Addisons disease

Endocrine glands
Definition:
Addison's disease is a hormone deficiency caused by damage to the outer layer of the adrenal
gland (adrenal cortex).
Alternative Names:
Adrenocortical hypofunction; Chronic adrenocortical insufficiency; Adrenal insufficiency
Causes, incidence, and risk factors:
The adrenal glands are small hormone-secreting organs 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 3 types of hormones: sex hormones, glucocorticoid hormones, and
mineralocorticoid hormones.

The sex hormones, androgens (male) and estrogens (female) affect sexual development
and reproduction.
The glucocorticoid hormones (such as cortisol) maintain glucose (sugar) regulation,
suppress the immune response, and help the body respond to stress.
The mineralocorticoid hormones (such as aldosterone) regulate sodium and potassium
balance.

Addison's disease results from damage to the adrenal cortex, which causes decreased production
of adrenocortical hormones. This damage may be caused by the following:

The immune system mistakenly attacking the gland (autoimmune disease)

Infections such as tuberculosis, HIV, or fungal infections

Hemorrhage, blood loss

Tumors

Use of blood-thinning drugs (anticoagulants)

Risk factors for the autoimmune type of Addison's disease include other autoimmune diseases:

Type I diabetes
Hypoparathyroidism

Hypopituitarism

Pernicious anemia

Testicular dysfunction

Graves' disease

Chronic thyroiditis

Candidiasis

Dermatis herpetiformis

Vitiligo

Myasthenia gravis

These may be caused by certain genetic defects.

Symptoms:
Extreme weakness

Fatigue

Unintentional weight loss

Nausea and vomiting

Chronic diarrhea

Loss of appetite

Darkening of the skin - patchy skin color

o

Unnaturally dark color in some locations

Paleness may also occur

Mouth lesions on the inside of a cheek (buccal mucosa) - pigmentation

Slow, sluggish, lethargic movement

Changes in the blood pressure or heart rate

Salt craving
Signs and tests:
Blood pressure is low.

Cortisol level is low.

Serum sodium is low.

A potassium test may show increased potassium.

An abdominal x-ray may show adrenal calcification.

An abdominal CT scan may show adrenal calcification, enlargement or atrophy.

This disease may also alter the results of the following tests:
Renin
Potassium test

Cortisol, urine

CO2

Aldosterone

ACTH

24 hour urinary aldosterone excretion rate

17-ketosteroids

17-hydroxycorticosteroids

Cortrosyn stimulation test

Blood eosinophil count (a special white blood cell)

Treatment:
Replacement therapy with corticosteroids will control the symptoms of this disease. However,
these drugs must usually be continued for life. Usually a combination of glucocorticoids
(cortisone or hydrocortisone) and mineralocorticoids (fludrocortisone) are given.

Medication may need to be increased during times of stress, infection, or injury.

Adrenal crisis is an extreme manifestation of symptoms of adrenal insufficiency brought on by
physical stress. Hydrocortisone must be injected immediately to sustain life. Supportive
treatment for low blood pressure is usually necessary as well.
Some people with Addison's disease are taught to give themselves an emergency injection of
hydrocortisone during stressful situations. It is important for the individual with Addison's
disease to always carry a medical identification card that states the type of medication and the
proper dose needed in case of an emergency.
Never skip doses of medication for this condition, as life-threatening reactions may occur. If
unable to retain medication due to vomiting, notify your health care provider, go to the
emergency room, or call the local emergency number (such as 911) immediately.
Also report sudden weight gain or fluid retention to the health care provider.

Expectations (prognosis):
With adequate replacement therapy, most people with Addison's disease are able to lead normal
lives.
Complications:
Complications may result from the following associated illnesses:

Diabetes
Thyrotoxicosis

Hashimoto's thyroiditis

Hypoparathyroidism

Pernicious anemia

Ovarian hypofunction or testicular failure

Calling your health care provider:
Call your health care provider if Addison's disease has been diagnosed and stress such as
infection, injury, trauma, or dehydration occurs. Medication adjustment may be indicated.

Call your provider if weight increases progressively, the ankles begin to swell, or other new
symptoms develop.
If symptoms of adrenal crisis (low blood pressure, diminished consciousness, difficulty
breathing, abdominal pain) occur, give an emergency injection of prescribed medication as
instructed or -- if this is not available -- go to the emergency room or call 911.
Review Date: 8/6/2004
Reviewed By: Aniket R. Sidhaye, M.D., Division of Endocrinology and Metabolism, Johns
Hopkins University School of Medicine, Baltimore, MD. Review provided by VeriMed
Healthcare Network.