Addison's Disease

What is it?
Addison's disease is a rare hormonal disorder involving disrupted functioning of the part of the adrenal gland called the cortex. It occurs when the adrenal glands, which are located just above the kidneys, do not produce enough of the hormone cortisol and in some cases, the hormone aldosterone. Cortisol is a very powerful hormone that effects nearly every organ and tissue throughout the body. It is thought to be one of the few hormones necessary for life. Helping the body respond to stress is one of its most important tasks. Cortisol is also involved in helping to maintain blood pressure and cardiovascular function; balancing the effects of insulin in breaking down sugar for energy; regulating the metabolism of proteins, carbohydrates, and fats; and slowing the immune system's inflammatory response. Aldosterone, also produced by the adrenal cortex, helps maintain a balance of water and salts in the body. When there is not enough aldosterone produced, the kidneys are not able to regulate salt and water balance, causing blood volume and blood pressure to drop. Addison's disease is also called primary adrenocortical or chronic adrenal insufficiency. It was first identified by Dr. Thomas Addison in 1849.
Who Gets It?
Addison's Disease affects about 1 in 100,000 people. It occurs in all age groups and strikes men and women equally.
What Causes It?
Gradual destruction and/or shrinking of the adrenal cortex is the most common cause of Addison's Disease. Autoimmune disorders are the cause in about 70 percent of all reported cases. Instead of identifying foreign invaders such as bacteria or viruses and killing them, the immune system makes antibodies that attack the body's own tissues or organs and slowly destroys them. When at least 90 percent of the adrenal cortex has been destroyed, adrenal insufficiency, i.e., Addison's Disease occurs. Tuberculosis accounts for about 20 percent of all cases in developed countries. Less common causes of Addison's disease may be due to fungal infections; cancer cells spreading from other parts of the body to the adrenal glands; surgical removal of the adrenal glands and amyloidosis. In amyloidosis disease a starchy substance called amyloid is deposited in abnormal places throughout the body interfering with the function of whatever structure it is present within.
What are the Symptoms?
Addison's Disease is characterized by unusual areas of darkened skin and freckling; fatigue and loss of energy; decreased appetite; nausea; vomiting; diarrhea; abdominal pain; weight loss; muscle weakness; dizziness when standing and dehydration. As the disease progresses, the skin takes on even more of a tanned appearance with a darkening of the lining of the mouth, vagina, rectum and the area around the nipples (aereola). Gradually, as dehydration becomes more severe, blood pressure continues to drop and the patient feels increasingly weak and light-headed. Some patients experience depression and irritability. Women stop having periods and lose pubic and underarm hair. About 25% of all Addison's disease patients are identified after becoming ill with an infection or stressed by an injury, which causes the disease to suddenly progress at a rapid rate, becoming life-threatening. This "Addisonian crisis" brings abnormal heart rhythms, severe pain in the back and abdomen, uncontrollable nausea and vomiting, a drastic drop in blood pressure, kidney failure, and unconsciousness.
How is it diagnosed?
Except for the onset of an "Addisonian crisis", most patients do not recognize the usually gradual progression (weeks or months), of symptoms in Addison's disease. It is ultimately revealed when a physician notices the areas of increased pigmentation of the skin. Once suspected, blood tests will reveal abnormally low blood concentrations of sodium and glucose, a greater than normal level of serum potassium, and a decreased urinary output of certain steroids. Since normal levels of the steroid cortisol vary widely, the diagnosis is established if the amount of cortisol in the plasma and steroid in the urine does not increase after the patient is given a testing dose of a synthetic hormone (ACTH).
What is the treatment?
Addison's disease treatment involves replacing the missing or low levels of cortisol. Glucocorticoid, adrenal genital corticoids, and mineralocorticoid drugs are used. In addition, an adequate fluid intake will be necessary, and control of sodium and potassium balance, and a diet high in complex carbohydrate and protein. Patients will need to take a steroid preparation (hydrocortisone) and a replacement for aldosterone (fludrocortisone) by mouth the rest of their lives.
Self-Care Tips
Under a physician's care, after diagnosis and appropriate treatment has been administered, patients can expect to enjoy a normal life. Without treatment patients are always at risk for developing "Addisonian crisis".