Addison's disease

Alternative names:

adrenal insufficiency; adrenocortical hypofunction; chronic adrenocortical insufficiency

Definition:

Adrenocortical hormone deficiency due to damage to the outer layer of the adrenal gland (adrenal cortex). The damage may be caused by the immune system attacking the gland (an autoimmune process), tuberculosis, hemorrhage, cancer, or infection.

A complication of Addison's disease, acute adrenal failure or adrenal crisis, is a life-threatening emergency that requires immediate treatment with corticosteroids.

Causes, incidence, and risk factors:

The adrenal glands are located on top of each kidney. They consist of the outer portion, called the cortex, and the inner portion, called the medulla. The cortex produces three types of hormones called corticosteroids, which are essential for life: androgens and estrogens, glucocorticoid hormones, and mineralocorticoid hormones. The androgens and estrogens affect sexual development and reproduction. The glucocorticoid hormones (such as cortisol) maintain glucose regulation, suppress the immune response, and provide for the response to stress. The mineralocorticoid hormones (such as aldosterone) regulate sodium and potassium balance.

Addison's disease is a rare disorder that can occur at any age and affects men and women equally. The incidence is 8 in 100,000 people.

Risk factors include rare familial autoimmune diseases and other diseases. These include: premature cessation of menstruation, type I diabetes mellitus, hypoparathyroidism, hypopituitarism, pernicious anemia, testicular dysfunction, Graves' disease, chronic thyroiditis, candidiasis, dermatis herpetiformis, vitiligo and myasthenia gravis.