Addison disease
Adrenocortical hypofunction; Chronic adrenocortical insufficiency; Primary adrenal insufficiency
Addison disease is a disorder that occurs when the adrenal glands do not produce enough hormones.
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.
Adrenal glands
The adrenal glands are two triangle-shaped glands. One gland is located on top of each kidney.
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.
Immune response
The immune response is how your body recognizes and defends itself against bacteria, viruses, and substances that appear foreign and harmful....
- 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
Tuberculosis
Pulmonary tuberculosis (TB) is a contagious bacterial infection that involves the lungs. It may spread to other organs.
- Hemorrhage into the adrenal glands
- Tumors
Risk factors for the autoimmune type of Addison disease include other autoimmune diseases :
Autoimmune diseases
An autoimmune disorder occurs when the body's immune system attacks and destroys healthy body tissue by mistake. There are more than 80 types of aut...
-
Swelling (inflammation) of the thyroid gland that often results in reduced thyroid function (
chronic thyroiditis
)
Chronic thyroiditis
Chronic thyroiditis is caused by a reaction of the immune system against the thyroid gland. It often results in reduced thyroid function (hypothyroi...
-
Thyroid gland produces too much thyroid hormone (
overactive thyroid
)
Overactive thyroid
Hyperthyroidism is a condition in which the thyroid gland makes too much thyroid hormone. The condition is often called overactive thyroid.
-
Itchy rash with bumps and blisters (
dermatitis herpetiformis
)
Dermatitis herpetiformis
Dermatitis herpetiformis is an extremely itchy rash consisting of bumps and blisters. The rash is chronic (long-term), which means it continues over...
-
Autoimmune disorder that leads to overactivity of the thyroid gland (
Graves disease
)
Graves disease
Graves disease is an autoimmune disorder that leads to an overactive thyroid gland (hyperthyroidism). An autoimmune disorder is a condition that occ...
-
Parathyroid glands in the neck do not produce enough parathyroid hormone (
hypoparathyroidism
)
Hypoparathyroidism
Hypoparathyroidism is a disorder in which the parathyroid glands in the neck do not produce enough parathyroid hormone (PTH).
-
Pituitary gland does not produce normal amounts of some or all of its hormones (
hypopituitarism
)
Hypopituitarism
Hypopituitarism is a condition in which the pituitary gland does not produce normal amounts of some or all of its hormones.
-
Autoimmune disorder that affects the nerves and the muscles they control (
myasthenia gravis
)
Myasthenia gravis
Myasthenia gravis is a neuromuscular disorder. Neuromuscular disorders involve the muscles and the nerves that control them.
-
Body does not have enough healthy red blood cells (
pernicious anemia
)
Pernicious anemia
Anemia is a condition in which the body does not have enough healthy red blood cells. Red blood cells provide oxygen to body tissues. There are man...
-
Testicles cannot produce sperm or male hormones (
testicular failure
)
Testicular failure
Testicular failure occurs when the testicles cannot produce sperm or male hormones.
-
Type I diabetes
Type I diabetes
Type 1 diabetes is a lifelong (chronic) disease in which there is a high level of sugar (glucose) in the blood.
-
Loss of brown color (pigment) from areas of the skin (
vitiligo
)
Vitiligo
Vitiligo is a skin condition in which there is a loss of color (pigment) from areas of skin. This results in uneven white patches that have no pigme...
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
Dehydration
Dehydration occurs when your body does not have as much water and fluids as it should. Dehydration can be mild, moderate, or severe, based on how muc...
- Dizziness when standing up
- Paleness
-
Extreme
weakness
,
fatigue
, and slow, sluggish movement
Fatigue
Fatigue is a feeling of weariness, tiredness, or lack of energy.
-
Mouth lesions
on the inside of a cheek (buccal
mucosa
)
Mouth lesions
There are different types of mouth sores. They can occur anywhere in the mouth including bottom of the mouth, inner cheeks, gums, lips, and tongue....
Mucosa
Mucosa is moist tissue that lines certain parts of the inside of your body. It is in your: NoseMouthLungsUrinary and digestive tracts Glands in this...
- Salt craving
- Weight loss with reduced appetite
Exams and Tests
Laboratory tests may show:
-
Increased
potassium
Potassium
This test measures the amount of potassium in the fluid portion (serum) of the blood. Potassium (K+) helps nerves and muscles communicate. It also ...
- Low blood pressure, especially with a change in body position
-
Low
cortisol level
Cortisol level
The cortisol blood test measures the level of cortisol in the blood. Cortisol is a steroid (glucocorticoid) hormone produced by the adrenal gland. C...
-
Low
serum sodium
Serum sodium
The sodium blood test measures the amount of sodium in the blood. Sodium can also be measured using a urine test.
- Low blood pH
- Normal sex hormone levels
-
High
eosinophil count
in the blood
Eosinophil count
An absolute eosinophil count is a blood test that measures the number of white blood cells called eosinophils. Eosinophils become active when you ha...
Additional laboratory tests may be ordered.
Other tests may include:
-
Abdominal x-ray
Abdominal x-ray
An abdominal x-ray is an imaging test to look at organs and structures in the abdomen. Organs include the spleen, stomach, and intestines. When the ...
-
Abdominal CT scan
Abdominal CT scan
An abdominal CT scan is an imaging method. This test uses x-rays to create cross-sectional pictures of the belly area. CT stands for computed tomog...
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.
Adrenal crisis
Acute adrenal crisis is a life-threatening condition that occurs when there is not enough cortisol. This is a hormone produced by the adrenal glands...
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:
-
Diabetes
Diabetes
Diabetes is a chronic disease in which the body cannot regulate the amount of sugar in the blood.
- Chronic thyroiditis
- Hypoparathyroidism
-
Ovarian hypofunction
or testicular failure
Ovarian hypofunction
Ovarian hypofunction is reduced function of the ovaries (including decreased production of hormones).
- Pernicious anemia
- Overactive thyroid
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
Cushing syndrome
Cushing syndrome is a disorder that occurs when your body has a high level of the hormone cortisol.
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
Low blood pressure
Low blood pressure occurs when blood pressure is much lower than normal. This means the heart, brain, and other parts of the body do not get enough ...
- 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.
-
Endocrine glands - illustration
Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
Endocrine glands
illustration
-
Endocrine glands - illustration
Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the pace of chemical activity in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
Endocrine glands
illustration
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.