Addison’s Disease

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Addison's disease, identified in the 1850’s by Thomas Addison, is also known as primary adrenal insufficiency and hypocortisolism. It is a long-term endocrine disorder in which the adrenal glands do not produce enough cortisol and often insufficient levels of aldosterone as well.

The adrenal glands are just above your kidneys. They are really two endocrine glands in one. As part of the endocrine system, they produce hormones that give instructions to virtually every organ and tissue in the body.

The inner part of the adrenal gland (called the medulla) produces epinephrine (also called adrenaline) which is produced at times of stress and helps the body respond to “fight or flight” situations by raising the pulse rate, adjusting blood flow, and raising blood sugar. However, the absence of the adrenal medulla and epinephrine does not cause disease.

In contrast, the outer portion of the adrenal gland, the cortex, is more critical. It produces a group of hormones called corticosteroids, which include glucocorticoids, mineralocorticoids and male sex hormones (androgens).

• Glucocorticoids. These hormones, which include cortisol, influence your body's ability to convert food fuels into energy, play a role in your immune system's inflammatory response and help your body respond to stress.
  • Cortisol mobilizes nutrients, modifies the body’s response to inflammation, stimulates the liver to raise the blood sugar, and also helps to control the amount of water in the body.
    • Cortisol production is regulated by another hormone, adrenocorticotrophic hormone (ACTH), made in the pituitary gland which is located just below the brain.
• Mineralocorticoids. These hormones, which include aldosterone, maintain your body's balance of sodium and potassium to keep your blood pressure normal.
  • Aldosterone regulates salt and water levels which affects blood volume and blood pressure.
• 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, libido and a sense of well-being in both men and women.

Classical Addison’s disease results from a loss of both cortisol and aldosterone secretion due to the near total or total destruction of both adrenal glands. This condition is also called primary adrenal insufficiency.

If ACTH is deficient, there will not be enough cortisol produced, although aldosterone may remain adequate. This is secondary adrenal insufficiency, which is distinctly different, but similar to Addison’s disease, since both include a loss of cortisol secretion.

Causes:

The failure of the adrenal glands to produce adrenocortical hormones is most commonly the result of the body attacking itself (autoimmune disease). For unknown reasons, the immune system views the adrenal cortex as foreign, something to attack and destroy. Infections and medications may also cause the disease. Most cases of Addison’s disease occur due to damage or destruction of the adrenal cortex, the outermost layers of the adrenal glands (zona fasciulata, which secretes cortisol and zona glomerulosa, which secretes aldosterone). Symptoms usually do not develop until 90 percent of the adrenal cortex has been damaged.

In the past, tuberculosis was the major cause of Addison’s disease and still remains a major cause of the disorder in developing countries. Less common causes of Addison’s disease include repeated infections especially fungal infections, the spread of cancer from another area of the body to the adrenal glands, bleeding (hemorrhaging) into the adrenal gland, and the abnormal accumulation of a fatty-like substance with the adrenals (amyloidosis).

Major symptoms include chronic or long lasting fatigue (most common), muscle weakness, gastrointestinal abnormalities (loss of appetite; weight loss), abdominal pain, and changes in skin color (darkening of the skin or hyperpigmentation). Behavior and mood changes may also occur in some individuals with Addison's disease. Increased excretion of water and low blood pressure (hypotension) can lead to extremely low concentrations of water in the body (dehydration). The symptoms of Addison's usually develop slowly, but sometimes can develop rapidly, a serious condition called acute adrenal failure. In most cases, Addison's disease occurs when the body's immune system mistakenly attacks the adrenal glands causing slowly progressive damage to the adrenal cortex.

The slowly progressing symptoms of adrenal insufficiency are often ignored until a stressful event, such as surgery, a severe injury, an illness, or pregnancy, causes them to worsen.

Adrenal Crisis

Sudden, severe worsening of adrenal insufficiency symptoms is called adrenal crisis. If the person has Addison’s disease, this worsening can also be called an Addisonian crisis. In most cases, symptoms of adrenal insufficiency become serious enough that people seek medical treatment before an adrenal crisis occurs. However, sometimes symptoms appear for the first time during an adrenal crisis.

• Symptoms of adrenal crisis include:
  • sudden, severe pain in the lower back, abdomen, or legs
  • severe vomiting and diarrhea
  • dehydration
  • low blood pressure
  • loss of consciousness
  • If not treated, an adrenal crisis can cause death.

Treatment for Addison's disease involves taking hormones to replace the insufficient amounts being made by your adrenal glands, in order to mimic the beneficial effects produced by your naturally made hormones.

• As long as the proper dose of replacement medication is taken every day, an Addisonian can have a normal crisis-free life. There are no specific physical or occupational restrictions.
• Routine care includes regular physician visits, avoidance of dehydration, and the use of extra medication during illness. Pregnancy is possible, but will require extra monitoring of the replacement medication.
• Every Addisonian should wear an identification bracelet or preferably a necklace stating that he or she has the disease, to insure proper emergency treatment. An identification card outlining treatment is also suggested.
• Today, people with Addison’s disease should have a normal life expectancy.

Statistics:

• Addison's disease occurs in all age groups and affects both sexes.
• Addison's disease can be life-threatening.
• The frequency rate of Addison's disease in the human population is sometimes estimated at roughly one in 100,000.
• Some put the number closer to 40–144 cases per million population (1/25,000–1/7,000).
• Addison's can affect persons of any age, sex, or ethnicity, but it typically presents in adults between 30 and 50 years of age.
• Research has shown no significant predispositions based on ethnicity.
• Affects about one in 40,000 people in Australia (A rare but deadly disease. By Judy Adamson, June 12, 2003)
• Addison's disease is still a potentially lethal condition, with excess mortality in acute adrenal failure, infection, and sudden death in patients diagnosed at young age. Otherwise, the prognosis is excellent for patients with Addison's disease. (EJE - Normal overall mortality rate in Addison's disease, but young patients are at risk of premature death. (Norwegian Clinical Study, Nov. 2008))

Notables:

• Eunice Mary Shriver (Kennedy) (1921-2009)
• John F Kennedy, 35th President of the USA (1917-1963)
• George Summerbee - (Oct 1914-Apr 1955) - professional football player; (George Summerbee)
• Nathan Gafuik (1985) - Canadian Olympic gymnast
• Sabino Arana (1865-1903) Spanish journalist, writer and politician; (Sabino Arana; died of Addison’s disease)
• Jane Austen (1775-1817) died of Addison’s disease; (A rare but deadly disease. By Judy Adamson, June 12, 2003)

Resources & Additional Reading:

• Wikipedia - Addison’s Disease
• Mayo Clinic - Addison’s disease
• A rare but deadly disease. By Judy Adamson, June 12, 2003
• Medline Plus - Addison Disease
• National Adrenal Disease Foundation - Addison’s Disease
• NIH - Adrenal Insufficiency & Addison’s Disease
• National Organization for Rare Disorders (NORD) - Addison’s Disease (Has list of organizations associated with Addison’s Disease with addresses, phone numbers, email & websites provided.)