Overview

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.

Nov. 10, 2018
References
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