Addison's disease

Addison's disease is a rare condition that happens when the body doesn't make enough of some hormones. Another name for Addison's disease is primary adrenal insufficiency. With Addison's disease, the adrenal glands make too little of the hormone cortisol. Often, they also make too little of another hormone called aldosterone.

Damage to the adrenal glands causes Addison's disease. Symptoms can start slowly. Early symptoms may include extreme tiredness, salt cravings and weight loss.

Addison's disease can affect anyone. Without treatment, it can be life-threatening. Treatment involves taking lab-made hormones to replace those that are missing.

Addison's disease symptoms usually happen slowly, often over months. The disease may happen so slowly that people who have it might ignore the symptoms at first. Physical stress such as an illness or injury can make symptoms get worse fast.

Early symptoms of Addison's disease can affect you in various ways. Some early symptoms can cause discomfort or loss of energy, including:

• Extreme tiredness, also called fatigue.
• Dizziness or fainting when standing after sitting or lying down. This is due to a type of low blood pressure called postural hypotension.
• Sweating due to low blood sugar, also called hypoglycemia.
• Upset stomach, diarrhea or vomiting.
• Pain in the stomach area, also called the abdomen.
• Muscle cramps, weakness, widespread pain or joint pain.

Other early symptoms can cause changes in how you look, such as:

• Body hair loss.
• Areas of darkened skin, especially on scars and moles. These changes may be harder to see on Black or brown skin.
• Weight loss due to less hunger.

Early Addison's disease symptoms also can affect emotions, mental health and desires. These symptoms include:

• Depression.
• Irritable mood.
• Lower sex drive in women.
• Salt craving.

Emergency symptoms due to adrenal crisis

Sometimes the symptoms of Addison's disease become worse fast. If this happens, it's an emergency known as an adrenal crisis. You also may hear it called an addisonian crisis or acute adrenal failure. Call 911 or your local emergency number if you have Addison's disease with any the following symptoms:

• Serious weakness.
• Sudden, terrible pain in the lower back, stomach area or legs.
• Severe upset stomach, vomiting or diarrhea.
• Extreme loss of body water, also called dehydration.
• Fever.
• Confusion or much less awareness of the surroundings.
• Loss of consciousness.
• Low blood pressure and fainting.

Without fast treatment, an adrenal crisis can lead to death.

When to see a doctor

See a healthcare professional if you have common symptoms of Addison's disease, such as:

• Long-lasting fatigue.
• Muscle weakness.
• Loss of appetite.
• Darkened areas of skin.
• Weight loss that doesn't happen on purpose.
• Serious upset stomach, vomiting or stomach pain.
• Lightheadedness or fainting with standing.
• Salt cravings.

Get emergency care right away if you have any symptoms of an adrenal crisis.

Damage to the adrenal glands causes Addison's disease. These glands sit just above the kidneys. The adrenal glands are part of the system of glands and organs that makes hormones, also called the endocrine system. The adrenal glands make hormones that affect almost every organ and tissue in the body.

The adrenal glands are made up of two layers. The inner layer, called the medulla, makes hormones such as adrenaline. Those hormones control the body's response to stress. The outer layer, called the cortex, makes a group of hormones called corticosteroids. Corticosteroids include:

• Glucocorticoids.These hormones include cortisol, and they affect the body's ability to turn food into energy. They also play a role in the immune system and help the body respond to stress.
• Mineralocorticoids. These hormones include aldosterone. They balance the body's sodium and potassium to keep blood pressure in a healthy range.
• Androgens. In all people, the adrenal glands make small amounts of these sex hormones. They cause male sexual development. And they affect muscle mass, body hair, sex drive, and a sense of well-being in all people.

Addison's disease also is known as primary adrenal insufficiency. A related condition is called secondary adrenal insufficiency. These conditions have different causes.

