Addison's Disease

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What is Addison’s disease?

Addison’s disease, also called primary adrenal insufficiency, occurs when your adrenal glands are not able to make enough hormones that are required for a variety of processes in the body. Damage to your adrenal glands is the most common cause of Addison’s disease. Although current statistics are not available, Addison’s disease is considered a rare disorder, affecting one to four of every 100,000 people (Source: NIDDK).

Your adrenal glands are located just above your kidneys. They are part of a complex system called the endocrine system. The cortex or outer part of your adrenal gland is responsible for making three types of hormones: glucocorticoids, mineralocorticoids, and sex hormones. Glucocorticoids, such as cortisol, help your body regulate energy needs, immune function, and the stress response. Mineralocorticoids, such as aldosterone, work to regulate the sodium and potassium balance in your body. Glucocorticoids and mineralocorticoids also both help control blood pressure. Sex hormones, such as estrogen and testosterone, regulate sexual development and sex drive. In Addison’s disease, damage to your adrenal cortex results in the glands not being able to produce enough of these hormones, particularly cortisol and aldosterone.

Addison’s disease affects both males and females of all ages. Most cases of Addison’s disease are caused by gradual destruction of the adrenal gland by the body’s immune system. This is known as an autoimmune disorder. Less frequent causes of Addison’s disease include infections, bleeding into the adrenal glands, tumors, genetic defects, and certain medications. Although the damage cannot be reversed, Addison’s disease is readily treatable with replacement hormones. Most people with Addison’s disease can lead normal lives by taking an adrenal hormone replacement.

A serious complication of Addison’s disease called Addisonian crisis (adrenal cris is) requires emergency medical care. In Addisonian crisis, a person has critically low levels of cortisol resulting in low blood pressure and other serious problems. Symptoms include abdominal pain, difficulty breathing, low blood pressure, and altered consciousness. Symptoms of low blood pressure may include blurry vision, confusion, dizziness, faintness, light-headedness, sleepiness and weakness. Seek immediate medical care (call 911) if you, or someone you are with, have any of these symptoms.

Seek prompt medical care if you are being treated for Addison’s disease and you are vomiting and unable to keep your medication down, have an infection, are injured, become dehydrated, are gaining weight, have swollen ankles, or develop other new symptoms.

What are the symptoms of Addison’s disease?

Symptoms of Addison’s disease usually begin gradually and may be overlooked because they progress slowly. Often, a stressful event, such as an accident or illness, will cause symptoms to worsen and progress more rapidly. The most common symptoms of Addison’s disease include fatigue, weakness, loss of appetite, and unintentional weight loss. Other symptoms may affect the digestive tract, respiratory system, nervous system, reproductive system, cardiovascular system, or integumentary system (skin and associated tissues).

Common symptoms of Addison’s disease

The most common symptoms of Addison’s disease include:

Other symptoms of Addison’s disease

Other possible symptoms of Addison’s disease include:

Symptoms that might indicate a serious condition

Seek prompt medical care if you, or someone you are with, are being treated for Addison’s disease and have any of the following symptoms:

  • Gradual weight gain

  • New symptoms that you have not had previously

  • Not able to keep your medications down due to vomiting

  • Swelling of your ankles

  • Unusual stressor, such as an infection, injury or trauma

Symptoms that might indicate a life-threatening condition

Symptoms of Addison’s disease may progress suddenly and become severe, leading to a life-threatening situation called an Addisonian crisis (acute adrenal crisis). Seek immediate medical care (call 911) if you, or someone you are with, have any of the following symptoms:

  • Change in mental status such as confusion

  • Difficulty breathing

  • Loss of consciousness or change in consciousness such as passing out

  • Low blood pressure symptoms, such as blurry vision, dizziness, fainting, or light-headedness

  • Severe nausea and vomiting

  • Sudden abdominal pain, low back pain, or leg pain

  • Symptoms of dehydration, such as dry mouth and cracked lips, dizziness, headache, and not urinating or urinating very small amounts of dark urine

What causes Addison’s disease?

Addison’s disease is caused by damage, destruction or malfunction of the adrenal glands. Your adrenal glands are located just above your kidneys and are responsible for producing various hormones. These hormones are responsible for regulating your body’s stress response, energy needs, immune function, salt and water balance, and sexual development. When your adrenal glands are damaged or destroyed, your body no longer has enough of these hormones and adverse symptoms occur.

Autoimmune causes of Addison’s disease

In most cases, Addison’s disease is caused by an autoimmune disorder or the gradual destruction of your adrenal glands by your body’s immune system. Autoimmune disorders occur when your body’s immune system makes antibodies that attack your own organs and tissues as if they were foreign invaders.

