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What is Addison’s Disease
Addison’s disease is a disorder that occurs when your body produces insufficient amounts of certain hormones produced by your adrenal glands. In Addison’s disease, your adrenal glands produce too little cortisol and often insufficient levels of aldosterone as well.
Also called adrenal insufficiency, Addison’s disease occurs in all age groups and affects both sexes. Addison’s disease can be life-threatening.
Treatment for Addison’s disease involves taking hormones to replace the insufficient amounts being made by your adrenal glands, in order to mimic the beneficial effects produced by your naturally made hormones.
Causes Addison’s Disease
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 three 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:
- Chronic thyroiditis
- Dermatis herpetiformis
- Graves’ disease
- Hypoparathyroidism
- Hypopituitarism
- Myasthenia gravis
- Pernicious anemia
- Testicular dysfunction
- Type I diabetes
- Vitiligo
Certain rare genetic defects may also cause adrenal insufficiency.
Symptoms of Addison’s Disease
People who have Addison’s disease may experience the following symptoms:
- weakness in the muscles
- fatigue and tiredness
- darkening in skin color
- weight loss or decreased appetite
- a decrease in heart rate or blood pressure
- low blood sugar levels
- fainting spells
- sores in the mouth
- cravings for salt
- nausea
- vomiting
- irritability or depression
If Addison’s disease goes untreated for too long, it can become an Addisonian crisis. An Addisonian crisis is a life threatening medical emergency. Call 911 immediately if you or someone you know begins to experience:
- mental status changes (confusion, fear, or restlessness)
- loss of consciousness
- high fever
- sudden pain in the lower back, belly, or legs
An untreated Addisonian crisis can lead to shock and death.
Diagnosis of Addison’s disease
Diagnosis may involve:
- a complete detailed family history, with special attention to any other endocrine disorders
- biochemical tests, which measure cortisol levels before and after a challenge injection of synthetic ACTH, known as a ‘short synacthen test’. Synacthen tests will indicate the person’s baseline level of cortisol production and their response to an increased need for cortisol in the body. An person who suffers from Addison’s may show a flat or reduced response
- blood electrolyte and plasma renin tests, which will indicate if there is a need for mineralocorticoid replacement
- anti-adrenal antibody test if the result is positive, primary Addison’s disease is definitively diagnosed. However, even if these antibodies do not exist, the person may still have Addison’s disease
- x-rays, ultrasounds and CAT scans of the abdominal region to check for visual signs of damage and the size of adrenal glands.
How is Addison’s Disease Treated?
Treatment of Addison’s disease involves replacing, or substituting, the hormones that the adrenal glands are not making. Cortisol is replaced orally with hydrocortisone tablets, a synthetic glucocorticoid, taken once or twice a day. If aldosterone is also deficient, it is replaced with oral doses of a mineralocorticoid called fludrocortisone acetate (Florinef), which is taken once a day. Patients receiving aldosterone replacement therapy are usually advised by a doctor to increase their salt intake. Because patients with secondary adrenal insufficiency normally maintain aldosterone production, they do not require aldosterone replacement therapy. The doses of each of these medications are adjusted to meet the needs of individual patients.
During an addisonian crisis, low blood pressure, low blood glucose, and high levels of potassium can be life threatening. Standard therapy involves intravenous injections of hydrocortisone, saline (salt water), and dextrose (sugar). This treatment usually brings rapid improvement. When the patient can take fluids and medications by mouth, the amount of hydrocortisone is decreased until a maintenance dose is achieved. If aldosterone is deficient, maintenance therapy also includes oral doses of fludrocortisone acetate.
Surgery
Patients with chronic adrenal insufficiency who need surgery with general anesthesia are treated with injections of hydrocortisone and saline. Injections begin on the evening before surgery and continue until the patient is fully awake and able to take medication by mouth. The dosage is adjusted until the maintenance dosage given before surgery is reached.
Pregnancy
Women with primary adrenal insufficiency who become pregnant are treated with standard replacement therapy. If nausea and vomiting in early pregnancy interfere with oral medication, injections of the hormone may be necessary. During delivery, treatment is similar to that of patients needing surgery; following delivery, the dose is gradually tapered and the usual maintenance doses of hydrocortisone and fludrocortisone acetate by mouth are reached by about 10 days after childbirth.
