as from trauma or severe bleeding (hemorrhage). The most obvious and common symptoms of Addison's disease include:

  • Fatigue and muscle weakness that gradually get worse over time.
  • Weight loss. Profound weight loss is a common symptom.
  • Loss of appetite.

Other symptoms include:

  • Darkening of the skin, or hyperpigmentation. This occurs especially:
    • Over new scars.
    • In skin folds.
    • On the lips and tissue lining the mouth and nose.
    • Over joints such as the elbows, knees, knuckles, and toes.
    • In the creases of the palms.
    • On the nipples and surrounding areas (areola).
  • Light-headedness or fainting when getting up from a sitting or lying position. This is called orthostatic hypotension.
  • Nausea, vomiting, diarrhea, and belly pain.
  • A craving for salty foods.
  • Shakiness. People with Addison's disease sometimes have low blood sugar.
  • Difficulty concentrating, increased irritability, and depression.

Because symptoms develop slowly, they often are not obvious until an adrenal crisis (Addisonian crisis) occurs. An adrenal crisis is caused by a stressful event, such as a severe infection, trauma, surgery, or dehydration. The body is not able to produce enough cortisol to cope with stress. If an adrenal crisis is not treated, you may die from shock caused by a serious drop in blood pressure. Symptoms of an adrenal crisis include:

  • Severe vomiting and diarrhea that lead to a dangerous loss of body fluids (dehydration).
  • Extreme weakness, light-headedness, or feeling that you are going to pass out.
  • Sudden pain in the belly, lower back, and legs.
  • Abnormal behaviour, such as restlessness, confusion, or feeling fearful.
  • Changes in level of consciousness, such as having difficulty staying awake, or a total loss of consciousness.
  • High fever.
  • Pale face and blue lips and earlobes.

A wide range of illnesses—many of them not serious—can have symptoms similar to those of Addison's disease, although they are usually not as severe. Other conditions with similar symptoms range from diarrhea and other gastrointestinal problems to more serious illnesses such as hepatitis or cancer.

Examinations and Tests

Addison's disease develops when the adrenal glands, located above the kidneys, are not able to produce enough of the hormones cortisol and aldosterone. Your doctor may suspect Addison's disease from your medical history and physical examination and when blood tests show high potassium, low sodium, and high levels of certain types of white blood cells.

If your doctor suspects Addison's disease, you will have a blood test to determine your cortisol level.

Low blood cortisol levels do not always indicate Addison's disease. During a full day, cortisol levels are highest in the morning, drop during the afternoon, and are lowest overnight. Cortisol levels that are normal at one time of the day may be low at other times of the day. But the level of cortisol should be high when the body is physically or emotionally stressed. The diagnosis of Addison's disease is usually confirmed by doing an ACTH stimulation test, which helps show how your hormone levels react to stress. If your test results show you have high cortisol levels when checked at random or after the ACTH stimulation test, then you do not have adrenocortical disease.

  • Low cortisol blood levels that fail to increase after an injection of ACTH indicate adrenal insufficiency.
  • The levels of ACTH in the blood before an injection of ACTH indicate whether you have Addison's disease or secondary adrenocortical insufficiency, in which an inadequate amount of ACTH leads to low cortisol production. This may be caused by a problem with the pituitary gland, which controls hormone production.

X-rays, including CT scan or MRI, can also be used to check for damage to the adrenal glands.

If Addison's disease is diagnosed, your doctor will also look for thyroid problems, such as hyperthyroidism, hypothyroidism, and low production of parathyroid hormone, which causes a low calcium level in the blood. Your doctor will also look for other problems such as type 2 diabetes, pernicious anemia, and failure of the reproductive glands (testes and ovaries). One or more of these conditions are present in about one-half of those with Addison's disease. It is especially important for people with Addison's disease to know if they also have thyroid disease. In people with low thyroid, cortisol lasts longer in the blood. Correcting a low level of thyroid hormone can lead to low cortisol levels and an adrenal crisis.

You may need to see an endocrinologist if the diagnosis or treatment of Addison's disease is uncertain.

Treatment Overview

Addison's disease develops when the adrenal glands, located above the kidneys, are not able to produce enough of the hormones cortisol and aldosterone. Treatment is usually lifelong. After proper treatment is started, most people with Addison's disease can lead healthy lives.

Treatment includes:

  • Medicines to replace the hormones cortisol and aldosterone. Many doctors use a short-acting medicine called hydrocortisone, given 2 or 3 times a day. In some people, long-acting medicines such as prednisone, methylprednisolone, or dexamethasone can be used. You may also need a medicine that can function like aldosterone (usually fludrocortisone). Hydrocortisone is used because it can function like both of these hormones, although some people still need to take additional fludrocortisone. You will need a higher dose of medicine during childbirth; when you have an injury, surgery, or a serious infection; or during severe stress, such as the death of a loved one.
  • Increasing salt in the diet. Because people with adrenal failure tend to lose sodium, you will need extra salt (sodium chloride) in your diet, especially during hot and humid weather and vigorous exercise. But because people with Addison's disease retain potassium, you need to avoid salt substitutes, which usually contain potassium chloride salts.
  • Regular medical checkups to monitor symptoms and blood pressure. Your doctor also may need to do lab tests to evaluate and monitor blood levels of potassium and sodium.

During a stressful event, such as a severe infection, trauma, surgery, childbirth, dehydration, or an emotional event such as the death of a loved one, you could develop an adrenal crisis. Your body is not able to produce enough cortisol for the stressful condition. If an adrenal crisis is not treated, you may die from shock caused by a serious drop in blood pressure. You would need treatment in the hospital.

