Addison's Disease

Addison's disease is a chronic condition that results when the adrenal cortex, the outer layer of the adrenal glands, is damaged, destroyed, or otherwise functions incorrectly, and the glands are unable to produce enough of certain important hormones. This condition can lead to fatigue, low blood pressure, loss of appetite, and darkening of the skin. While Addison's disease is highly treatable with daily oral medication, it can be fatal if care is inadequate or lacking.




The adrenal glands are located above each of the two kidneys. When people have Addison's disease, the adrenal glands do not produce enough of the hormones cortisol and aldosterone.





The adrenal glands are located above each of the two kidneys. When people have Addison's disease, the adrenal glands do not produce enough of the hormones cortisol and aldosterone.
Illustration by Frank Forney. © 2016 Cengage Learning®.

JFK's Story

Many people knew that U.S. President John F. Kennedy (1917–1963) suffered from back pain most of his life. Only after he died from an assassin's bullet in 1963, however, did the public learn that the president also had Addison's disease, a rare condition that results when the body fails to produce enough of certain hormones * .

What Is Addison's Disease?

The adrenal glands are thin triangular groups of cells about the size of an adult thumb. One adrenal gland is located above each of the two kidneys. Among other functions, the glands release hormones known as cortisol and aldosterone. In people with Addison's disease, the adrenal cortex is damaged or destroyed, and the glands no longer release enough cortisol and aldosterone to keep the body functioning normally.

Cortisol and aldosterone

Cortisol helps the body respond to stresses such as diseases and infections. It also helps the body use sugars, proteins, carbohydrates, and other substances in food for energy. Aldosterone helps signal the kidneys to regulate the amount of salt and water retained in the body, which is important because without the proper concentrations of salt and water, blood pressure can drop to dangerously low levels.

Autoimmunity

Normally, the immune system releases antibodies to fight foreign substances in the body such as viruses. The cause in about 70 percent of Addison's disease cases is a faulty immune system that turns against the body—a situation known as autoimmunity—and targets the adrenal cortex. Specifically, the antibodies and cells of the immune system destroy the adrenal cortex and cause the glands to release inadequate amounts of cortisol and aldosterone. As of 2015, scientists were still not sure why this autoimmune response occurs.

Other causes

The disease can also result from conditions that affect the pituitary gland, which is located at the base of the brain. Normally, this gland secretes a hormone called adrenocorticotropin, which stimulates the production of cortisol in the adrenal glands. When the pituitary is compromised, cortisol levels can drop and cause Addison's disease. In this case, levels of aldosterone are not affected.

A similar condition can occur in patients who have been medicated with daily high doses of cortisol-like drugs, usually for a serious disorder involving the immune system. In these patients, the sudden cessation of the medication, or a sudden increase in stress, such as major surgery or a serious infection, can produce symptoms and threats to health that are similar to many of those associated with Addison's disease. These patients should always wear an identification bracelet indicating their drug usage, and carry with them a supply of extra medication to meet any needs that arise.

Incidence

Addison's disease is rare in the early twenty-first century. It strikes only about one of every 100,000 persons.




President John F. Kennedy at his inauguration on January 20, 1961. The public did not learn until after his death that President Kennedy had Addison's disease.





President John F. Kennedy at his inauguration on January 20, 1961. The public did not learn until after his death that President Kennedy had Addison's disease.
Arnold Sachs/Consolidated News/Getty Images.

What Are Those Puzzling Feelings?

The first signs of Addison's disease can be puzzling to patients and their doctors. The lack of hormones in the body causes individuals to feel tired and weak. Patients can also feel dizzy due to low blood pressure. Appetite drops, and they start to lose weight from not eating as much. Because salt levels are out of balance in the body, people with Addison's might also feel hungry for salty foods, such as potato chips.

Sometimes people get sick to their stomachs and vomit, and they can develop dark areas on the skin, as if they are tanning. They can also seem unreasonably upset in response to certain circumstances or become depressed.

Addison's usually develops slowly over many years. The symptoms might be noticed, but ignored or explained away as the result of working too hard or not exercising enough. About 25 percent of people with Addison's disease are not aware they have the disease, and do not seek medical help until an accident or other illness triggers a sudden worsening of their symptoms. Without medical help, the sudden worsening can be fatal.

How Do Doctors Diagnose and Treat Addison's Disease?

THOMAS ADDISON

The 19th-century British physician Thomas Addison (1793–1860) was the first to connect the symptoms of Addison's disease to problems with the adrenal glands. At that time, the disease was more common because tuberculosis was widespread, and people with tuberculosis may develop Addison's disease if the infection involves and destroys the adrenal glands.

* scan, to look at the pituitary gland.

People with Addison's disease need to take prescription hormones to do the work of the missing cortisol and, if necessary, aldosterone. Most of the time, taking the prescribed hormones allows people with Addison's to avoid the disease's symptoms.

Individuals might experience a return of severe symptoms, such as low blood pressure or elevated potassium levels, if they become ill with another condition. Because these symptoms can be fatal, doctors recommend that people with Addison's wear a medical identification bracelet that explains their condition. The bracelet is especially helpful if the individual suddenly becomes sick and is unable to communicate with those trying to help.

Autoimmunity

Addison's disease is one of many diseases that are considered autoimmune disorders. Normally, the immune system protects the body from foreign substances, such as viruses. Sometimes, however, it attacks the body's own cells, tissues, or organs, causing a wide variety of medical conditions. AIDS, lupus, type 1 diabetes mellitus, and rheumatoid arthritis are just a few of the more than 80 known autoimmune disorders. Together, autoimmune diseases affect an estimated 5 to 7 percent of the population.

With treatment, however, people with Addison's disease can live as long and as well as people without the disease typically do.

See also Autoimmune Disorders: Overview • Metabolic Disease • Skin Conditions: Overview • Tuberculosis

Resources

Books and Articles

American Autoimmune Related Diseases Association. “Tips for Getting a Proper Diagnosis of an Autoimmune Disease.” http://www.aarda.org/wp-content/uploads/2013/08/tips_for_auto_diagnosis.pdf (accessed June 1, 2015).

Websites

Pituitary Network Association. “Adrenal Insufficiency (Addison's Disease).” Pituitary.org . http://pituitary.org/knowledge-base/disorders/adrenal-insufficiency-addison-s-disease (accessed June 16, 2015).

Organizations

American Autoimmune Related Diseases Association. 22100 Gratiot Ave., Eastpointe, MI 48021. Telephone: 586-776-3900. Website: http://www.aarda.org (accessed June 1, 2015).

* hormones are chemical substances that are produced by various glands and sent into the bloodstream carrying messages that have certain effects on other parts of the body.

* computed tomography (kom-PYOO-ted toe-MAHgruh- fee) or CT, also called computerized axial tomography (CAT), is a technique in which a machine takes many x-rays of the body to create a three-dimensional picture.

Disclaimer:   This information is not a tool for self-diagnosis or a substitute for professional care.

(MLA 8th Edition)