Addison's disease, also called primary adrenal insufficiency

This condition happens when the outer layer of the adrenal glands becomes damaged and can't make enough hormones. Most often, the damage is due to a disease in which the immune system attacks healthy tissues and organs by mistake. This is called an autoimmune disease. People with Addison's disease are more likely than are other people to have another autoimmune disease as well.

Other causes of Addison's disease can include:

• A serious infection called tuberculosis that mainly affects the lungs and also can destroy the adrenal glands.
• Other infections of the adrenal glands.
• Spread of cancer to the adrenal glands.
• Bleeding into the adrenal glands.
• A group of genetic conditions present at birth that affect the adrenal glands. This is called congenital adrenal hyperplasia.
• Medicines that block the body's ability to make glucocorticoid, such as ketoconazole (Ketozole), mitotane (Lysodren) and etomidate (Amidate). Or medicines that block the action of glucocorticoid in the body, such as mifepristone (Mifeprex, Korlym).
• Treatment for cancer with medicines called checkpoint inhibitors.

Secondary adrenal insufficiency

This type of adrenal insufficiency has many symptoms in common with Addison's disease. But it's more common than Addison's disease. Secondary adrenal insufficiency happens when the pituitary gland near the brain doesn't prompt the adrenal glands to make cortisol.

Typically, the pituitary gland makes a hormone called adrenocorticotropic hormone (ACTH). ACTH in turn causes the outer layer of the adrenal glands to make its hormones, including glucocorticoids and androgens. But with secondary adrenal insufficiency, too little ACTH causes the adrenal glands to make too little of these hormones.

Most symptoms of secondary adrenal insufficiency are like those of Addison's disease. But people with secondary adrenal insufficiency don't develop darkened skin. And they're less likely to have serious dehydration or low blood pressure. They're more likely to have low blood sugar.

Factors that can cause the pituitary gland to make too little ACTH include:

• Pituitary tumors that aren't cancer.
• Surgery or radiation therapy of the pituitary gland.
• Brain injury.

A short-term cause of secondary adrenal insufficiency can happen in people who suddenly stop taking medicines called corticosteroids. These medicines treat conditions such as asthma and arthritis. But stopping the medicine suddenly rather than tapering off can lead to secondary adrenal insufficiency.

Most people who get Addison's disease don't have any factors that put them at higher risk of developing the condition. But the following may raise the risk of adrenal insufficiency:

• A history of having a disease or surgery that affects the pituitary gland or the adrenal glands.
• Certain genetic changes that affect the pituitary or adrenal glands. These include gene changes that cause the inherited disease congenital adrenal hyperplasia.
• Other autoimmune endocrine conditions, such as hypothyroidism or type 1 diabetes.
• Traumatic brain injury.

Addison's disease can lead to other health conditions called complications. These include adrenal crisis, also called addisonian crisis. If you have Addison's disease and haven't started treatment, you may develop this life-threatening complication.

Stress on the body such as injury, infection or illness can trigger adrenal crisis. Typically, the adrenal glands make two or three times the usual amount of cortisol in response to physical stress. But with adrenal insufficiency, the adrenal glands don't make enough cortisol to meet this need. And that can lead to adrenal crisis.

Adrenal crisis results in low blood pressure, low blood levels of sugar and high blood levels of potassium. This complication needs treatment right away.

Addison's disease can't be prevented. But you can take steps to lower the risk of adrenal crisis:

• Talk with your healthcare professional if you always feel tired or weak or are losing weight without trying. Ask if you should get tested for adrenal insufficiency.
• If you have Addison's disease, ask your healthcare professional what to do when you're sick. You'll likely need to learn how to adjust the amount of medicine that you take. You also may need to take the medicine as a shot.
• If you become very sick, go to an emergency room. This is crucial if you're vomiting and you can't take your medicine.

Some people with Addison's disease worry about serious side effects from corticosteroid medicines. But people with Addison's disease aren't likely to get the side effects of high-dose corticosteroids used to treat many other diseases. That's because the dose prescribed is much lower and only replaces the amount that's missing.

