Addison's Disease Health Article

Diagnosis

Many patients do not recognize the slow progression of symptoms and the disease is ultimately identified when a physician notices the areas of increased pigmentation of the skin. Once suspected, a number of blood tests can lead to the diagnosis of Addison's disease. It is not sufficient to demonstrate low blood cortisol levels, as normal levels of cortisol vary quite widely. Instead, patients are given a testing dose of another hormone called corticotropin (ACTH). ACTH is produced in the body by the pituitary gland, and normally acts by promoting growth within the adrenal cortex and stimulating the production and release of cortisol. In Addison's disease, even a dose of synthetic ACTH does not increase cortisol levels.

To distinguish between primary adrenocortical insufficiency (Addison's disease) and secondary adrenocortical insufficiency (caused by failure of the pituitary to produce enough ACTH), levels of ACTH in the blood are examined. Normal or high levels of ACTH indicate that the pituitary is working properly, but the adrenal cortex is not responding normally to the presence of ACTH. This confirms the diagnosis of Addison's disease.

Treatment

Treatment of Addison's disease involves replacing the missing or low levels of cortisol. In the case of Addisonian crisis, this will be achieved by injecting a potent form of steroid preparation through a needle placed in a vein (intravenous or IV). Dehydration and salt loss will also be treated by administering carefully balanced solutions through the IV. Dangerously low blood pressure may require special medications to safely elevate it until the steroids take effect.

Patients with Addison's disease will need to take a steroid preparation (hydrocortisone) and a replacement for aldosterone (fludrocortisone) by mouth for the rest of their lives. When a patient has an illness which causes nausea and vomiting (such that they cannot hold down their medications), he or she will need to enter a medical facility where IV medications can be administered. When a patient has any kind of infection or injury, the normal dose of hydrocortisone will need to be doubled.

Prognosis

Prognosis for patients appropriately treated with hydrocortisone and aldosterone is excellent. These patients can expect to enjoy a normal lifespan. Without treatment, or with substandard treatment, patients are always at risk of developing Addisonian crisis.

BOOKS

Williams, Gordon H., and Robert G. Dluhy. "Hypofunction of the Adrenal Cortex." In Harrison's Principles of Internal Medicine, ed. Anthony S. Fauci, et al. New York: McGraw-Hill, 1997.

PERIODICALS

Brosnan, C. M., and N. F. C. Gowing. "Addison's Disease." British Medical Journal 312, no. 7038 (27 Apr. 1996): 1085+.

Oelkers, Wolfgang. "Adrenal Insufficiency." New England Journal of Medicine 335, no. 16 (17 Oct. 1996): 1206+.

ORGANIZATIONS

National Adrenal Disease Foundation. 505 Northern Boulevard, Suite 200, Great Neck, NY 11021. (516) 487-4992.

Rosalyn Carson-DeWitt, MD

KEY TERMS

Gland—A collection of cells whose function is to release certain chemicals, or hormones, which are important to the functioning of other, sometimes distantly located, organs or body systems.

Hormone—A chemical produced in one part of the body, which travels to another part of the body in order to exert its effect.