Addison's Disease

What is Addison's disease?

Addison’s disease is a chronic condition in which your adrenal glands don’t produce enough of the hormones cortisol and aldosterone.

Your adrenal glands, also known as suprarenal glands, are small, triangle-shaped glands that are located on top of each of your two kidneys. They’re a part of your endocrine system.

Cortisol is a hormone that helps your body respond to stress, including the stress of illness, injury or surgery. It also helps maintain your blood pressure, heart function, immune system and blood glucose (sugar) levels. Cortisol is essential for life.

Aldosterone is a hormone that affects the balance of sodium (salt) and potassium in your blood. This in turn controls the amount of fluid your kidneys remove as urine (pee), which affects blood volume and blood pressure.

Addison’s disease is also called primary adrenal insufficiency. A related disorder, secondary adrenal insufficiency, happens when your pituitary gland doesn’t release enough adrenocorticotropic hormone (ACTH), which activates your adrenal glands to produce cortisol.

What’s the difference between Addison's disease and Cushing's syndrome?

Addison’s disease happens when your body doesn’t have enough cortisol (and aldosterone), whereas Cushing’s syndrome happens when your body has too much cortisol (hypercortisolism).

Who does Addison’s disease affect?

Addison’s disease can affect people of all age groups, but it’s most common in people 30 to 50 years old.

People who have autoimmune polyendocrine syndrome, a rare, inherited condition in which your immune system mistakenly attacks many of your tissues and organs, are much more likely to have Addison’s disease. Your mucous membranes, adrenal glands and parathyroid glands are commonly affected by this syndrome, though it can affect other types of tissues and organs.

People who have the following autoimmune disease are also at higher risk of developing the autoimmune (most common) form of Addison's disease:

• Type I diabetes.
• Pernicious anemia.
• Graves' disease.
• Chronic thyroiditis.
• Dermatitis herpetiformis.
• Vitiligo.
• Myasthenia gravis.

How common is Addison’s disease?

Addison’s disease is rare. In the United States, it affects 1 in 100,000 people.

What are the symptoms of Addison’s disease?

With Addison’s disease, the damage to your adrenal glands usually happens slowly over time, so symptoms occur gradually. Symptoms vary from person to person.

Symptoms of Addison’s disease include:

• Steadily worsening fatigue (most common symptom).
• Patches of dark skin (hyperpigmentation), especially around scars and skin creases and on your gums.
• Abdominal pain.
• Nausea and vomiting.
• Diarrhea.
• Loss of appetite and unintentional weight loss.
• Muscle pain, muscle spasms and/or joint pain.
• Dehydration.
• Low blood pressure, which can cause lightheadedness or dizziness upon standing.
• Changes in mood and behavior, such as irritability, depression and poor concentration.
• A craving for salty food.
• Low blood sugar (hypoglycemia).

People assigned female at birth with Addison’s disease may also have abnormal menstruation (periods), lose body hair and have a decreased sexual drive.

In some cases — such as after an injury or severe illness or time of intense stress — symptoms can come on quickly and cause a life-threatening event called an addisonian crisis or acute adrenal failure.

An addisonian crisis is a medical emergency. If it’s not treated, it can lead to shock and death. Symptoms of an addisonian crisis include:

• Extreme weakness.
• Sudden, severe pain in your lower back, belly or legs.
• Feeling restless, confused, afraid or other mental changes.
• Severe vomiting and diarrhea, potentially leading to dehydration.
• Low blood pressure.
• Loss of consciousness.

Get to the nearest hospital as soon as possible if you’re having these symptoms.

What causes Addison’s disease?

The most common cause of Addison’s disease is an autoimmune response, which occurs when your immune system attacks healthy tissues for an unknown reason. With Addison’s disease, your immune system attacks the outer portion of your adrenal glands (the adrenal cortex), where they make cortisol and aldosterone. Symptoms don’t usually develop until 90% of the adrenal cortex has been damaged, which can take several months to years.

Approximately 75% of cases of Addison’s disease are due to an autoimmune attack. Autoimmune Addison’s disease may happen by itself or as part of a rare, inherited syndrome, specifically autoimmune polyendocrine syndromes I (APS type-1) and II (Schmidt syndrome).

In the past, tuberculosis was a major cause of Addison’s disease. It remains a prominent cause of the condition in developing countries.

