Addison's Disease: The Basics

Medically Reviewed by Jabeen Begum, MD on May 23, 2024
9 min read

One way the body keeps itself in balance is by using chemical messengers called hormones to regulate various functions. Just above each of your kidneys is a small adrenal gland. These glands make hormones essential to a healthy life. When they don't make enough of these hormones, Addison's disease is the result.

Addison's disease is a rare condition. Only one in 100,000 people have it. It can happen at any age to either men or women. People with Addison's disease can lead normal lives as long as they take their medication. President John F. Kennedy had the condition.

In Addison's disease, also called primary adrenal insufficiency, the adrenal glands don't make enough of a hormone called cortisol, or less often, a related hormone called aldosterone.

Cortisol's most important function is to help the body respond to stress. It also helps control your body's use of protein, carbohydrates, and fat, helps maintain blood pressure and cardiovascular function, and controls inflammation.

Aldosterone helps your kidneys regulate the amount of salt and potassium in your body, regulating your blood volume and keeping your blood pressure in check. When aldosterone levels drop too low, your kidneys cannot keep your salt levels in balance. This, in turn, makes your blood pressure drop.

Primary vs. secondary adrenal insufficiency

Adrenal insufficiency occurs when the adrenal glands don’t make enough hormones vital for healthy functioning. It can be primary, secondary, or tertiary. 

Primary adrenal insufficiency, or Addison’s disease, happens when the adrenal gland stops working as it should, making it unable to make enough aldosterone and cortisol. It mostly happens when the immune system mistakenly attacks and damages the adrenal cortex, the largest part of the adrenal gland.

Secondary adrenal insufficiency occurs when the pituitary, a pea-sized organ at the base of the brain above the back of the nose, stops making adrenocorticotropin (ACTH). This hormone helps the adrenal cortex work as well as it should. Though the adrenal gland stops producing enough cortisol, it can still make aldosterone. It often happens due to traumatic brain injury and panhypopituitarism, a condition where the pituitary gland stops making most or all the hormones it should make.

Tertiary adrenal insufficiency happens when the hypothalamus, a small area of the brain above the pituitary gland, stops making enough corticotropin-releasing hormone (CRH). This hormone tells the pituitary to make ACTH. Without enough ACTH, the adrenal glands can’t make enough cortisol. It often happens when you suddenly stop taking steroid medicines after using them for a while.

Addison's disease happens when the adrenal glands, which sit on your kidneys, get damaged. Your adrenal glands make cortisol when you're under stress. Addison's disease can happen quickly or come on gradually.

Addison's disease, also known as primary adrenal insufficiency, has symptoms that are vague and nonspecific. Addison's disease can be difficult to diagnose, and it often takes years for a diagnosis to be made. Symptoms of Addison's disease include:

  • Chronic fatigue and muscle weakness
  • Loss of appetite, inability to digest food, and weight loss
  • Low blood pressure (hypotension), which drops further when standing and can lead to dizziness, or even fainting
  • Blotching, dark tanning, and freckling of the skin, especially on sun-exposed areas of the body, as well as unexposed areas such as the gums. Darkened skin is particularly likely to occur on the forehead, knees, and elbows or along scars, skin folds, and creases, such as on the palms.
  • Low blood sugar, including dangerously low levels (hypoglycemia)
  • Nausea, vomiting, and diarrhea
  • Inability to deal with stress
  • Moodiness, irritability, and depression
  • Intolerance to heat or cold
  • Craving for salty foods
  • Abnormal periods in people who menstruate
  • Muscle pain spasms
  • Dehydration
  • Joint pain
  • Body hair loss and lower sexual drive in people assigned female at birth

Some of these symptoms may indicate conditions other than Addison's disease.

Addison’s disease happens when there’s a problem with the adrenal glands themselves. It can be due to the following factors:

Autoimmune disease. Autoimmune disease accounts for 70% of cases of Addison’s disease. This occurs when the body's immune system mistakenly attacks the adrenal glands. This autoimmune assault destroys the outer layer of the glands. You’re more likely to have Addison’s disease with other autoimmune conditions, including autoimmune thyroid disease or type 1 diabetes.

Infections. Long-lasting infections -- such as tuberculosis, HIV, sepsis, cytomegalovirus (CMV) infection, and some fungal infections -- can harm the adrenal glands and lead to Addison’s disease. Although rare, the adrenal glands can be injured due to a bacterial infection.

Cancer. Although rare, cancer cells that spread from other parts of the body to the adrenal glands can also cause Addison's disease. This happens when cancer cells destroy over 90% of the adrenal cortex. Cancers that have been reported to have moved into the adrenal glands include lung, breast, skin, gastric, blood, and colorectal cancer.

Genetic factors. Genetic factors, including genes called the human leukocyte antigen (HLA) complex, may contribute to Addison’s disease. Genetic conditions such as X-linked adrenoleukodystrophy and autoimmune polyglandular syndrome type 1 may also cause Addison’s disease. That’s why Addison’s disease may run in families.

Internal bleeding. Less commonly, internal bleeding in the adrenal glands can cause Addison’s disease. Bleeding in the adrenal glands may happen due to:

  • Trauma, such as from a car accident or falls
  • Serious infections
  • Heart attack or heart failure
  • Clotting disorders
  • Liver transplant
  • COVID infection
  • Surgery involving the adrenal glands
  • Tumors in the adrenal glands
  • Tendency to bleed from taking blood thinners
  • Newborn birth injury

Surgery. You may be more likely to have Addison’s disease if you had surgery that involved your pituitary or adrenal glands, such as an adrenalectomy, which is a surgery to remove your adrenal glands.

