Definition

Addison disease is a disorder of the adrenal glands. The adrenal glands sit on top of the kidneys and make important hormones. Hormones affect systems throughout the body including blood pressure, metabolism, and the immune system.

With Addison disease, the adrenal glands do not make enough of certain hormones. The hormone cortisol is always affected but aldosterone may be low as well. Addison disease may be:

• Primary Addison disease—adrenal gland tissue can not make hormones
• Secondary Addison disease—other hormones that tell adrenal glands what to do are missing or low

A severe complication of Addison's disease is the Addisonian or adrenal crisis. This is a life threatening crisis.

Adrenal Glands Addison's occurs because of damage to the cortex. Copyright © Nucleus Medical Media, Inc.

Causes    TOP

Primary Addison disease is caused by damage to the outer layer of the adrenal gland. This damage can happen slowly overtime for a number of reasons.

Addison disease is most often caused by an autoimmune disease when the immune system begins to attack healthy tissue. In this case, it attacks tissue of the adrenal gland. The cause of autoimmune diseases is unclear.

Damage may also be caused by infections such as:

• Tuberculosis
• Cytomegalovirus (CMV) infection
• Fungal infections

Less common causes include:

• Certain cancers, including those that have spread
• Amyloidosis—deposit of abnormal protein in tissue
• Bleeding within the adrenal glands—may happen because of anticoagulant medications or shock
• Surgical removal of glands
• Genetic factors or birth defects that make it difficult for the adrenal glands to work properly
• Certain medications

Secondary Addison disease can be caused by damage to the pituitary gland. The pituitary gland sends hormones that control the adrenal gland. If the pituitary gland is damaged it will affect the adrenal glands.

Adrenal insufficiency may also occur after long term corticosteroids are stopped.

Risk Factors    TOP

Factors that may increase your chance of Addison's disease include:

• Autoimmune disorders, such as:
  • Type I diabetes
  • Pernicious anemia
  • Hypoparathyroidism
  • Hypopituitarism
  • Hyperthyroidism or hypothyroidism
  • Myasthenia gravis
• Anticoagulant medications
• Abdominal injury
• Family members with autoimmune-caused Addison's disease
• Long-term steroid medication treatment, followed by:
• Previous surgery on adrenal glands
• Hereditary disorders, such as Prader-Willi syndrome and congenital adrenal hyperplasia

Symptoms    TOP

Symptoms of Addison disease may include:

• Extreme weakness, fatigue
• Weight loss
• Nausea or vomiting
• Chronic diarrhea
• Muscle weakness or pain
• Darkening of freckles, nipples, scars, skin creases, gums, mouth, nail beds, and vaginal lining
• Emotional changes, especially depression
• Cognitive impairment or confusion
• Craving salty foods
• Abdominal pain
• Lack of appetite
• Amenorrhea

Symptoms of an adrenal crisis include:

• High or low body temperature
• Severe abdominal, back, or leg pain
• Fainting
• Severe dehydration
• Severe nausea, vomiting, and diarrhea
• Bluish skin color
• Muscle weakness

Diagnosis    TOP

You will be asked about your symptoms and medical history. A physical exam will be done. If a problem with hormones is suspected your doctor may order:

• Blood tests and urine tests to measure the amount of cortisol
• ACTH stimulation test—should make the adrenal glands release cortisol
• CRH stimulation test—can help determine if it is primary or secondary Addison disease

Imaging tests may be needed to look at the adrenal glands. Tests may include:

• MRI scan
• CT scan

Treatment    TOP

Addison disease cannot be cured but can be managed with medication. These medications replace the missing hormones to decrease symptoms. They can also help prevent an adrenal crisis. An adrenal crisis will need immediate medical attention to try to balance the hormones again.

Regular blood tests are needed to monitor your response to medication. Wear a medical alert bracelet that states adrenal insufficiency or Addison disease. This will let others know of your condition if you are unable to communicate.

Cortisol helps the body deal with stress. Those with Addison disease need to take extra care during times of stress. Extra treatment may be needed during physical stress or recovery such as:

• Surgery
• Pregnancy
• Severe illnesses or injuries

Prevention    TOP

There are no current guidelines to prevent Addison disease.