Adrenal Insufficiency (Addison Disease)

What is adrenal insufficiency?

Adrenal insufficiency occurs when the adrenal glands don’t make enough of the hormone cortisol. Cortisol helps with your stress response, regulates metabolism, regulates inflammation, and affects how your immune system works. Cortisol is able to affect nearly every organ system of your body

You have two adrenal glands, one on top of each kidney. They work in conjunction with the hypothalamus and pituitary glands in the brain.

Adrenal insufficiency can be primary, secondary, or tertiary:

• Primary adrenal insufficiency. This is known as Addison disease. It occurs when the adrenal glands are damaged. They don’t make enough of the hormones cortisol and aldosterone. This condition is rare. It may occur at any age. Addison disease is an autoimmune condition in which your own antibodies attack the adrenal gland.

• Secondary adrenal insufficiency. This starts when the pituitary gland doesn’t make enough of the hormone ACTH (adrenocorticotropin). As a result, the adrenal glands don’t make enough cortisol.

• Tertiary adrenal insufficiency. This starts when the hypothalamus doesn't make enough corticotropin-releasing hormone. As a result the pituitary gland doesn't make enough ACTH. The result is that the adrenal glands don't make enough cortisol.

What causes adrenal insufficiency?

Primary adrenal insufficiency is most often caused when your immune system attacks your healthy adrenal glands by mistake. Other causes may include:

• Cancer

• Fungal infections

• Tuberculosis infection of the adrenal glands 

• Inherited disorders of the endocrine glands.

• Hemorrhage

A lack of the hormone ACTH leads to secondary adrenal insufficiency. That can happen if you must take certain steroids for a long time because of a health problem. For example, people with asthma or rheumatoid arthritis may need to take prednisone. Other causes include:

• Pituitary gland tumors

• Loss of blood flow to the pituitary 

• Removal of the pituitary gland or radiation treatment of the pituitary gland

• Removal of parts of the hypothalamus

What are the symptoms of adrenal insufficiency?

Each person’s symptoms will vary. Symptoms may include:

• Weakness

• Tiredness and lack of energy (fatigue)

• Dizziness

• Darkened skin, often on the face, neck, and back of hands (Addison disease only)

• Bluish-black color around the nipples, mouth, rectum, scrotum, vagina, or other places (Addison disease only)

• Weight loss

• Fluid loss (dehydration)

• Lack of appetite

• Craving of salty foods

• Muscle aches

• Upset stomach (nausea)

• Vomiting

• Diarrhea

• Low blood pressure

• Low sugar levels

• In women, irregular or no menstrual periods

If not treated, adrenal insufficiency may lead to:

• Severe belly (abdominal) pain

• Extreme weakness

• Low blood pressure

• Kidney failure

• Shock

These symptoms may look like other health problems. Always see your healthcare provider for a diagnosis.

How is adrenal insufficiency diagnosed?

Your healthcare provider will ask about your health history. You will also need a physical exam. Tests that can help your provider diagnose or find the cause of adrenal insufficiency include:

• Blood, urine, or saliva tests. These can check levels of the adrenal hormones and ACTH. They can also check changes in the sodium and potassium levels in your blood.

• Tuberculosis tests. These tests look for the germ that causes tuberculosis.

• Imaging tests. These include X-rays, ultrasound, CT scan, and MRI.

How is adrenal insufficiency treated?

Treatment will depend on your symptoms, age, and general health. It will also depend on how severe the condition is.

You will need to take hormones to replace those that your adrenal glands are not making. That mainly means cortisol.

Addison disease can be deadly. Treatment often starts with IV (intravenous) fluids and medicines called corticosteroids. You may take these medicines by mouth or by IV. You may have to take them for the rest of your life. You may also need to take other medicines (mineralocorticoids, such as fludrocortisone). These can help keep your body's sodium and potassium levels normal. Don't suddenly stop taking any medicines you are taking for other reasons (such as inhaled steroids for asthma) without talking with your healthcare provider. This is because your adrenal glands will not be able to make cortisol right away.

What are possible complications of adrenal insufficiency?

You may have sudden severe symptoms. This is called acute adrenal insufficiency, or Addisonian crisis. This can occur when your body is stressed. It can happen for many reasons, such as an illness, fever, surgery, or dehydration. You may also have a crisis if you stop taking your corticosteroids or lower the amount of your medicines suddenly. The symptoms of an Addisonian crisis include the symptoms of adrenal insufficiency or Addison disease. But if an Addisonian crisis is not treated, it can lead to:

• Shock

• Seizures

• Coma

• Death

Living with adrenal insufficiency

Take your medicines exactly as prescribed. You should also carry a medical alert card or tag with you at all times. This can make sure you get correct treatment if there is an emergency. When traveling, always carry an emergency kit with a shot of cortisol.

When should I call my healthcare provider?

Any condition that stresses your body can affect how much medicine you need. Call your healthcare provider if:

• You have any kind of illness, especially a fever, vomiting, or diarrhea.

• You become pregnant.

• You need surgery.

Get medical help right away if you have sudden severe symptoms (Addisonian crisis).

Key points about adrenal insufficiency (Addison disease)

• Adrenal insufficiency occurs when the adrenal glands don’t make enough of the hormone cortisol. There are 3 types of this disorder.

• The primary type is known as Addison disease. It's rare. It's when the adrenal glands don’t make enough of the hormones cortisol and aldosterone.

• The secondary type occurs when the pituitary gland doesn’t make enough of the hormone ACTH. The adrenal glands then don’t make enough cortisol.

• The tertiary type occurs when the hypothalamus doesn't make enough corticotropin-releasing hormone. Then the pituitary gland doesn't make enough ACTH. As a result, the adrenal glands don't make enough cortisol.

• Mild symptoms may be seen when a person is under physical stress. Other symptoms may include weakness, tiredness and lack of energy, and weight loss.

• You will need to take hormones to replace those that the adrenal glands are not making.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with you to help you ask questions and remember what your provider tells you.

• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.

• Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your provider if you have questions.