Other less common causes of Addison’s disease include:

• Repeated infections, including HIV/AIDS-related infections and fungal infections.
• When cancer cells from another part of your body invade your adrenal glands.
• Bleeding (hemorrhaging) into your adrenal glands.
• Surgical removal of your adrenal glands.
• Amyloidosis (a condition in which amyloid proteins build up in vital organs, causing damage).

How is Addison’s disease diagnosed?

Since symptoms of Addison’s disease usually develop slowly over time and are usually vague and common to many different conditions, it often leads to a delay in the proper diagnosis.

Healthcare providers often “accidentally” discover Addison’s disease when a routine blood test, such as a basic metabolic panel, shows low levels of sodium or high levels of potassium.

Dark patches on your skin are another common symptom that signals healthcare providers to test for Addison’s disease.

If your provider suspects you may have Addison’s disease based on your symptoms, they’ll order more tests to officially diagnose the condition.

What tests will be done to diagnose Addison’s disease?

To determine if you have Addison’s disease, your healthcare provider may order the following tests:

• Blood tests: Your provider will likely order certain tests to measure the levels of sodium, potassium, cortisol and ACTH in your blood.
• ACTH stimulation test: This test measures your adrenal glands’ response after you’re given a shot of artificial ACTH. If your adrenal glands produce low levels of cortisol after the shot, they may not be functioning properly.
• Insulin-induced hypoglycemia test: Your provider may order this test to determine if your symptoms are due to problems with your pituitary gland (secondary adrenal insufficiency) instead of your adrenal glands. This test measures blood sugar (glucose) levels before and after the injection of fast-acting insulin, which should lead to a drop in blood sugar (hypoglycemia) and a rise in cortisol.
• Computed tomography (CT scan): Computed tomography is an imaging test that uses computers to combine many X-ray images into cross-sectional views. Your provider may order a CT scan to evaluate your adrenal glands and/or pituitary gland. For example, it can show if your immune system has damaged your adrenal glands or if the glands are infected.

How is Addison’s disease treated?

Addison’s disease is treated by replacing the missing hormones, cortisol and aldosterone, with synthetic versions of them.

Cortisol is replaced by the drug hydrocortisone, and aldosterone is replaced by the drug fludrocortisone. Addison’s disease is a chronic condition, so you’ll need to take medication for the rest of your life.

The dosages of these medications are different for each person, and your healthcare provider may increase the dosage when you’re experiencing an infection, trauma, surgery and other stressful situations to prevent an acute adrenal crisis.

If you’re taking fludrocortisone, your provider might tell you to increase your salt intake, especially in hot and humid weather and after vigorous exercise.

Can Addison’s disease be prevented?

Unfortunately, there’s nothing you can do to prevent Addison’s disease.

What is the prognosis (outlook) for Addison’s disease?

The prognosis for Addison’s disease is generally good. Although people who have Addison’s disease will need to take medicine for the rest of their lives, they can live normal, healthy lives.

The dosages of these medications, however, need to be closely monitored to prevent over- or under-treatment. Over-treatment with glucocorticoids (hydrocortisone) may result in obesity, Type 2 diabetes and osteoporosis. Over-treatment with fludrocortisone can cause high blood pressure (hypertension).

Up to 50% of people with Addison’s disease develop another autoimmune condition.

How do I take care of myself if I have Addison’s disease?

If you have Addison’s disease, you should carry an identification card and wear a medical alert bracelet or necklace at all times to let medical professionals know you have the condition in emergencies.

Talk to your healthcare provider about what you should do when you become sick or are experiencing intense stress since you’ll likely need to increase your medication dosages.

Ask your provider about keeping a shot of cortisol for emergencies, and be sure someone with you knows how to give you the shot.

When should I see my healthcare provider about Addison’s disease?

If you have Addison’s disease, you’ll need to see your healthcare provider (likely an endocrinologist) regularly to make sure your medication dosages are working for you.

Call your provider if you have major stress — such as an injury, illness or the death of a loved one — because you might need an adjustment to your medicine.

Seek medical attention right away if you have any of the symptoms of an addisonian crisis, such as sudden, extreme weakness and intense pain.

A note from Cleveland Clinic

Receiving a diagnosis of a chronic condition can be overwhelming and stressful. The good news is that Addison’s disease is treatable with medication. It’s important to see your healthcare provider regularly to make sure your dosages are correct and to prevent an episode of acute adrenal failure. If you have any questions about managing Addison’s disease, talk to your provider. They’re always available to help you.