Radiation. Addison’s disease may happen as a complication of treating adrenal glands that have cancer cells in them with radiation.

Blood test results that point to Addison's disease include a low sodium or high potassium level, anemia (low iron), or a high level of eosinophils (a kind of white blood cell). Often, it's first found through regular blood tests in a hospital or doctor's office. The doctor will also check for hyperpigmentation, or darkening, of the skin or gums -- a sign of long-term Addison's disease.

The most definitive way to diagnose Addison’s is to measure hormone levels in the blood before and after giving ACTH. ACTH is a hormone released in the brain that normally increases cortisol release from the adrenal glands. With Addison's disease, the adrenal glands cannot respond to ACTH stimulation, and cortisol levels remain low. Measuring cortisol and ACTH levels can help determine whether adrenal insufficiency is there, and if so, whether the problem is with the adrenal glands or brain.

An evaluation of Addison's disease can include a CT scan of the adrenal glands to look for infection, cancer, or bleeding in the adrenal glands. A tuberculosis test may also be done because up to 20% of cases are caused by tuberculosis. But the majority of cases are autoimmune.

Because Addison's disease is caused by a lack of normal hormones produced by the adrenal glands, it can be treated by replacing those hormones. This can be done with once- or twice-daily tablets of hydrocortisone, a steroid hormone. If needed, aldosterone can be replaced with a synthetic steroid, fludrocortisone acetate (Florinef), which is taken by mouth once a day. These medications would need to be increased during times of stress, infection, surgery, or injury.

Treatment is almost always completely successful. When treated, people with Addison's disease can lead a full, normal life. It is important to carry a medic alert bracelet and emergency ID card at all times. You should also keep a small supply of medicine at work or school. Missing even one dose can be dangerous.

In people who may be having an Addisonian crisis, doctor-prescribed injections of salt, fluids, and glucocorticoid hormones may be given immediately -- even before a diagnosis of Addison's disease is confirmed.

The most serious complication that can come from Addison’s disease is an adrenal crisis or Addisonian crisis.

Addisonian crisis

Because symptoms of Addison's disease progress slowly, they may go unrecognized until a physically stressful event — such as another illness, surgery, or an accident — worsens symptoms quickly. When this happens, it's called an Addisonian crisis.

For 1 in 4 people with Addison's disease, this is the first time they realize they are ill. An Addisonian crisis is considered a medical emergency because it can be fatal.

Symptoms of an Addisonian crisis include:

  • Shock, when the body does not get enough blood flow
  • Multiple organ failure, including kidneys, if the blood circulation cannot be restored
  • Sudden penetrating pain in the lower back, abdomen, or legs
  • Severe vomiting and diarrhea, followed by dehydration
  • Fever
  • Weakness and fatigue
  • Low blood pressure
  • Loss of consciousness

If you have Addison’s disease, you may receive care from many specialists, including an endocrinologist and nutritionist, to manage your symptoms. You may receive:

  • A medicine called hydrocortisone to replace cortisol
  • A medicine called fludrocortisone to replace aldosterone

Your doctor may give you a higher dose of these medicines when you have an infection, injury, surgery, or other stressful conditions to avoid an adrenal crisis.

Although Addison’s disease doesn’t go away, you can live better with it by following these steps:

  • Take your medicines regularly. You can set a daily reminder on your computer device so you don’t miss a dose.
  • Always wear a card, tag, or medical alert bracelet or necklace that says you have Addison’s disease. This will let health care professionals know how to treat you best in case of a medical emergency.
  • Always tell your doctor when you’re feeling stressed or unwell, as they may need to increase your medicine dosage.
  • Always have corticosteroid shots with you and tell people you live with how to give you the shot in an emergency. Ask your doctor about the shots if you don‘t already have it.
  • Ask your doctor for corticosteroid shots if you’re pregnant and having symptoms such as nausea and vomiting that may prevent you from taking your medicines.

Addison’s disease diet

Your doctor may advise you to increase how much salt you have from your foods. Ask your doctor how much salt is safe for you and the best ways to have more salt in your foods. 

Corticosteroids for treating Addison’s disease may also affect your bone health. Have foods rich in calcium and vitamin D, such as salmon, cheese, yogurt, and milk to support your bone health. You can also talk to your doctor about taking supplements to protect your bones.

Addison’s disease is a lifelong condition that can seriously affect your functioning and quality of life. See a doctor if you’re having symptoms that don’t go away, such as extreme tiredness, muscle cramps, hair loss, nausea, vomiting, diarrhea, dehydration, and more. They can let you know what might be the cause and treat accordingly. Left untreated, Addison’s disease can cause you to have a serious and life-threatening complication involving symptoms such as low blood pressure and multiple organ failure. Also, ask your doctor about lifestyle changes that can make it easier to live with Addison’s disease.

Can you suddenly get Addison's disease?

You can have Addison’s disease for months to years without knowing, but symptoms can suddenly start, causing an adrenal crisis.

Can you live a normal life with Addison's disease?

Yes, you can live a normal and healthy life with Addison’s disease by staying on top of your treatments and speaking with your doctor as often as you need to about your treatment, such as if you’re unwell or pregnant.