Addison's disease

Addison's disease, also called adrenal insufficiency, is an uncommon disorder that occurs when your body doesn't produce enough of certain hormones. In Addison's disease, your adrenal glands, located just above your kidneys, produce too little cortisol and, often, too little aldosterone.

Addison's disease occurs in all age groups and both sexes, and can be life-threatening. Treatment involves taking hormones to replace those that are missing.

Symptoms

Addison's disease symptoms usually develop slowly, often over several months. Often, the disease progresses so slowly that symptoms are ignored until a stress, such as illness or injury, occurs and makes symptoms worse. Signs and symptoms may include:

• Extreme fatigue
• Weight loss and decreased appetite
• Darkening of your skin (hyperpigmentation)
• Low blood pressure, even fainting
• Salt craving
• Low blood sugar (hypoglycemia)
• Nausea, diarrhea or vomiting (gastrointestinal symptoms)
• Abdominal pain
• Muscle or joint pains
• Irritability
• Depression or other behavioral symptoms
• Body hair loss or sexual dysfunction in women

Acute adrenal failure (addisonian crisis)

Sometimes the signs and symptoms of Addison's disease may appear suddenly. Acute adrenal failure (addisonian crisis) can lead to life-threatening shock. Seek emergency medical treatment if you experience the following signs and symptoms:

• Severe weakness
• Confusion
• Pain in your lower back or legs
• Severe abdominal pain, vomiting and diarrhea, leading to dehydration
• Reduced consciousness or delirium

In an addisonian crisis you will also have:

• Low blood pressure
• High potassium (hyperkalemia) and low sodium (hyponatremia)

When to see a doctor

See your doctor if you have common signs and symptoms of Addison's disease, such as:

• Darkening areas of skin (hyperpigmentation)
• Severe fatigue
• Unintentional weight loss
• Gastrointestinal problems, such as nausea, vomiting and abdominal pain
• Lightheadedness or fainting
• Salt cravings
• Muscle or joint pains

Causes

Addison's disease is caused by damage to your adrenal glands, resulting in not enough of the hormone cortisol and, often, not enough aldosterone as well. Your adrenal glands are part of your endocrine system. They produce hormones that give instructions to virtually every organ and tissue in your body.

Your adrenal glands are composed of two sections. The interior (medulla) produces adrenaline-like hormones. The outer layer (cortex) produces a group of hormones called corticosteroids. Corticosteroids include:

• Glucocorticoids. These hormones, which include cortisol, influence your body's ability to convert food into energy, play a role in your immune system's inflammatory response and help your body respond to stress.
• Mineralocorticoids. These hormones, which include aldosterone, maintain your body's balance of sodium and potassium to keep your blood pressure normal.
• Androgens. These male sex hormones are produced in small amounts by the adrenal glands in both men and women. They cause sexual development in men, and influence muscle mass, sex drive (libido) and a sense of well-being in both men and women.

Primary adrenal insufficiency

When the cortex is damaged and doesn't produce enough adrenocortical hormones, the condition is called primary adrenal insufficiency. This is most commonly the result of the body attacking itself (autoimmune disease). For unknown reasons, your immune system views the adrenal cortex as foreign, something to attack and destroy. People with Addison's disease are more likely than others to have another autoimmune disease as well.

Other causes of adrenal gland failure may include:

• Tuberculosis
• Other infections of the adrenal glands
• Spread of cancer to the adrenal glands
• Bleeding into the adrenal glands. In this case, you may have an addisonian crisis without any previous symptoms.

Secondary adrenal insufficiency

The pituitary gland makes a hormone called adrenocorticotropic hormone (ACTH). ACTH in turn stimulates the adrenal cortex to produce its hormones. Benign pituitary tumors, inflammation and prior pituitary surgery are common causes of not producing enough pituitary hormone.

Too little ACTH can lead to too little of the glucocorticoids and androgens normally produced by your adrenal glands, even though your adrenal glands themselves aren't damaged. This is called secondary adrenal insufficiency. Mineralocorticoid production is not affected by too little ACTH.

Most symptoms of secondary adrenal insufficiency are similar to those of primary adrenal insufficiency. However, people with secondary adrenal insufficiency don't have hyperpigmentation and are less likely to have severe dehydration or low blood pressure. They're more likely to have low blood sugar.

A temporary cause of secondary adrenal insufficiency occurs when people who take corticosteroids (for example, prednisone) to treat chronic conditions, such as asthma or arthritis, stop taking the corticosteroids all at once rather than tapering off.

Complications

Addisonian crisis

If you have untreated Addison's disease, you may develop an addisonian crisis as a result of physical stress, such as an injury, infection or illness. Normally, the adrenal glands produce two to three times the usual amount of cortisol in response to physical stress. With adrenal insufficiency, the inability to increase cortisol production with stress can lead to an addisonian crisis.

An addisonian crisis is a life-threatening situation that results in low blood pressure, low blood levels of sugar and high blood levels of potassium. You will need immediate medical care.

People with Addison's disease commonly have associated autoimmune diseases.

Prevention

Addison's disease can't be prevented, but there are steps you can take to avoid an addisonian crisis:

• Talk to your doctor if you always feel tired, weak, or are losing weight. Ask about having an adrenal shortage.
• If you have been diagnosed with Addison's disease, ask your doctor about what to do when you're sick. You may need to learn how to increase your dose of corticosteroids.
• If you become very sick, especially if you are vomiting and you can't take your medication, go to the emergency room.