If you take corticosteroids, follow up with your healthcare professional regularly to make sure your dose is not too high.

Diagnosis involves the steps that your healthcare team takes to find out if you have Addison's disease. Your healthcare professional talks with you about your medical history and your symptoms. You might have some of the following tests that check for Addison's disease or for secondary adrenal insufficiency:

• Blood test. This test can measure blood levels of sodium, potassium, cortisol and adrenocorticotropic hormone (ACTH). A blood test also can measure proteins called antibodies related to Addison's disease caused by an autoimmune disease.
• ACTH stimulation test. ACTH tells the adrenal glands to make cortisol. This test measures the level of cortisol in the blood before and after a shot of lab-made ACTH.
• Insulin-induced hypoglycemia test. This test is done to find out if the pituitary gland is causing secondary adrenal insufficiency. The test involves checking blood sugar and cortisol levels after a shot of insulin.
• Imaging tests. A CT scan of the stomach area checks the size of the adrenal glands and looks for other issues. An MRI of the pituitary gland can spot damage that may cause secondary adrenal insufficiency.

Addison's disease treatment involves taking medicines to correct the levels of steroid hormones that the body isn't making enough of. Some treatments include corticosteroid medicines taken by mouth such as:

• Hydrocortisone (Cortef), prednisone (Rayos, Prednisone Intensol) or methylprednisolone (Medrol) to replace cortisol. You take these medicines on a schedule. This helps mimic the changes in cortisol levels the body typically goes through over 24 hours.
• Fludrocortisone acetate to replace aldosterone.

You'll likely need plenty of sodium in your diet. This is especially true during heavy exercise and when the weather is hot. It's also true if you have digestive troubles such as diarrhea.

Your healthcare professional may tell you to raise the dose of your medicine for a short time if your body is stressed. Such stress can come from having surgery, an infection or a minor illness. If you're vomiting and can't keep down your medicine, you may need shots of corticosteroids.

Follow these treatment recommendations as well:

• Carry a medical alert card and bracelet at all times. A steroid emergency card and medical alert bracelet or tag let emergency care professionals know what kind of care you need. Also have a written action plan.
• Keep extra medicine handy. It can be dangerous to miss even one day of medicine. So keep a small supply at work and take extra medicine with you when you travel.
• Carry a corticosteroid medicine injection kit. The kit contains a needle, a syringe and an injectable form of corticosteroids to use in case of emergency.
• Stay in contact with your healthcare professional. Your healthcare team can monitor your hormone levels. If you have trouble with your medicine, your healthcare professional may need to change the doses or when you take them.
• Have yearly checkups. At least once a year, see your healthcare professional or a doctor who treats hormone conditions. Your healthcare professional may recommend yearly screening tests for autoimmune diseases.

Addisonian crisis is a medical emergency. Treatment typically includes medicines or solutions given through a vein. These include:

• Corticosteroids.
• Saline solution.
• Sugar.

You're likely to start by seeing your primary healthcare professional. Then, you might be referred to a doctor called an endocrinologist who treats conditions related to hormones.

Here's information to help you get ready for your appointment.

What you can do

Take a family member or friend with you if you can. This person can help you remember the information you get.

Make a list of:

• Your symptoms and when they began.
• Key personal information, including major stresses or recent life changes.
• All medicines, vitamins or other supplements you take, including doses.
• Questions to ask your healthcare professional.

For Addison's disease, questions might include:

• What is likely causing my symptoms or condition?
• What tests do I need?
• Is my condition likely short-term or long lasting?
• What is the best course of action?
• I have other health conditions. How can I best manage them together?
• Are there restrictions I need to follow?
• Are there brochures or other printed material I can have? What websites do you recommend?

Feel free to ask other questions as well.

What to expect from your doctor

Your healthcare professional may ask:

• Do you have symptoms all the time or only sometimes?
• How bad are your symptoms?
• What, if anything, makes your symptoms better?
• What, if anything, makes your symptoms worse?