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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).
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.
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.
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.