Infectious causes of Addison’s disease

Addison’s disease may arise from infectious diseases, such as:

Other causes of Addison’s disease

Various other causes of Addison’s disease include:

  • Amyloidosis (rare immune-related disorder characterized by protein buildup in organs and tissues that can cause serious complications)

  • Bleeding or hemorrhage of the adrenal glands

  • Genetic defects

  • Surgical removal of the adrenal glands and certain other endocrine glands such as the pituitary gland

  • Tumor or adrenal cancer

  • Use of certain drugs, such as anticoagulants (blood thinners), ketoconazole (Nizoral), rifampin (Rifadin, Rimactane), phenytoin (Dilantin), etomidate (Amidate), and barbiturates

What are the risk factors for Addison’s disease?

Certain autoimmune disorders, genetic defects, and conditions are thought to increase your chances of developing the autoimmune form of Addison’s disease. These include:

  • Chronic thyroiditis or Hashimoto’s disease (inflammation of the thyroid gland)

  • Dermatitis herpetiformis (chronic, itchy rash)

  • Family history of Addison’s disease caused by an autoimmune disease

  • Grave’s disease (type of hyperthyroidism resulting in excessive thyroid hormone production)

  • Hypoparathyroidism (underactive parathyroid gland) or hypopituitarism (underactive pituitary gland)

  • Myasthenia gravis (autoimmune neuromuscular disorder that causes muscle weakness)

  • Pernicious anemia (decrease in red blood cells due to poor vitamin B12 absorption)

  • Psychological or physical stress, such as injury, illness, infection, or major surgery, especially if you have been taking steroid medications for a long time

  • Testicular dysfunction

  • Type 1 diabetes (chronic condition where the pancreas produces too little or no insulin so your body cannot properly process sugar)

  • Vitiligo (autoimmune skin disorder that causes white patchy areas of skin)

How is Addison’s disease treated?

There is no cure once your adrenal glands are damaged to the point that Addison’s disease develops. However, the symptoms of Addison’s disease can be controlled by replacing the hormones that your adrenal glands can no longer make. This medication therapy will likely be needed throughout your life.

When your adrenal glands cannot make cortisol, it can be replaced with a synthetic glucocorticoid, such as dexamethasone (Decadron), hydrocortisone (Cortef), or prednisone (Deltasone). If your aldosterone levels are also low, you can be given a mineralocorticoid called fludrocortisone (Florinef).

It is extremely important not to skip any doses of your medication. Missing doses can lead to life-threatening situations. It is also important to contact your health care provider if you have any added stress in your life, either due to illness or mental stress including:

  • Anxiety and fear

  • Dehydration

  • Illness

  • Infection

  • Injury

  • Trauma

Your health care provider may need to adjust the dose of your medication to help your body handle these types of stresses. Do not change the amount of medication you take without first talking with your health care provider.

Addisonian crisis or adrenal crisis

An Addisonian crisis or adrenal crisis is a life-threatening condition that requires immediate medical care. Standard treatment consists of intravenous glucocorticoids, large volumes of intravenous saline with dextrose (sugar), and blood pressure support.

People with Addison’s disease are often taught to give themselves an emergency injection of hydrocortisone when they experience symptoms of an Addisonian crisis. Symptoms of an Addisonian crisis include abdominal pain, difficulty breathing, low blood pressure, and altered consciousness. Symptoms of low blood pressure may include blurry vision, confusion, dizziness, faintness, light-headedness, sleepiness, and weakness. If you have access to this emergency injection, administer the drug and seek immediate medical care (call 911).

It is important to carry medical identification, such as a medical identification card, that lists Addison’s disease and the medications you take to control your condition. It may also be helpful to wear a Medic-Alert tag to alert health care professionals about your condition.

What are the potential complications of Addison’s disease?

Complications of Addison’s disease include Addisonian crisis (adrenal crisis) in which a person has critically low levels of cortisol resulting in low blood pressure and other life-threatening problems. In addition, a life-threatening situation can develop if you take too little or too much replacement hormone medication. These complications can be serious and even life threatening.

You can best treat Addison’s disease and lower your risk of complications by taking your medication exactly as directed and following the treatment plan you and your health care professional design specifically for you. You can also help avoid complications by talking with your health care provider if you develop dehydration, illness, infection, injury, trauma, or any other unusual stress.

Complications can also develop from a related or underlying disease, disorder or condition including:

  • Chronic thyroiditis or Hashimoto’s disease

  • Diabetes

  • Hypoparathyroidism

  • Ovarian or testicular failure

  • Pernicious anemia

  • Thyrotoxicosis

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Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2020 Nov 1
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THIS TOOL DOES NOT PROVIDE MEDICAL ADVICE. It is intended for informational purposes only. It is not a substitute for professional medical advice, diagnosis or treatment. Never ignore professional medical advice in seeking treatment because of something you have read on the site. If you think you may have a medical emergency, immediately call your doctor or dial 911.
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