Recent small studies suggest that women with Addison's disease may benefit from taking a hormone replacement called dehydroepiandrosterone (DHEA). Some women who take this medicine every day may experience a greater sense of well-being and improvement in depression, anxiety, sexuality, and sensitivity to insulin.1 If you are considering DHEA, talk to your doctor.

Home Treatment

Addison's disease develops when the adrenal glands, located above the kidneys, are not able to produce enough of the hormones cortisol and aldosterone. Home treatment is very important. If you have Addison's disease:

  • Take your medicine daily as recommended. Medicine should be taken at the same times of day when your body would normally need the hormones (usually more in the morning and less in the evening). Let your doctor know how you are doing. For example, if your face is puffy or if you are gaining weight or bruising more easily or extensively than usual, the amount of your medicine may be too high. If you are still fatigued, have no appetite, or are feeling generally unwell, the amount of your medicine may be too low.
  • Don't restrict salt in your diet. Your body needs a certain amount of salt. With Addison's disease, your body may lose too much salt. You may need to add extra salt to your food during hot and humid weather or when you are exercising and sweating.
  • Seek medical help in an emergency. If you have symptoms of adrenal crisis, take your emergency medicine (such as dexamethasone) as directed and seek medical help immediately. An adrenal crisis is caused by a stressful event, such as a severe infection, trauma, surgery, or dehydration. Your body is not able to produce enough cortisol for the stressful condition. If this is not treated, you may die from shock caused by a serious drop in blood pressure. Have an injection of emergency medicine ready at your home, work or school, and in the car. Know when and how to give the medicine. Have instructions written out, and teach someone else how to give you the medicine in case you can't give it to yourself. Replace unused syringes of emergency medicine each year or if the solution begins to look cloudy or coloured.
  • Carry a medical identification card or bracelet (such as a medical alert bracelet) that states that you have Addison's disease. This information helps medical personnel decide whether to give you a cortisol shot if you are unconscious, severely injured, or unable to answer questions.
  • Monitor your weight regularly, especially if your appetite has been poor or you have been vomiting. Weigh yourself at the same time of day and wearing the same amount of clothing. Ask when your doctor wants to be notified about weight loss or frequent vomiting.
  • Monitor your blood pressure. High blood pressure and swelling may indicate that your medicine needs to be adjusted. Also, if you notice that you become light-headed when you first get up in the morning, your blood pressure may be low. Sit on the edge of your bed for a while before standing. Let your doctor know if this problem becomes worse.

In addition, you need to be prepared for and deal with minor illness or stress. During illness or stress, you may need to:

  • Increase your medicine dosage. Talk with your doctor about when and how much you should increase your medicine dosage during a minor illness or time of stress. Have clear instructions written out for which medicine should be increased and how much you should increase it.
  • Know when to contact your doctor if you have either stress or a minor infection that won't go away. If you notice signs of a severe infection, such as high fever, frequent cough, or burning with urination, call right away.
  • Contact your doctor right away if you are unable to take your cortisol medicine because of nausea or vomiting, or if you have ongoing diarrhea.

Other Places To Get Help

Organizations

Canadian Addison Society
193 Elgin Avenue West
Goderich, ON  N7A 2E7
Phone: 1-888-550-5582
Web Address: www.addisonsociety.ca
 

The Canadian Addison Society (CAS) provides basic information about Addison's disease and related conditions, resources for people who have AD and their families, and links to local chapters. The CAS also publishes a quarterly newsletter.


U.S. National Adrenal Disease Foundation
505 Northern Boulevard
Suite 200
Great Neck, NY  11021
Phone: (516) 487-4992
Email: nadfmail@aol.com
Web Address: www.nadf.us
 

The U.S. National Adrenal Diseases Foundation is a consumer-based organization providing information and support for people with adrenal-related diseases.


References

Citations

  1. Alkatib AA, et al. (2009). A systematic review and meta-analysis of randomized placebo-controlled trials of DHEA treatment effects on quality of life in women with adrenal insufficiency. Journal of Clinical Endocrinology and Metabolism, 94(10): 3676–3681.

Other Works Consulted

  • Aron DC, et al. (2007). Glucocorticoids and adrenal androgens. In DG Gardner, D Shobeck, eds., Greenspan's Basic and Clinical Endocrinology, 8th ed., pp. 367–378. New York: McGraw-Hill.
  • Loriaux DL (2009). Adrenal. In EG Nabel, ed., ACP Medicine, section 3, chap. 4. Hamilton, ON: BC Decker.
  • Malchoff C (2009). Adrenal insufficiency. In RE Rakel, ET Bope, eds., Conn's Current Therapy 2009, pp. 637–640. Philadelphia: Saunders Elsevier.
  • Miller M (2007). Selected endocrine problems. In LR Barker et al., eds., Principles of Ambulatory Medicine, 7th ed., pp. 1367–1394. Philadelphia: Lippincott Williams and Wilkins.
  • Stewart PM (2008). Glucocorticoid deficiency section of The adrenal cortex. In HM Kronenberg et al., eds., Williams Textbook of Endocrinology, 11th ed., pp. 445–485. Philadelphia: Saunders.

Credits

By Healthwise Staff
Primary Medical Reviewer E. Gregory Thompson, MD - Internal Medicine
Primary Medical Reviewer Anne C. Poinier, MD - Internal Medicine
Specialist Medical Reviewer David C.W. Lau, MD, PhD, FRCPC - Endocrinology
Last Revised April 15, 2010

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