Some people with Addison's disease worry about serious side effects from hydrocortisone or prednisone because they know these occur in people who take these steroids for other reasons.

However, if you have Addison's disease, the adverse effects of high-dose glucocorticoids should not occur, since the dose you are prescribed is replacing the amount that is missing. Make sure to follow up with your doctor on a regular basis to make sure your dose is not too high.

Diagnosis

Your doctor will talk to you first about your medical history and your signs and symptoms. You may undergo some of the following tests:

• Blood test. Tests can measure your blood levels of sodium, potassium, cortisol and adrenocorticotropic hormone (ACTH), which stimulates the adrenal cortex to produce its hormones. A blood test can also measure antibodies associated with autoimmune Addison's disease.
• ACTH stimulation test. ACTH signals your adrenal glands to produce cortisol. This test measures the level of cortisol in your blood before and after an injection of synthetic ACTH.

• Insulin-induced hypoglycemia test. You may be given this test if doctors think you may have adrenal insufficiency as a result of pituitary disease (secondary adrenal insufficiency). The test involves checking your blood sugar (blood glucose) and cortisol levels after an injection of insulin. In healthy people, glucose levels fall and cortisol levels increase.

  In certain situations doctors may do alternative tests for secondary adrenal insufficiency, such as a low-dose ACTH stimulation test, prolonged ACTH stimulation test or glucagon stimulation test.

• Imaging tests. You may undergo a computerized tomography (CT) scan of your abdomen to check the size of your adrenal glands and look for other abnormalities. You may also undergo an MRI scan of your pituitary gland if testing indicates you might have secondary adrenal insufficiency.

Treatment

All treatment for Addison's disease involves medication. You will be given hormone replacement therapy to correct the levels of steroid hormones your body isn't producing. Some options for treatment include oral corticosteroids such as:

• Hydrocortisone (Cortef), prednisone or methylprednisolone to replace cortisol. These hormones are given on a schedule to mimic the normal 24-hour fluctuation of cortisol levels.
• Fludrocortisone acetate to replace aldosterone.

You will need to get plenty of salt (sodium) in your diet, especially during heavy exercise, when the weather is hot or if you have gastrointestinal upsets, such as diarrhea.

Your doctor will also suggest a temporary increase in your medication dosage if your body is stressed, such as from an operation, an infection or a minor illness. If you're ill with vomiting and can't keep down oral medications, you may need injections of corticosteroids.

Other treatment recommendations include:

• Carry a medical alert card and bracelet at all times. A steroid emergency card and medical alert identification will let emergency medical personnel know what kind of care you need. Also have a written action plan.
• Keep extra medication handy. Missing even one day of medication may be dangerous, so keep a small supply of medication at work and with you whenever you travel.
• Carry a glucocorticoid injection kit. The kit contains a needle, syringe and injectable form of corticosteroids to use in case of emergency.
• Stay in contact with your doctor. Keep an ongoing relationship with your doctor to make sure that the doses of replacement hormones are adequate, but not excessive. If you're having ongoing problems with your medications, you may need adjustments in the doses or timing of the medications.
• Have annual checkups. See your doctor or an endocrinology specialist at least once a year. Your doctor may recommend annual screening for a number of autoimmune diseases.

Treatment for an addisonian crisis, which is a medical emergency, typically includes intravenous injections of:

• Corticosteroids
• Saline solution
• Sugar (dextrose)

Potential future treatments

Researchers are working to develop delayed-release corticosteroids, which act more like the human body. They are also working on pumps implanted under the skin that can deliver steroids in more-accurate doses.

Future treatment may eventually involve using adrenocortical stem cells combined with immunomodulatory treatment — modifying the immune response or the immune system — as well as gene therapy.

Coping and support

Support groups are available through the National Adrenal Diseases Foundation.

Preparing for an appointment

You're likely to start by seeing your family doctor or a general practitioner. However, when you call to set up an appointment, you may be referred to an endocrinologist.

Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well-prepared for your appointment. Here's some information to help you get ready for your appointment, and what to expect from your doctor.

What you can do

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
• Write down key personal information, including any major stresses or recent life changes.
• Make a list of all medications, vitamins or supplements that you're taking.
• Take a family member or friend along, if possible. Sometimes it can be difficult to recall all the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions will help you make the most of your time together. List your questions from most important to least important in case time runs out. For Addison's disease, some basic questions to ask your doctor include:

• What is likely causing my symptoms or condition?
• Other than the most likely cause, what are possible causes for my symptoms or condition?
• What kinds of tests do I need?
• Is my condition likely temporary or chronic?
• What is the best course of action?
• What are the alternatives to the primary approach you're suggesting?
• I have other health conditions. How can I best manage them together?
• Are there any restrictions I need to follow?
• Should I see a specialist?
• Is there a generic alternative to the medicine you're prescribing me?
• Are there any brochures or other printed material I can take home with me? What websites do you recommend?

In addition to the questions you've prepared to ask your doctor, don't hesitate to ask questions during your appointment.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to spend more time on. Your doctor may ask:

• When did you first begin experiencing symptoms?
• Have your symptoms been continuous or occasional?
• How severe are your symptoms?
• What, if anything, seems to improve your symptoms?
• What, if anything, appears to worsen your symptoms?

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