In 1855, the British physician Thomas Addison described a patient who showed “general languor and debility, remarkable feebleness of the heart’s action, irritability of the stomach, and a peculiar change of the color of the skin.” This classic description summarizes the major features of chronic adrenocortical deficiency—also known as Addison’s disease or chronic glucocorticoid deficiency.
SymptomsAn unusually persistent tanning following exposure to the sun may be one of the first signs of Addison’s disease. Other symptoms are a feeling of physical weakness and loss of weight.
Addison’s disease may be primary—due to inability of the cortex of the adrenal glands to produce adequate quantities of steroid hormones, or secondary—due to failure of the pituitary gland to produce the corticotropin (ACTH) necessary to stimulate production of the adrenocortical hormones.Primary adrenocortical insufficiency is relatively rare but the secondary type is becoming more common. This is due to increasing use of large doses of corticosteroids which suppress the activity of the pituitary to secrete ACTH. Once off the large doses of corticosteroids, the individual may manifest a blunted pituitary-adrenal response to stress for as long as a year or more.
Diagnosis of primary adrenal insufficiency is relatively simple when the disease is advanced but is difficult in the early stages. The cardinal sign of the disease is weakness, increasing as the disease progresses; the most striking sign is the diffuse brown, tan, or bronze darkening of exposed and unexposed points such as creases of the hands or the elbows. In fact, sometimes the first thing the patient notices is an unusually persistent tanning following exposure to
the sun. Low blood pressure and digestive disorders ranging from loss of appetite to overwhelming nausea, vomiting, diarrhea, and abdominal pain occur frequently.
Definitive diagnosis is made by a number of laboratory tests: measuring the quantities of specific hormones—17 hydroxycorticosteroids and 17 ketosteroids—in a specimen of urine collected over a 24-hour period; measuring the amount of the hormone aldosterone that is excreted in the urine, and measuring the levels of hydrocortisone in blood after ACTH stimulation.
The outlook for patients is excellent when the disease is diagnosed in the earlier stages, since continuous treatment with replacement hormones provides complete correction of the derangement. For example, President John F. Kennedy had Addison’s disease but was on an effective program of replacement therapy and had no trouble maintaining a very active life, Patients with adrenal insufficiency usually are educated completely in the nature of their disease, and should carry medical identification, and be registered with a national medical alerting system.