Last Updated: October 24, 2017

Addison's Disease and Addisonian Crisis - Adrenal Insufficiency

Overview

Addison’s disease is a rare disorder in which the adrenal glands fail to produce adequate amounts of certain hormones. The resulting low hormone levels impair a number of essential bodily functions.

The adrenal glands are small tissues located just above the kidneys. Normally, the outer part of the adrenal gland (adrenal cortex) produces hormones that regulate metabolism, maintain blood pressure, and support sexual reproduction. The inner part of the adrenal gland (adrenal medulla) produces hormones that help the body respond to high levels of stress.

In Addison’s disease, the adrenal cortex is damaged and becomes unable to produce adequate amounts of the following hormones:

This disease can affect both males and females of any age. Most cases of Addison’s disease occur in adults age 30-50.

adrenal glands

Causes of Primary and Secondary Adrenal Insufficiency

Primary adrenal insufficiency

Addison’s disease is also known as primary adrenal insufficiency. This condition is caused by disorders that directly damage the adrenal cortex, including:

Secondary adrenal insufficiency

Another type of adrenal gland dysfunction, secondary adrenal insufficiency, is caused by a problem outside of the adrenal glands themselves. Normally, the amount of cortisol produced by the adrenal glands is regulated by the hypothalamus and pituitary glands in the brain. The hypothalamus gland secretes corticotropin-releasing hormone (CRH), which triggers the pituitary gland to secrete adrenocorticotropic hormone (ACTH), which in turn stimulates the adrenal glands to produce cortisol. Secondary adrenal insufficiency occurs when there is a problem in the pituitary gland that lowers ACTH and cortisol. Secondary adrenal insufficiency can also be caused by long-term use of corticosteroid medication that suppresses adrenal gland production of cortisol.

Symptoms of the Disease and Addisonian Crisis

Usually the initial stage of Addison’s disease progresses slowly and produces vague symptoms that are often ignored until there is a stressful event, such as surgery or trauma, which causes the symptoms to worsen abruptly.

The most common signs and symptoms of Addison’s disease include:

Untreated Addison’s disease can result in Addisonian crisis, a life-threatening illness associated with the following signs and symptoms:

Diagnosis

At the onset of Addison’s disease, the symptoms are often nonspecific, making the disease very difficult to diagnose.

The physical exam may show darkening of the skin and mucous membranes, signs of dehydration, and low blood pressure.

When a health care provider suspects a patient has Addison’s disease, a number of laboratory tests are used to confirm the diagnosis.

Treatment

In Addison’s disease, the basis of treatment is the replacement of the hormones that the adrenal glands are not producing. Treatment controls symptoms but does not cure the disease; life-long therapy is required. The hormone replacement therapy includes following:

Addisonian crisis is a life-threatening emergency that is fatal if not treated promptly. The following treatments are administered:

Preventing Addisonian Crisis

Currently, there are no known methods to prevent Addison’s disease. However, steps can be taken to prevent Addisonian crisis in people already diagnosed with the disease.

Immediately prior to undergoing any surgery, a person with Addison’s disease should be treated with an intravenous injection of corticosteroid to support the blood pressure during the stress of the procedure and prevent Addisonian crisis. Also, during a major illness, the dose of corticosteroid needs to be increased. Patients are advised to wear a medical alert bracelet indicating their diagnosis of adrenal insufficiency and carry a dose of injectable corticosteroid in case of an emergency.

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Carla Hightower, MD

Dr. Hightower is an experienced physician who studied medicine at Northwestern University, where she also earned an MBA. As the founder of Living Health Works, she offers health coaching to individuals, private groups and corporations.